Pathoma Ch04 Heme

{{c1::Primary}} hemostasis forms a weak platelet plug Secondary hemostasis
stabilizes the weak platelet plug into a thrombus via formation of a cross linked
fibrin mesh BGedit EXPANSION FA2018 PathomaHeme hematology hemostasis
Primary hemostasis is mediated by interactions between {{c1::platelets}} and the
{{c2::vessel wall}} BGedit EXPANSION PathomaHeme fa2018 hematology
hemostasis
{{c1::Secondary}} hemostasis stabilizes the platelet plug The goal of secondary
hemostasis is to form a fibrin mesh around the platelet plug, forming a thrombus
BGedit EXPANSION PathomaHeme fa2018 hematology hemostasis
Secondary hemostasis is mediated by the {{c1::coagulation cascade}} In the
context of endothelial damage / inflammation; the coagulation cascade is initiated
by the tissue factor (extrinsic) pathway, and then maintained / amplified by the
contact activation (intrinsic) pathway BGedit EXPANSION PathomaHeme fa2018
hematology hemostasis
The first step of primary hemostasis is {{c1::transient vasoconstriction}} of the
damaged vessel; which is mediated by {{c2::reflex neural}} stimulation and
{{c3::endothelin-1}} release from endothelial cells BGedit EXPANSION
PathomaHeme fa2018 hematology hemostasis
Aggregation is a balance between pro and anti aggregation factors: Pro-aggregation
- {{c2::TXA2}} - released by platelets Anti-aggregation - {{c1::PGI2}} and
{{c1::NO}} - released by endothelial cells BGedit EXPANSION PathomaHeme
fa2018 hematology hemostasis
Is the plug formed by primary hemostasis stable? {{c1::No}}- Unstable, and easily
dislodged - While temporary, it will stop the bleeding BGedit EXPANSION
Following transient vasoconstriction (primary hemostasis), there is platelet
{{c1::adhesion}} to the surface of the disrupted vessel - this is due to vWF
(from endothelial cells and platelets) cross linking platelets (via GpIb) and
exposed subendothelial collagen - patients with collagen synthesis deficiencies
(Scurvy, Ehlers-Danlos) will thus be susceptible to bleedinBGedit EXPANSION
When a blood vessel is injured, {{c1::von Willebrand factor (vWF)}} binds exposed
{{c2::subendothelial collagen}} - in sites of endothelial damage (high shear
stress); the vWF protein (a large multimer) uncoils to a relaxed state, which can
bind to subendothelial collagen and platelets - thus patients with vWF disease or
collagen synthesis deficiencies will be susceptible to bleeding BGedit EXPANSION
In order to adhere to the damaged endothelium, Platelets bind {{c1::vWF}} using the
{{c2::GPIb}} receptor - In Bernard Soullier, this ligand is defective resulting
in a lack of platelet adhesion --> bleeding BGedit EXPANSION PathomaHeme fa2018
One source of von Willebrand factor (vWF) is from the {{c1::Weibel-Palade bodies}}
of {{c2::endothelial cells}} - synthesized as a monomer, dimerizes and is stored,
then multimerizes to the ultra large form (cleaved on release by ADAMTS13 to the
plasma form) - stored with Factor-VIII as a carrier protein (thus vWF disease can
have increased PTT due to decreased half life of Factor VIII) BGedit EXPANSION
One source of von Willebrand factor (vWF) is from the {{c1::α}} granules of
{{c2::platelets}} - vWF is originally synthesized in the megakaryocyte, and then
when the platelet blebs off in the pulmonary vasculature, the alpha-granules go
with them - this form is NOT bound to Factor-VIII BGedit EXPANSION FA2018
PathomaHeme Uworld hematology hemostasis
Following platelet adhesion (primary hemostasis), there is platelet
{{c1::degranulation}} Platelets release: - Alpha-granules (Fibrinogen, vWF, PF4)
- Dense-granules (ADP, Ca2+, Serotonin) - TXA2 BGedit EXPANSION PathomaHeme fa2018
Binding of ADP to the P2Y1 receptor in primary hemostasis causes {{c1::shape}}
change by upregulating intracellular {{c2::Ca2+}}, promoting effective platelet
plug formation - This is to a stellate shape - Ca2+ is also released into the
environment; helping activate the enzymes of the coagulation cascade BGedit
EXPANSION PathomaHeme fa2018 hematology hemostasis
Binding of ADP to the P2Y12 receptor promotes optimal exposure of the
{{c1::GPIIb/IIIa}} receptor on platelets - induces conformational change of the
receptor to increase affinity for fibrinogen - upregulates more GpIIb//IIIa
receptors to the cell surface - this is the basis for P2Y12 inhibitors
(ticlopidine, clopidogrel) BGedit EXPANSION PathomaHeme fa2018 hematology
hemostasis
Binding of ADP to the P2Y12 receptor leads to {{c2::decreased}} {{c1::cAMP}}
formation which favors platelet activation - basis for PDE inhibitor drugs
(increase cAMP - ex. cilastazol) BGedit EXPANSION fa2018 hematology hemostasis
PathomaHeme
What ion is released from dense granules of platelets during degranulation?
{{c1::Ca2+}} - This helps activate enzymes of the Coagulation Cascade that is
occuring at the same time (all steps requiring Ca2+ have a * by them) BGedit
Thromboxane A2 (TXA2) is synthesized by the platelet enzyme {{c1::cyclooxygenase-1
(COX-1)}} This is released from the platelets upon adhesion / activation; drugs
like celecoxib preserve the activity of COX 1 and thus prevent bleeding side
effects (but increase pro-thrombotic side effect) BGedit EXPANSION PathomaHeme
fa2018 hematology hemostasis
Thromboxane A2 promotes platelet {{c1::aggregation}} - upregulates the GpIIb/IIIa
receptor complex - basis for aspirin anti-platelet therapy BGedit EXPANSION
Following upregulation of GpIIb/IIIa, platelets aggregate at the site of injury via
crosslinking of GpIIb/IIIa receptors with {{c1::Fibrinogen}} to make a temporary
platelet plug - the temporary platelet plug is unstable and is easily dislodged
- Fibrinogen is released from alpha granules of platelets on activation as well as
from the liver BGedit EXPANSION PathomaHeme fa2018 hematology hemostasis
Aggregation is a balance between pro and anti aggregation factors: Pro-aggregation
- {{c1::decreased}} blood flow Anti-aggregation - {{c1::increased}} blood flow
- decreased blood flow encourages accumulation of pro-clotting factors BGedit
Binding between GPIIb/IIIa and fibrinogen results in formation of a {{c1::weak
platelet plug}} this platelet plug is stabilized via secondary hemostasis BGedit
Disorders of primary hemostasis are divided into {{c1::qualitative}} and
{{c1::quantitative}} disorders PathomaHeme hematology hemostasis
Disorders of primary hemostasis present with symptoms of mucosal and skin
{{c1::bleeding}} PathomaHeme hematology hemostasis
Disorders of primary hemostasis present with symptoms of mucosal bleeding, such as
{{c1::epistaxis}} (most common overall symptom) other mucosal bleeds include
hemoptysis, GI bleeding, hematuria, and menorrhagia PathomaHeme hematology
hemostasis
A feared complication of severe thrombocytopenia is {{c1::intracranial}} bleeding
PathomaHeme hematology hemostasis
Quantitative disorders of primary hemostasis present with symptoms of skin
bleeding, such as {{c1::petechiae}} (1-2 mm) petechiae are a sign of
thrombocytopenia; not usually seen with qualitative disorders of primary hemostasis
Disorders of primary hemostasis present with symptoms of skin bleeding, such as
{{c1::purpura}} (> 3 mm) PathomaHeme hematology hemostasis
{{c1::ecchymoses}} (> 1 cm) PathomaHeme hematology hemostasis
easy {{c1::bruising}} PathomaHeme hematology hemostasis
Petechiae are a sign of thrombocytopenia and are not usually seen with
{{c1::qualitative}} platelet disorders PathomaHeme hematology hemostasis
Platelet counts < {{c1::50K}}/μL lead to symptoms PathomaHeme hematology
hemostasis
Normal platelet counts are between {{c1::150K}} and {{c1::400K}}/μL
Bleeding time is normally between {{c1::2}} and {{c1::7}} minutes
{{c2::Bleeding time}} is {{c1::prolonged}} in all platelet disorders (both
quantitative and qualitative) - Test of platelet function; Small cut is made on
patients arm, filter paper applied / removed until bleeding stops - Gauges platelet
function well as superficial cuts are primarily clotted by platelets (coagulation
is not needed) - Really only seen on the boards now, not done much in clinical
practice, replaced by the platelet function analyzer BGedit Boards&Beyond
EXPANSION PathomaHeme Uworld hematology hemostasis
{{c1::Immune thrombocytopenic purpura (ITP)}} is due to autoimmune production of
Ig{{c2::G}} against {{c3::platelet antigens (e.g. GPIIb/IIIa)}} PathomaHeme
{{c1::Immune thrombocytopenic purpura}} is the most common cause of
thrombocytopenia in children and adults Interleukin-11 is used in the treatment
of thrombocytopenia. hematology hemostasis PathomaHeme
The autoantibodies in ITP are produced by plasma cells in the {{c1::spleen}}
The antibody-bound platelets in ITP are consumed by {{c1::splenic macrophages}},
resulting in thrombocytopenia PathomaHeme hematology hemostasis
Immune thrombocytopenic purpura commonly follows {{c1::viral}} infection
The acute form of ITP commonly arises in {{c1::children}} (age group) weeks after a
viral infection or immunization hematology hemostasis PathomaHeme
The {{c1::acute}} form of ITP is typically self-limited, usually resolving within
weeks of presentation PathomaHeme hematology hemostasis
What demographic is associated with the chronic form of immune thrombocytopenic
purpura? {{c1::Women of childbearing age}} may be primary or secondary (e.g.
SLE) PathomaHeme hematology hemostasis
Maternal ITP may cause short-lived thrombocytopenia in offspring since {{c1::anti-
platelet IgG}} can cross the placenta PathomaHeme hematology hemostasis
Immune thrombocytopenic purpura presents with {{c1::increased}} bleeding time / PFA
PFA = platelet function assay (analogous to BT) BGedit EXPANSION PathomaHeme
Uworld hematology hemostasis
Immune thrombocytopenic purpura presents with {{c1::decreased}} platelet count
often < 50K/μL PathomaHeme hematology hemostasis
Immune thrombocytopenic purpura presents with {{c1::normal}} PT and {{c1::normal}}
PTT coagulation factors are not affected PathomaHeme hematology hemostasis
Immune thrombocytopenic purpura presents with increased {{c1::megakaryocytes}} on
bone marrow biopsy hematology hemostasis PathomaHeme
The initial treatment for immune thrombocytopenic purpura is
{{c1::corticosteroids}} children respond well; adults may show early response but
often relapse PathomaHeme hematology hemostasis
What is used to transiently raise the platelet count due to symptomatic bleeding in
immune thrombocytopenic purpura? {{c1::IVIG}} splenic macrophages consume IVIG,
sparing the platelet-bound IgG transiently PathomaHeme hematology hemostasis
The treatment for refractory cases of ITP is {{c1::splenectomy}} eliminates both
the source of the antibody and the site of platelet destruction PathomaHeme
"{{c1::Microangiopathic hemolytic}} anemia occurs when RBCs are ""sheared"" as they
cross {{c2::microthrombi}}" hematology hemostasis PathomaHeme
Microangiopathic hemolytic anemia is often associated with formation of platelet
{{c1::microthrombi}} in small vessels PathomaHeme hematology hemostasis
Microangiopathic hemolytic anemia is seen in {{c1::TTP}}, {{c2::HUS}}, and
{{c3::HELLP}} syndrome may also be seen in SLE and malignant hypertension
"What type of red blood cell is associated with microangiopathic hemolytic anemia?
{{c1::Schistocytes (""helmet cells"")}}" PathomaHeme hematology hemostasis
{{c2::Thrombotic thrombocytopenic purpura (TTP)}} is due to inhibition or
deficiency of the {{c1::ADAMTS13 (vWF metalloprotease)}} enzyme - vWF is a
multimeric protein that exists in a coiled and uncoiled state (uncoils at areas of
high shear stress - ex. endothelial damage); the uncoiled form is important for
platelet binding as well as normal degradation (via ADAMTS13) - However, ADAMTS13
also helps maintain the size of the vWF protein so that it doesn't get too big,
ultra large vWF proteins can cause diffuse microvascular thrombosis and occlusion
BGedit EXPANSION PathomaHeme Uworld hematology hemostasis
{{c1::ADAMSTS13 (vWF metalloprotease)}} is an enzyme that normally cleaves vWF
multimers into smaller monomers for eventual degradation PathomaHeme
In TTP, large, uncleaved vWF multimers lead to increased platelet {{c1::adhesion}}
with consequent {{c2::microthrombi}} formation PathomaHeme hematology
hemostasis
What demographic is associated with thrombotic thrombocytopenic purpura?
{{c1::Adult females}} PathomaHeme hematology hemostasis
Decreased ADAMSTS13 (e.g. TTP) is usually due to an acquired {{c1::autoantibody}}
The pentad of classical symptoms seen in {{c6::thrombotic thrombocytopenic purpura
(TTP)}} may be remembered using the mnemonic FAT RN: F: {{c1::Fever (1/5
patients)}} A*: {{c2::Anemia (Microangiopathic Hemolytic Anemia)}} T*:
{{c3::Thrombocytopenia}} R: {{c4::Renal symptoms (1/2 of patients)}} N:
{{c5::Neurologic symptoms (2/3 of patients)}} *however, only MAHA and
thrombocytopenia are required for diagnosis; HUS presents with similar symptoms,
almost always characterized by normal PT / PTT - fever due to inflammation from
small vessel occlusion and tissue damage - patients can have Burr cells due to
kidney damage BGedit EXPANSION PathomaHeme Uworld fa2018 hematology hemostasis
Thrombotic thrombocytopenic purpura is associated with elevated serum {{c1::LDH}}
What is the treatment (3) for thrombotic thrombocytopenic purpura (TTP)?
{{c1::plasmapheresis}}, {{c2::corticosteroids}}, {{c3::rituximab}} BGedit
{{c1::Hemolytic uremic syndrome}} is characterized by thrombocytopenia,
microangiopathic hemolytic anemia, and {{c2::acute renal failure}} typically
due to endothelial damage by drugs or infection (ex EHEC Shiga Like Toxin); similar
clinical presentation to TTP BGedit EXPANSION PathomaHeme fa2018 hematology
hemostasis
{{c2::Hemolytic uremic}} syndrome is classically seen in {{c3::children}} with
{{c1::E. coli O157:H7}} dysentery, which results from exposure to undercooked beef
In children, typical HUS symptoms are accompanied by {{c1::diarrhea}} due to
infection with E. coli PathomaHeme hematology hemostasis
Does HUS in adults typically present with diarrhea? {{c1::No (E. coli infection not
required in adults)}} PathomaHeme hematology hemostasis
The E. coli O157:H7 {{c1::vero}}-toxin damages endothelial cells, resulting in
platelet microthrombi (e.g. HUS) - Aka Shiga Like Toxin (SLT); A subunit
catalyzes removal of adenine residue preventing tRNA binding to 60S ribosomal
subunit, inhibiting protein synthesis - Microthrombi form on damaged renal
endothelium, leading to platelet aggregation and platelet consumption BGedit
EXPANSION PathomaHeme Uworld fa2018 hematology hemostasis
TTP and HUS present with {{c1::increased}} bleeding time PathomaHeme
TTP and HUS present with {{c1::decreased}} platelet count PathomaHeme
TTP and HUS typically present with {{c1::normal}} PT and {{c1::normal}} PTT
coagulation factors are not affected PathomaHeme hematology hemostasis
The initial treatment for hemolytic uremic syndrome (HUS) is {{c1::plasmapheresis}}
{{c2::Bernard-Soulier}} syndrome is a qualitative platelet disorder due to a
genetic {{c1::GPIb}} deficiency PathomaHeme hematology hemostasis
{{c2::Bernard-Soulier}} syndrome is characterized by {{c1::enlarged platelets}} on
blood smear PathomaHeme hematology hemostasis
In Bernard-Soulier syndrome there is a defect in platelet {{c1::adhesion}}
specifically, a defect in platelet-to-vWF adhesion due to decreased GPIb
{{c2::Glanzmann thrombasthenia}} is a qualitative platelet disorder due to a
genetic {{c1::GPIIb/IIIa}} deficiency PathomaHeme hematology hemostasis
In Glanzmann thrombasthenia there is a defect in platelet {{c1::aggregation}}
specifically, a defect in plate-to-platelet aggregation due to decreased
GPIIb/IIIa PathomaHeme hematology hemostasis
Glanzmann thrombasthenia is characterized by no {{c1::platelet}} clumping on blood
smear Will also have Abnormal Platelet Aggregometry - platelets are mixed with ADP
and arachidonic acid, look to see if they aggregate - absent platelet aggregation
in response to stimuli BGedit Boards&Beyond EXPANSION PathomaHeme fa2018
Bernard-Soulier syndrome presents with {{c1::increased}} bleeding time
hematology hemostasis PathomaHeme
Bernard-Soulier syndrome presents with {{c1::normal}} or {{c1::decreased}} platelet
count mild thrombocytopenia due to slightly decreased half-life of platelets
associated with loss of GPIb PathomaHeme hematology hemostasis
Glanzmann thrombasthenia presents with {{c1::increased}} bleeding time
Glanzmann thrombasthenia presents with {{c1::normal}} platelet count
Aspirin irreversibly inactivates the enzyme {{c1::cyclooxygenase}}, thus decreasing
synthesis of {{c2::TXA2}} and impairing platelet {{c3::aggregation}}
PathomaHeme S2B3 hematology hemostasis
The coagulation cascade generates {{c1::thrombin (factor IIa)}}, which converts
fibrinogen in the platelet plug to {{c2::fibrin (factor Ia)}} PathomaHeme
S2B3 hematology hemostasis
After fibrin is formed from fibrinogen, it is {{c1::cross-linked}} by factor
{{c2::XIII}} yielding a stable platelet-fibrin thrombus A factor XIII deficiency
causes spontaneous or excessive bleeding, but does NOT prolong BT, PT, PTT, OR TT
BGedit EXPANSION PathomaHeme Uworld hematology hemostasis
Factors of the coagulation cascade are produced by the {{c1::liver}} in an inactive
state PathomaHeme hematology hemostasis
Activation of the coagulation cascade requires the {{c1::phospholipid}} surface of
platelets also requires Ca2+ from platelet dense granules PathomaHeme hematology
hemostasis
Activation of the coagulation cascade requires {{c1::Ca2+}} ions derived from
platelet {{c2::dense}} granules also requires the phospholipid surface of
platelets PathomaHeme hematology hemostasis
Disorders of secondary hemostasis typically present with {{c1::deep tissue}}
bleeding (location) e.g. bleeding into muscles and joints PathomaHeme
Disorders of secondary hemostasis typically present with {{c1::re-bleeding}} after
surgical procedures (e.g. dental procedures) PathomaHeme hematology
hemostasis
{{c1::Prothrombin time (PT)}} measures the {{c2::extrinsic}} and common pathways of
the coagulation cascade e.g. factor VII (extrinsic); II, V, X, and fibrinogen
(common) PathomaHeme S2B3 hematology hemostasis
{{c1::Partial thromboplastin time (PTT)}} measures the {{c2::intrinsic}} and common
pathways of the coagulation cascade e.g. factors VIII, IX, XI, XII (intrinsic); II,
V, X, and fibrinogen (common) PathomaHeme hematology hemostasis
International normalized ratio (INR) is calculated from {{c1::PT}} (PT or PTT)
1 = normal, > 1 = prolonged PathomaHeme hematology hemostasis
Which test (PT or PTT) is used to follow the effects of heparin? {{c1::PTT}}
Which test (PT or PTT) is used to follow the effects of warfarin? {{c1::PT (via
INR)}} PathomaHeme hematology hemostasis
Which coagulation factor(s) are part of the extrinsic coagulation cascade?
{{c1::Factor VII}} PathomaHeme hematology hemostasis
Which coagulation factor(s) are part of the instrinsic coagulation cascade?
{{c1::Factor 8, 9, 11, 12}} or VIII, IX, XI, XII... PathomaHeme hematology
hemostasis
Which coagulation factor(s) are part of the common coagulation cascade?
{{c1::Factor 10, 5, 2 (thrombin), 1 (fibrinogen)}} X, V, II, I PathomaHeme S2B3
Factor XI of the coagulation cascade is activated by factor {{c1::XIIa}}
Factor IX of the coagulation cascade is activated by factor {{c1::XIa}}
Factor X of the coagulation cascade may be activated by factor {{c1::VII}}
(extrinsic pathway) PathomaHeme hematology hemostasis
Factor V of the coagulation cascade is activated by factor {{c1::IIa}}
Factor II (prothrombin) is activated to thrombin by factor {{c1::Xa}}
PathomaHeme S2B3 hematology hemostasis
{{c1::Tissue thromboplastin}} activates factor {{c2::VII}}, which initiates the
extrinsic pathway of the coagulation cascade PathomaHeme hematology
hemostasis
{{c1::Subendothelial collagen}} activates factor {{c2::XII}}, which initiates the
intrinsic pathway of the coagulation cascade PathomaHeme hematology
hemostasis
Hemophilia {{c1::A}} is a coagulation disorder due to a genetic factor {{c2::VIII}}
deficiency MC in carboxy terminal and intron 22 inversion PathomaHeme hematology
hemostasis
What is the mode of inheritance of hemophilia A? {{c1::X-linked recessive}}
Hemophilia A can arise from a {{c1::new (de novo)}} mutation without any family
history (20%) PathomaHeme hematology hemostasis
Which coagulation disorder presents with recurrent hemarthroses, spontaneous / easy
bruising, and bleeding after surgery? {{c1::Hemophilia (A, B, C)}} - this type
of bleeding = macrohemorrhage - Chronic hemarthrosis can result in degradation of
the knee / weight bearing; hemarthrosis depicted below clinical severity depends on
degree of deficiency BGedit EXPANSION PathomaHeme fa2018 hematology hemostasis
Hemophilia {{c1::B (Christmas disease)}} is a coagulation disorder due to a genetic
factor {{c2::IX}} deficiency PathomaHeme hematology hemostasis
What is the mode of inheritance of hemophilia B? {{c1::X-linked recessive}}
Hemophilia {{c1::C}} is a coagulation disorder due to a genetic factor {{c2::XI}}
deficiency PathomaHeme hematology hemostasis
What is the mode of inheritance of hemophilia C? {{c1::Autosomal recessive}}
Treatment of hemophilia A involves {{c1::desmopressin}} plus recombinant
{{c2::factor VIII}} hemophilia B and C are treated similarly with recombinants
to their respective factor deficiencies PathomaHeme hematology hemostasis
Hemophilia (A, B, C) presents with {{c1::normal}} bleeding time PathomaHeme
Hemophilia (A, B, C) presents with {{c1::normal}} platelet count PathomaHeme
Hemophilia (A, B, C) presents with {{c1::normal}} PT and {{c1::increased}} PTT
instrinsic pathway coagulation defect PathomaHeme hematology hemostasis
A coagulation factor inhibitor is an {{c1::acquired antibody}} against a
coagulation factor, resulting in impaired factor function PathomaHeme
What is the most common coagulation factor inhibitor? {{c1::Anti-Factor VIII}}
presents similarly to hemophilia A PathomaHeme hematology hemostasis
In hemophilia A, mixing normal plasma with the patient's plasma (mixing study)
{{c1::does}} correct the PTT (does or doesn't) normal plasma replaces the
deficient factor VIII BGedit EXPANSION PathomaHeme fa2018 hematology hemostasis
With a factor VIII coagulation factor inhibitor, mixing normal plasma with a
patient's plasma {{c1::doesn't}} correct the PTT (does or doesn't) the
coagulation factor inhibitor inhibits the factor VIII in the normal plasma --> NO
CORRECTION BGedit EXPANSION PathomaHeme fa2018 hematology hemostasis
{{c1::von Willebrand disease}} is characterized by a genetic vWF deficiency
What is the most common inherited bleeding/coagulation disorder? {{c1::von
Willebrand disease}} PathomaHeme hematology hemostasis
What is the mode of inheritance of the most common subtype of von Willebrand
disease? {{c1::Autosomal dominant}} PathomaHeme hematology hemostasis
The most common subtype of von Willebrand disease presents with {{c1::decreased}}
vWF levels PathomaHeme hematology hemostasis
von Willebrand disease typically presents with mild {{c1::mucosal}} and
{{c1::skin}} bleeding PathomaHeme hematology hemostasis
Low vWF in von Willebrand disease impairs platelet {{c1::adhesion}}
von Willebrand disease presents with {{c1::increased}} bleeding time important
distinguishing feature from factor VIII deficiency PathomaHeme hematology
hemostasis
von Willebrand disease presents with {{c1::normal}} platelet count
von Willebrand disease presents with {{c1::normal}} PT and {{c1::normal}} or
{{c1::increased}} PTT despite elevated PTT, usually does not cause problems with
coagulation cascade PathomaHeme hematology hemostasis
Why may PTT be elevated in von Willebrand disease? {{c1::Decreased factor VIII
half-life (vWF normally stabilizes factor VIII)}} PathomaHeme hematology
hemostasis
von Willebrand disease is associated with an abnormal {{c1::Ristocetin}} assay
i.e. no platelet aggregation with ristocetin cofactor assay - Ristocetin
binds vWF and platelet glycoprotein Ib (thus platelet aggregation if vWF/GpIb
present) - patients serum + ristocetin are placed together, then platelet
aggregation stimuli are added (ADP, arachdonic acid) - normal aggregation = vWF
active, poor aggregation = vWF defective BGedit Boards&Beyond EXPANSION
PathomaHeme Uworld hematology hemostasis
What is the treatment for von Willebrand disease? {{c1::Desmopressin}}
Desmopressin increases release of {{c1::vWF}} from {{c2::Weibel-Palade bodies}}
Vitamin {{c1::K}} is activated by the enzyme {{c2::epoxide reductase}} in the
{{c3::liver}} oxidized vitamin K (inactive) --> reduced vitamin K (active)
hydroquinone PathomaHeme hematology hemostasis
Activated vitamin K acts as a co-factor for the enzyme {{c1::γ-glutamyl
carboxylase}} PathomaHeme hematology hemostasis
Vitamin K is necessary for {{c1::gamma carboxylation}} of factors II, VII, IX, X,
and proteins C and S gamma carboxylation gives coagulation factors a Ca2+
binding site PathomaHeme hematology hemostasis
Which coagulation factors (4) have decreased activity due to vitamin K deficiency?
{{c1::2, 7, 9, 10}} II, VII, IX, X PathomaHeme hematology hemostasis
What proteins (2) have decreased activity due to vitamin K deficiency?
{{c1::protein C and protein S}} PathomaHeme hematology hemostasis
Vitamin K deficiency may occur in {{c1::newborns}} due to lack of {{c2::colonic
bacteria}} that normally synthesize vitamin K vitamin K injection is given
prophylactically to all newborns at birth to prevent hemorrhagic disease of the
newborn BGedit EXPANSION PathomaHeme fa2018 hematology hemostasis
Vitamin K deficiency may occur due to long-term {{c1::antibiotic}} therapy, which
disrupts the gut flora PathomaHeme hematology hemostasis
Vitamin K deficiency may occur due to {{c1::fat}} malabsorption, which leads to
deficiency of fat-soluble vitamins PathomaHeme hematology hemostasis
Vitamin K deficiency presents with {{c1::normal}} bleeding time PathomaHeme
Vitamin K deficiency presents with {{c1::increased}} PT and {{c1::increased}} PTT
- Vitamin K deficiency is essentially acts like a patient with warfarin; PTT
is less sensitive than PT (since VII levels drop first) - This primarily prolongs
PT, with PTT prolongation occuring in severe cases BGedit Boards&Beyond
Abnormal secondary hemostasis may occur due to {{c1::liver}} failure, which causes
decreased production of coagulation factors PathomaHeme hematology
hemostasis
Abnormal secondary hemostasis may occur due to {{c1::liver}} failure, which causes
decreased activation of vitamin K PathomaHeme hematology hemostasis
The effects of liver failure on coagulation is followed using {{c1::PT}} (PT or
PTT) hematology hemostasis PathomaHeme
Abnormal secondary hemostasis may occur due to {{c1::large-volume transfusion}},
which dilutes coagulation factors, resulting in a relative deficiency
Alpha granules of platelets contain {{c1::vWF}} and fibrinogen PathomaHeme
Dense granules of platelets contain {{c1::Ca2+}} and {{c2::ADP}} PathomaHeme
Heparin-induced thrombocytopenia is a disorder that results in platelet
{{c1::activation}} secondary to heparin therapy - activated platelets form plugs
causing thrombosis; platelets are also opsonized and thus are consumed by liver
(and activation) - activated platelets release more PF4 (positive feedback loop) -
occurs in 0.2-5% of patients BGedit EXPANSION PathomaHeme hematology hemostasis
Heparin-induced thrombocytopenia occurs due to production of Ig{{c1::G}} antibodies
against {{c2::heparin-platelet factor IV}} complexes PathomaHeme hematology
hemostasis
The antibody-heparin-PF4 complex may activate {{c1::platelets}}, leading to
thrombosis and thrombocytopenia i.e. heparin-induced thrombocytopenia;
fragments of destroyed platelets activate remaining platelets, leading to
thrombosis PathomaHeme hematology hemostasis
{{c1::Disseminated intravascular coagulation (DIC)}} occurs due to pathologic
activation of the coagulation cascade PathomaHeme hematology hemostasis
Disseminated intravascular coagulation (DIC) is characterized by widespread
{{c1::microthrombi}} formation, resulting in ischemia and infarction
In disseminated intravascular coagulation, consumption of platelets and factors
results in {{c1::bleeding}} hematology hemostasis PathomaHeme
DIC is associated with bleeding, especially from {{c1::IV}} sites and
{{c2::mucosal}} surfaces PathomaHeme hematology hemostasis
"{{c8::Disseminated Intravascular Coagulation (DIC)}} is almost always secondary to
another disease, which may be remembered with ""STOP Making New Thrombi"" S:
{{c1::Sepsis}}, {{c1::Snake bite (rattlesnake)}} T: {{c2::Trauma}} O:
{{c3::Obstetric complications}} P: {{c4::Pancreatitis (acute)}} Making:
{{c5::Malignancy}} New: {{c6::Nephrotic syndrome}} Thrombi: {{c7::Transfusion}}"
Disseminated intravascular coagulation may occur secondary to obstetric
complications due to activation of the coagulation cascade by {{c1::tissue
thromboplastin}} in the amniotic fluid PathomaHeme hematology hemostasis
Disseminated intravascular coagulation may occur secondary to sepsis, especially
gram {{c1::negative}} bacteria e.g. E. Coli or N. meningitidis; endotoxins
from the bacterial wall and cytokines (IL-1, TNF) induce endothelial cells to make
tissue factor PathomaHeme hematology hemostasis
Disseminated intravascular coagulation may occur secondary to malignancy, such as
{{c1::adenocarcinoma}}, due to activation of the coagulation cascade by mucin
Disseminated intravascular coagulation may occur secondary to malignancy, such as
{{c1::acute promyelocytic leukemia}}, due to activation of the coagulation cascade
by primary granules PathomaHeme cancer hematology hemostasis
DIC typically presents with {{c1::increased}} PT and {{c1::increased}} PTT
DIC typically presents with {{c1::decreased}} platelet count important
distinguishing feature from fibrinolysis PathomaHeme hematology hemostasis
DIC typically presents with {{c1::increased}} bleeding time PathomaHeme
DIC typically presents with {{c1::decreased}} fibrinogen PathomaHeme
What type of red blood cell is associated with disseminated intravascular
coagulation? {{c1::Schistocytes (due to microangiopathic hemolytic anemia)}} In DIC
due to gram negative sepsis, wide spread deposition of fibrin leads to shearing
forces of erythrocytes resulting in schistocytes BGedit EXPANSION PathomaHeme
Uworld hematology hemostasis
DIC typically presents with increased {{c1::fibrin}} split products, particularly
{{c2::D-dimer}} PathomaHeme hematology hemostasis
What is the best screening test for DIC? {{c1::Elevated D-dimer}}
D-dimers are derived from splitting of {{c1::cross-linked fibrin}} not produced
from splitting of fibrinogen PathomaHeme hematology hemostasis
Are D-dimers generated from the splitting of fibrinogen? {{c1::No}}
DIC is associated with decreased factors {{c1::V}} and {{c2::VIII}}, which helps
distinguish it from vitamin K deficiency PathomaHeme hematology hemostasis
Treatment of DIC involves addressing the {{c1::underlying cause}}
Treatment of DIC includes transfusion of blood products and {{c1::cryoprecipitate}}
as needed hematology hemostasis PathomaHeme
Normal {{c1::fibrinolysis}} removes a thrombus after a damaged vessel heals
In the first step of fibrinolysis, {{c1::plasminogen}} is converted to
{{c2::plasmin}} via {{c3::tissue plasminogen activator (tPA)}} PathomaHeme
One role of plasmin is {{c1::cleavage}} of fibrin and serum fibrinogen
One role of plasmin is {{c1::destruction}} of coagulation factors
One role of plasmin is inhibition of platelet {{c1::aggregation}}
Plasmin is inactivated by {{c1::α2-antiplasmin}}, which is produced in the liver
Disorders of fibrinolysis are due to {{c1::plasmin}} overactivity, resulting in
excessive cleavage of serum {{c2::fibrinogen}} PathomaHeme hematology
hemostasis
Over-activity of plasmin may occur due to activation by {{c2::urokinase}}, which is
released following radical {{c1::prostastectomy}} hematology hemostasis
PathomaHeme
Over-activity of plasmin may occur due to reduced production of {{c1::α2-
antiplasmin}} by the liver (e.g. cirrhosis) PathomaHeme hematology
hemostasis
Disorders of fibrinolysis present with increased {{c1::bleeding}} (resembles DIC)
Disorders of fibrinolysis typically present with {{c1::increased}} PT and
{{c1::increased}} PTT plasmin destroys coagulation factors PathomaHeme
Disorders of fibrinolysis typically present with {{c1::normal}} platelet count
important distinguishing feature from DIC PathomaHeme hematology hemostasis
Disorders of fibrinolysis typically present with {{c1::increased}} bleeding time
plasmin blocks platelet aggregation PathomaHeme hematology hemostasis
Disorders of fibrinolysis typically present with {{c1::decreased}} fibrinogen
Disorders of fibrinolysis typically present with increased {{c1::fibrinogen}} split
products PathomaHeme hematology hemostasis
Do disorders of fibrinolysis present with elevated D-dimers? {{c1::No}}
important distinguishing feature from DIC PathomaHeme hematology hemostasis
Treatment for disorders of fibrinolysis is {{c1::aminocaproic acid}}, which blocks
activation of {{c2::plasminogen}} PathomaHeme hematology hemostasis
Factor X of the coagulation cascade may be activated by factor {{c1::IXa}} with
factor {{c1::VIIIa}} as a cofactor (intrinsic pathway) PathomaHeme
{{c1::Thrombosis}} is the pathologic formation of an intravascular blood clot
PathomaHeme RespiratoryPathology hemostasis thrombosis
Does thrombosis occur in arteries or veins? {{c1::Both :)}} PathomaHeme
RespiratoryPathology hemostasis thrombosis
Thrombosis most commonly occurs in the {{c1::deep veins (DVT)}} of the leg below
the knee - typically when this occurs, it has little clinical significance -
when thrombosis is above the knee (popliteal, femoral, iliac); patients are at risk
for embolization to lungs BGedit EXPANSION PathomaHeme RespiratoryPathology
Uworld hemostasis thrombosis
Thrombosis is characterized by {{c1::lines of Zahn}}, which are alternating layers
of {{c2::platelets/fibrin}} and {{c3::RBCs}} distinguishing feature of thrombus
from a post-mortem clot PathomaHeme RespiratoryPathology hemostasis thrombosis
Thrombosis is characterized by {{c1::attachment}} to a vessel wall
distinguishing feature of thrombus from a post-mortem clot PathomaHeme
The three major risk factors for thrombosis are {{c1::disruption in blood flow}},
{{c2::hypercoagulability}}, and {{c3::endothelial cell damage}} ({{c4::Virchow}}
triad) PathomaHeme RespiratoryPathology duplicate hemostasis thrombosis
{{c1::Stasis}} and {{c2::turbulence}} of blood flow increases risk for thrombosis
blood flow is normally continuous and laminar; keeps platelets and factors
dispersed and inactivated PathomaHeme RespiratoryPathology hemostasis
thrombosis
Immobilization, cardiac wall dysfunction (arrhythmia, MI), and aneurysm increase
risk of {{c1::thrombosis}} due to disruption of blood flow PathomaHeme
Endothelial cells prevent thrombosis by blocking exposure to underlying
{{c1::subendothelial collagen}} and {{c2::tissue thromboplastin}}
Endothelial cells prevent thrombosis by producing {{c1::prostacyclin (PGI2)}} and
{{c2::NO}} "These are strong vasodilators which increase blood flow, thus
""sweeping"" procoagulant factors out of the area; in pulmonary arterial
hypertension there is endothelial dysfunction which leads to increased
vasoconstrictors and decreased vasodilators" BGedit EXPANSION PathomaHeme
RespiratoryPathology Uworld hemostasis thrombosis
Prostacyclin (PGI2) and NO from endothelial cells cause vaso-{{c1::dilation}}
PathomaHeme RespiratoryPathology S2B3 hemostasis thrombosis
{{c2::Prostacyclin (PGI2)}} and {{c3::NO}} from endothelial cells cause inhibition
of platelet {{c1::aggregation}} PathomaHeme RespiratoryPathology S2B3
hemostasis thrombosis
Endothelial cells prevent thrombosis by secreting {{c1::heparin-like}} molecules,
which augment {{c2::antithrombin III (ATIII)}} ATIII inactivates thrombin and
coagulation factors PathomaHeme RespiratoryPathology S2B3 hemostasis thrombosis
Antithrombin III {{c1::inactivates}} (activates or inactivates) thrombin and
coagulation factors PathomaHeme RespiratoryPathology S2B3 hemostasis
thrombosis
Endothelial cells prevent thrombosis by secreting {{c1::tissue plasminogen
activator (tPA)}}, which converts plasminogen to plasmin plasmin then cleaves
fibrin/fibrinogen, destroys coagulation factors, and blocks platelet aggregation
Endothelial cells prevent thrombosis by producing {{c3::thrombomodulin}}, which
redirects {{c1::thrombin}} to activate {{c2::protein C}} protein C then
inactivates factors V and VIII PathomaHeme RespiratoryPathology S2B3
Which amino acid may cause endothelial cell damage when serum levels are elevated?
{{c1::Homocysteine}} other causes of cell damage include atherosclerosis and
vasculitis PathomaHeme RespiratoryPathology hemostasis thrombosis
Vitamin B12 and folate deficiency result in mildly elevated {{c1::homocysteine}}
levels, increasing risk for thrombosis due to decreased conversion of
homocysteine to methionine PathomaHeme RespiratoryPathology hemostasis
thrombosis
Which vitamin deficiencies (2) are associated with increased risk for thrombosis?
{{c1::Vitamin B12 and B9 (folate)}} due to elevated serum homecysteine PathomaHeme
Cystathionine beta synthase (CBS) deficiency results in high {{c1::homocysteine}}
levels, increasing risk for thrombosis CBS converts homocysteine to
cystathionine PathomaHeme RespiratoryPathology hemostasis thrombosis
A {{c1::hypercoagulable}} state is due to excessive procoagulant proteins or
defective anticoagulant proteins may be inherited or acquired PathomaHeme
The classic presentation of a hypercoagulable state is {{c1::recurrent DVTs}} or
{{c2::DVT at a young age}} usually in deep veins of the legs; other sites
include hepatic and cerebral veins PathomaHeme RespiratoryPathology hemostasis
thrombosis
What is the mode of inheritance of protein C or S deficiency? {{c1::Autosomal
dominant}} hemostasis PathomaHeme RespiratoryPathology thrombosis
Protein C and S normally {{c2::inactivate}} factors {{c1::Va}} and {{c1::VIIIa}}
{{c3::Protein C}} deficiency causes increased risk of {{c1::thrombotic skin
necrosis}} with hemorrhage after {{c2::warfarin}} administration - this is because
protein C is one of the first vitamin K dependent factors to be degraded, leaving
an imbalance of procoagulant (Factor X and Prothrombin) to anticoagulant (Protein
S, which only acts to stimulate Protein C) BGedit EXPANSION PathomaHeme
RespiratoryPathology fa2018 hemostasis thrombosis
{{c2::Warfarin (coumadin)}} is a drug that inhibits the enzyme {{c1::epoxide
reductase}} decreased active vitamin K, decreasing levels of factors 2, 7, 9, 10,
and protein C and S PathomaHeme RespiratoryPathology hemostasis thrombosis
In the initial stages of warfarin therapy, there is a temporary deficiency of
{{c1::protein C}} compared to factors II, VII, IX, and X in pre-existing protein
C deficiency, a severe deficiency is seen at the onset of warfarin therapy
(warfarin skin necrosis) BGedit EXPANSION PathomaHeme RespiratoryPathology
fa2018 hemostasis thrombosis
Why is there a relative protein C and S deficiency in the initial stages of
warfarin therapy? {{c1::Protein C and S have a shorter half-life than factors II,
VII, IX, and X}} PathomaHeme RespiratoryPathology hemostasis thrombosis
{{c1::Factor V Leiden}} is a mutated form of factor V that is resistant to
degradation by activated {{c2::protein C}} lacks the cleavage site for protein
C and S PathomaHeme RespiratoryPathology hemostasis thrombosis
Factor V Leiden is due to a point mutation near the {{c1::cleavage}} site of factor
V PathomaHeme RespiratoryPathology hemostasis thrombosis
What point mutation is the cause of Factor V Leiden? {{c1::Arg506Gln (G -> A point
mutation)}} arginine --> glutamine; AA abbreviations are hard :( PathomaHeme
What is the most common inherited cause of hypercoagulable state in Caucasians?
{{c1::Factor V Leiden}} PathomaHeme RespiratoryPathology hemostasis
thrombosis
{{c2::Prothrombin 20210A}} is an inherited point mutation in the prothrombin gene
that results in {{c1::increased}} gene expression thus increased plasma
thrombin and thrombus formation PathomaHeme RespiratoryPathology S2B3
Prothrombin 20210 mutation is a point mutation in the {{c1::3' untranslated}}
region of the gene PathomaHeme RespiratoryPathology S2B3 hemostasis
thrombosis
{{c1::ATIII}} deficiency decreases the protective effect of heparin-like molecules
produced by the endothelium PathomaHeme RespiratoryPathology hemostasis
thrombosis
What drug enhances activity of antithrombin? {{c1::Heparin}} heparin works by
binding and activating ATIII PathomaHeme RespiratoryPathology S2B3 hemostasis
thrombosis
In {{c1::ATIII}} deficiency, {{c2::PTT}} doesn't rise with standard {{c3::heparin}}
dosing no direct effect on the PT, PTT, or thrombin time PathomaHeme
RespiratoryPathology S2B3 hemostasis thrombosis
What coagulation factors (6) are inhibited by antithrombin? {{c1::2, 7, 9, 10, 11,
12}} or II, VII, IX, X, XI, XII if you like roman numerals... PathomaHeme
RespiratoryPathology S2B3 hemostasis thrombosis
What coagulation factors (2) are the principal targets of antithrombin? {{c1::IIa
(thrombin) and Xa}} PathomaHeme RespiratoryPathology S2B3 hemostasis
thrombosis
What is the treatment for ATIII deficiency? {{c1::High dose heparin, followed by
warfarin}} high doses of heparin activate limited ATIII; coumadin then maintains
an anticoagulated state PathomaHeme RespiratoryPathology hemostasis thrombosis
What is the effect of oral contraceptives on coagulability? {{c1::Increases
coaguability}} due to increased production of coagulation factors secondary to
estrogen PathomaHeme RespiratoryPathology hemostasis thrombosis
What is the effect of estrogen on production of coagulation factors?
{{c1::Increased production}} PathomaHeme RespiratoryPathology hemostasis
thrombosis
{{c2::Hyper}}-coagulable states are often complicated by recurrent
{{c1::pregnancy}} loss (women) other complications include DVTs and cerebral
brain thromboses PathomaHeme RespiratoryPathology hemostasis thrombosis
Tissue plasminogen activator (tPA) may be administered clinically as a
{{c1::thrombolytic}} PathomaHeme RespiratoryPathology hemostasis
thrombosis
The three major risk factors for thrombosis are {{c1::disruption in blood flow
(stasis)}}, {{c2::hypercoagulability}}, and {{c3::endothelial cell damage}}
({{c4::Virchow}} triad) Causes of stasis include (surgery, long drive / flight,
CHF, obesity) causes of hypercoagulability include (factor V leiden / protein C/S
deficiency, OCPs / SERMs / Pregnancy, severe burns, cancer) cause of endothelial
damage include (trauma, fracture, previous DVT, glucagonoma) BGedit EXPANSION
PathomaHeme RespiratoryPathology fa2018 hemostasis thrombosis
The imaging test of choice for deep venous thrombosis is {{c1::compression
ultrasound}} PathomaHeme RespiratoryPathology hemostasis thrombosis
{{c1::DVT}} is a blood clot within a deep vein, causing swelling, redness, warmth,
and pain PathomaHeme RespiratoryPathology hemostasis thrombosis
What lab test is used clinically to rule out DVT (high sensitivity, low
specificity)? {{c1::D-dimer}} hemostasis PathomaHeme RespiratoryPathology
thrombosis
Lower limb {{c3::DVT}} may be associated with {{c1::calf pain}} upon
{{c2::dorsiflexion}} of the foot ({{c4::Homan}} sign) PathomaHeme
{{c1::Unfractioned or LMW heparin}} is used for prophylaxis and acute management of
DVT LMWH includes enoxaparin / dalteparin, use especially for DVT during
pregnancy UFH is given subcutaneously BGedit EXPANSION PathomaHeme
RespiratoryPathology Uworld hemostasis thrombosis
{{c1::Oral anticoagulants (e.g. warfarin)}} are used for treatment and long-term
prevention of DVT PathomaHeme RespiratoryPathology hemostasis thrombosis
A {{c1::thromboembolus}} is due to a thrombus that dislodges (most common type of
embolus, >95%) PathomaHeme RespiratoryPathology embolism hemostasis
{{c1::Atherosclerotic}} embolus is due to an atherosclerotic plaque that embolizes
PathomaHeme RespiratoryPathology embolism hemostasis
{{c2::Atherosclerotic}} embolus is characterized by the presence of
{{c1::cholesterol clefts}} in the embolus PathomaHeme RespiratoryPathology
embolism hemostasis
{{c1::Fat}} embolus is associated with bone {{c3::fractures}}, particularly
{{c2::long}} bones and soft tissue trauma affects less than 10% of patients with
severe skeletal injuries, Results in Fat Embolism Syndrome often require mechanical
ventilation due to ARDS BGedit EXPANSION MedBullets PathomaHeme
RespiratoryPathology Uworld embolism hemostasis
When (chronologically) does a fat embolus typically occur following a bone
fracture? {{c1::While fracture still present or shortly after repair}}
What surgical procedure is associated with fat embolus? {{c1::Liposuction}}
{{c4::Fat}} emboli are characterized by a triad of: - {{c1::hypox}}emia -
{{c2::neurologic}} abnormalities - {{c3::petechial rash overlying the chest}}
These symptoms are seen 1-3 days after the injury - fat globules can escape
lungs by opening precapillary arteriovenous shunts that open due to increased
pulmonary artery pressure (allowing dissemination to the brain --> confusion and
neurologic abnormalities) - once past lungs, may disseminate to dermal capillaries;
petechial rash due to thrombocytopenia (platelets adhere to and coat fat
macroglobules) - anemia is also seen, due to increased RBC aggregation /
destruction, as well as possible pulmonary hemorrhage BGedit Boards&Beyond
EXPANSION PathomaHeme RespiratoryPathology Uworld embolism hemostasis
Fat emboli commonly presents with {{c1::dyspnea}} due to fat, often with bone
marrow elements, in the pulmonary vessels PathomaHeme RespiratoryPathology
embolism hemostasis
{{c1::Gas (air)}} embolus is classically seen in {{c2::decompression}} sickness
({{c3::Caisson}} disease) PathomaHeme RespiratoryPathology embolism
hemostasis
Gas emboli occur due to bubbling of {{c1::nitrogen gas}} out of blood, due to
{{c2::rapid ascent}} by a diver PathomaHeme RespiratoryPathology embolism
hemostasis
{{c1::Gas}} emboli present with joint and muscle pain ('bends') and respiratory
symptoms ('chokes') PathomaHeme RespiratoryPathology embolism hemostasis
Chronic Caisson disease (decompression sickness) is characterized by multifocal
ischemic necrosis of {{c1::bone}} PathomaHeme RespiratoryPathology embolism
hemostasis
What is the treatment for a gas (air) embolus? {{c1::Hyperbaric O2}}
Gas emboli may be iatrogenic, secondary to {{c1::laparoscopic}} procedures as air
is pumped into the abdomen may be caused by other invasive procedures as well
(e.g. central line placement) BGedit EXPANSION PathomaHeme RespiratoryPathology
Uworld embolism hemostasis
{{c1::Amniotic fluid}} emboli enter maternal circulation during labor or delivery
Amniotic fluid emboli can lead to massive hemorrhage and {{c1::DIC}}, especially
postpartum; due to the thrombogenic nature of amniotic fluid This is seen in
the second phase of symptoms; due to amniotic fluid containing high levels of
tissue thromboplastin - seizures can occur in this phase - This is often fatal
BGedit EXPANSION PathomaHeme RespiratoryPathology Uworld embolism hemostasis
Amniotic fluid emboli may present with {{c2::neurologic}} symptoms and
{{c1::respiratory distress}} This is typically seen in Phase I of symptoms
(respiratory distress, hypoxemia, hypotension - caused by arachidonic acid
metabolites in the amniotic fluid) - pulmonary artery vasospasm --> pulmonary HTN
(leading to RHF and hypoxia); - Myocardial capillary damage --> left heart failure
- Pulmonary capillary damage --> ARDS seizures and coma can also be seen in this
phase BGedit Boards&Beyond EXPANSION PathomaHeme RespiratoryPathology Uworld
embolism hemostasis
{{c1::Amniotic fluid}} emboli are characterized by {{c2::squamous}} cells and
{{c3::keratin}} debris in the embolus from fetal skin PathomaHeme
RespiratoryPathology embolism hemostasis
{{c2::Systemic}} embolism is usually due to a thromboembolus that arises from the
{{c1::left heart}} (most common) travel down systemic circulation to occlude
flow to organs, most commonly the lower extremities embolism hemostasis
PathomaHeme RespiratoryPathology
The most common site for systemic emboli to lodge is in the {{c1::lower}}
extremities PathomaHeme RespiratoryPathology embolism hemostasis
Most pulmonary emboli arise via a thromboembolus from the {{c3::proximal}}
{{c1::deep veins}} of the {{c2::lower}} extremity usually involving the
femoral, iliac, or popliteal veins PathomaHeme RespiratoryPathology embolism
hemostasis
What is the most common symptom of pulmonary embolus? {{c1::Most often clinically
silent}} due to dual blood supply of the lung via pulmonary and bronchial
arteries PathomaHeme RespiratoryPathology embolism hemostasis
Pulmonary emboli are usually clinically silent due to the dual blood supply of the
lungs via the {{c1::pulmonary}} and {{c2::bronchial}} arteries BGedit
EXPANSION PathomaHeme RespiratoryPathology Uworld embolism hemostasis
Pulmonary emboli are usually clinically silent as the embolus is usually
{{c1::small}} (size) and self-resolves PathomaHeme RespiratoryPathology
embolism hemostasis
Pulmonary {{c1::infarction}} due to PE occurs if a large- or medium-sized artery is
obstructed in patients with pre-existing {{c2::cardiopulmonary}} compromise only
10% of pulmonary emboli PathomaHeme RespiratoryPathology embolism hemostasis
Pulmonary infarction due to PE may present with {{c1::pleuritic}} chest pain and
pleural effusion PathomaHeme RespiratoryPathology embolism hemostasis
Pulmonary infarction due to PE may present with {{c1::tachypnea}} (RR) and
{{c2::tachycardia}} (HR) PathomaHeme RespiratoryPathology embolism
hemostasis
Pulmonary infarction due to PE may present with hemoptysis and sudden-onset
{{c1::dyspnea}} and chest pain PathomaHeme RespiratoryPathology embolism
hemostasis
In a patient with a pulmonary emboli, V/Q lung scan shows mismatch due to abnormal
{{c1::perfusion}} PathomaHeme RespiratoryPathology embolism hemostasis
The V/Q mismatch seen in pulmonary embolism can result in {{c1::hypoxemia}}, which
causes {{c2::hyper}}-ventilation with consequent respiratory {{c2::alkalosis}}
hyperventilation results in decreased PaCO2 with little effect on PaO2
typically, dead space does NOT lead to hypoxemia, but since dead space is so large,
this causes a hypoxic vasoconstriction shunting of blood to other alveoli resulting
in increased blood flow to remainder of lung additionally, ischemic injury results
in inflammation which results in surfactant deficiency and atelectasis in the
surrounding lung regions; these leads to a high volume of deoxygenated blood
traversing poorly ventilated lung regions (results in right-to-left intrapulmonary
shunting --> Hypoxemia) This will increase Q, leading to a V/Q mismatch - thus some
alveoli are high (V/Q = infinity b/c no blood flow), some alveoli have low V/Q (b/c
of shunted blood flow) - this leads to hypoxemia BGedit EXPANSION PathomaHeme
RespiratoryPathology Uworld embolism hemostasis
{{c1::Spiral CT (CT pulmonary angiography)}} is the imaging test of choice for
pulmonary embolism may also use lower extremity compression ultrasound to look
for DVT; use VQ scan in patients with renal insufficiency or contrast allergies
What may be seen on spiral CT in a patient with a pulmonary embolism?
{{c1::Vascular filling defects}} PathomaHeme RespiratoryPathology embolism
hemostasis
Pulmonary embolism may present with {{c1::elevated}} D-dimer due to lysis of
the PE and DVT PathomaHeme RespiratoryPathology embolism hemostasis
Gross examination of pulmonary infarction due to PE reveals a hemorrhagic,
{{c1::wedge}}-shaped infarct wedge points towards the area of occlusion
The wedge-shaped infarct due to pulmonary embolism points {{c1::towards}} the area
of occlusion (towards or away) PathomaHeme RespiratoryPathology embolism
hemostasis
{{c1::Large saddle}} pulmonary emboli may cause sudden death blocks both left
and right pulmonary arteries or significant occlusion of a large pulmonary artery
Death from a saddle pulmonary emboli is due to {{c1::electromechanical}}
dissociation heart is depolarized but unable to pump blood; also known as
pulseless electrical activity (PEA) BGedit EXPANSION PathomaHeme
RespiratoryPathology embolism fa2018 hemostasis
Pulmonary {{c1::hypertension}} may arise with chronic emboli that are reorganized
over time PathomaHeme RespiratoryPathology embolism hemostasis
A pre-mortem thrombi is distinguishable from a post-mortem thrombi by the presence
of {{c1::lines of Zahn}} and {{c2::attachment}} to a vessel wall PathomaHeme
Iron is absorbed by enterocytes in the {{c1::duodenum}} of the small intestine
RBC_biochem biochemistry vitamins
{{c1::Heme}} iron (meat-derived) is readily absorbed and enters the duodenal cell
via the {{c2::heme}} transporter RBC_biochem biochemistry vitamins
{{c1::Non-heme (Fe3+)}} iron (vegetable-derived) must be converted to Fe2+ in the
duodenum / proximal jejunum by {{c2::cytochrome B}} and/or {{c2::Vitamin C}} for
absorption However, reduction from Fe3+ to Fe2+ occurs in the stomach as well (via
low pH and vitamin C) BGedit EXPANSION RBC_biochem Uworld biochemistry vitamins
After non-heme iron is converted to Fe2+ by cytochrome B, it enters the duodenal
cell by the {{c1::divalent metal transporter 1 (DMT1)}} transporter
Ferrous (Fe2+) iron within the duodenal cell is transported into the blood by the
{{c1::ferroportin}} transporter RBC_biochem biochemistry vitamins
In the plasma, Fe2+ is oxidized to Fe3+ by {{c1::ferroxidase}} enzymes (e.g.
hephaestin, ceruloplasmin) RBC_biochem biochemistry vitamins
The Fe3+ iron in the plasma is bound to {{c1::transferrin}} for transport in the
blood if not transferred to the plasma, iron is stored as ferritin in the cell and
lost in the feces when the intestinal cell sloughs RBC_biochem biochemistry
vitamins
{{c1::Hepcidin}} binds to the {{c2::ferroportin}} transporter on intestinal mucosal
cells and macrophages, thus {{c3::inhibiting}} iron absorption also limits iron
transfer from macrophages to erythroid precursors RBC_biochem biochemistry
vitamins
What gene (and its respective gene product) regulates hepcidin? {{c1::HFE}}
Some of the iron absorbed in the duodenum is taken up by {{c1::hepatocytes}} for
storage as {{c2::ferritin}} RBC_biochem biochemistry vitamins
Some of the iron absorbed in the duodenum is taken up by erythroid precursors in
bone marrow to make {{c1::hemoglobin}} for RBCs RBC_biochem biochemistry
vitamins
After ~120 days, red blood cells are removed by macrophages of the
{{c1::reticuloendothelial}} system RBC_biochem biochemistry vitamins
In the stomach, dietary vitamin B12 is bound to the protein {{c1::R-binder}}, which
is secreted from the {{c2::salivary}} glands RBC_biochem S2B3 biochemistry
vitamins
In the duodenum, B12 dissociates from R-binder via {{c1::pancreatic proteases}} and
attaches to {{c2::intrinsic factor}} intrinsic factor is produced by parietal
cells in the stomach RBC_biochem S2B3 biochemistry vitamins
The B12-IF complex from the duodenum is transported to the {{c1::terminal ileum}},
where it binds to IF receptors and is endocytosed RBC_biochem S2B3
biochemistry vitamins
In the cells of the ileum, the vitamin B12-IF complex dissociates and B12 is bound
to {{c1::transcobalamin II}} RBC_biochem S2B3 biochemistry vitamins
The transcobalamin II-B12 complex is transported to the {{c1::bone marrow}}
(hematopoiesis) and {{c2::liver}} (storage) RBC_biochem S2B3 biochemistry
vitamins

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{{c1::Primary}} hemostasis forms a weak platelet plug Secondary hemostasis

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