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Review.
Soham Puvvada.
Objectives:
Anemia: focus on Iron Deficiency
Anemia.
Myeloproliferative disorders: focus
on P.Vera.
Malignant Heme: focus on CLL.
Peripheral Smear Review.
Production deficiency.
Maturation Defects.
Survival Defects.
Sequestration.
Blood Loss.
Underproduction Anemias:
Kidney Disease: Normochromic, Normocytic with low retic
Maturation Defects
Cytoplasmic:
AOCD
MCV
<85
72-100
MCHC
<32
<36
Iron
Low (<60)
Low (<60)
TIBC
High (>400)
Low (<250)
TIBC sat
Ferritin
Low (<15)
Soluble Transferrin
Receptor
High
Normal
Stainable Marrow
Iron
Absent
Present
Treatment:
Reticulocytosis in 4-7 days
Increased hemoglobin in first several weeks (4-6 classically)
Anemia usually resolves in 4-6 months (depending on etiology of
iron deficiency)
Continue oral replacement for several months after anemia has
resolved to replete iron stores.
Oral Iron: treatment failure sec to non-compliance, treat
constipation
Parental Iron: dextran: can give total dose replacement in
single dose, rate of anaphylaxis 0.6%.
Ferrlecit (Sodium Ferric Gluconate): Usually do not give as a
single dose as total replacement can cause hypotension from
excess of free iron
MKSAP QUESTIONS:
64 y.o man is evaluated for worsening dyspnea and
gradual increase in exercise tolerance over the
past 2 months associated with COPD. He had an
ACS event 2 years ago and his medications include
daily aspirin, bronchodilators, inhaled
corticosteroids, aspirin and statin. On exam, P=90,
BP=130/90, R=20/min. Labs include Hgb
=9.6g/dl(96 x109 L), and MCV=78fL. Stool is
positive for occult blood. Iron deficiency anemia
is diagnosed. Upper endoscopy reveals chronic
gastritis and the daily aspirin is stopped.
A. Blood Transfusion
B. IV Iron
C. Oral Iron
D. Erythropoetin.
A. Folate Deficiency
B. Vitamin B12 deficiency
C Autoimmune Hemolytic Anemia
D. Myelodysplasia
Trait-asymptomatic.
B thal intermedia: anemic, not transfusion dependent.
B thal major: cooleys anemia: severe, growth
retardation, iron overload. Hemoglobin electrophoresis:
persistent elevation of hgbF, variable levels of hgbA2,
and absent HgbA.
Alpha thal:
Survival Defects:
Intrinsic (inherited defects)
Extrinsic (acquired)
MKSAP Questions:
A 20 y/o woman is evaluated for excessive
fatigue. The remainder of the history and
physical exam are non contributory. Labs show
Hgb of 10g/dl, MCV=60fL, RBC count=5.5 million
cells/ul. The leukocyte, platelet counts and
results of Hgb electrophoresis are normal.
Peripheral smear is shown.
/-, /
/-, /-/-, -/
-/-, -/-
Answer: B-corticosteroids.
The patient has warm AIHA.
Polychromasia results from reticulocytosis.
First Rx-steroids.
IVIG and splenectomy are also treatment
options.
Plasmapheresis is not used.
Anemia of
Sequestration:
hypersplenism
usually from portal
hypertension or
splenic
sequestration
crises
Anemia of Blood
loss: self
explanatory.
when loss exceeds
marrow production
may result in a
maturation defect
(iron, b12, folate)
Myeloproliferative Disorders.
CML
Polycythemia Vera
Essential Thrombocythemia.
High risk of thrombosis.
Polycythemia Vera.
Characterized by erythrocytosis.
Proliferative phase, spent phase, secondary AML
Proliferative : Pruritus, erythromelalgia, s/s of
hyperviscosity, thrombosis (arterial or venous),
hemorrhage, GI s/s.
Spent phase: anemia, leukopenia, myelofibrosis,
hepatosplenomegaly.
Exam: may show dilated retinal veins as well as
gouty arthritis.
Diagnosis:
First r/o causes of secondary erythrocytosis.
Lab findings:
Hgb/Hct
WBC in 45%
Plts in 65%
Basophilia (seen in all MPDs)
Uric acid (can lead to gout) and B12
Leukocyte alkaline phosphatase score
Low epo levels
Positive JAK2 V617F
Minor:
Epo.
Endogenous erythroid colony formation in vitro
BMBx showing hypercellularity with prominent
erythroid and megakaryocytic proliferation.
Treatment:
Phlebotomy, goal HCt<45%
Low Dose aspirin to decrease risk of
thrombosis.
Hydroxyurea.
Interferon Alpha.
MKSAP questions
50 y/o man is evaluated for recent onset of
pruritus while showering. He has previously been in
excellent health, eats a normal diet, never smoked,
does not take meds. On exam there are ruddy
facies and a palpable spleen tip. FOBT is negative.
O2 sat=99% RA. Labs show a Hgb of 61.0%
compared with a value of 44.5% documented 5
years ago, WBC=11,000, MCV=79fL, platelet
count= 550,000/ul. Chem nl except for serum
iron concentration and serum ferritin
concentration. Results of upper and lower
endoscopy nl.
Answer :D-phlebotomy
and low dose aspirin.
Pt has P.vera: hct, wbc count, plt count.
Phlebotomy with goal hct 45%, low dose aspirin
to prevent thrombotic complications.
If pts plt count 600,000, hydroxyurea
preferable since it would lower counts of all 3
cell lines.
Anagrelide used to lower plt count-more in ET.
CLL
CLL and SLL are malignant monoclonal accumulation
of immunologically incompetent mature Blymphocytes in blood (>5000/mm3), bone marrow,
or lymph nodes
Characteristic phenotype: CD19,CD20, CD23+ B
cells and also CD5+ (Tcell assoc antigen)
Smudge cells on peripheral smear - reflect
fragility of cells
Presentation
Often asymptomatic, identified on routine CBC.
Lymphadenopathy(80%), Hepatosplenomegaly(50%).
AIHA, ITP.
Hypogammaglobulinemia, increased susceptibility to
infections.
Bone marrow failure
5% monoclonal gammopathy
5% develop Richters transformation; into high grade
lymphoma-usually DLBCL.
Diagnosis:
Prognosis/Treatment:
CLL with somatic mutations of IgG heavy
chain region has indolent course: median
survival 25 years.
CLL without such mutations-with surrogate
marker ZAP 70 =much worse prognosis,
median survival 8 yrs.
Treatment options mirror those for
Follicular lymphoma indolent very
successful in inducing remission, but not
cure
MKSAP question
32 y/o woman is evaluated in ED for acute onset
of fevers, chills nausea, and weakness. Two weeks
ago, she presented to her physician for a
symptomatic UTI and was treated with bactrim.
After 5 days of Rx, she is unable to continue the
medication because of nausea, vomitting. On
exam, she is acutely ill, mottled, lethargic.
Hr=140/min, BP=70/30 mmhg. An indwelling foley
cath is inserted, 20 cc conc. Urine obtained and
sent for culture.
A. Prednisone
B. Cytarabine and Anthracycline
chemotherapy.
C. Granulocyte Colony Stimulating
factor.
D. IVIG.
Answer: C-GCSF.
Sorry, couldnt find a good CLL question.
Severe neutropenia secondary to bactrim.
Her granulocyte count should recover in 10-12
days.
GCSF will shorten the recovery period and may
help with the treatment of severe infection.
Spherocytes
Target Cells
Teardrop Cells
Burr Cells
(echinocytes)
Uremic patients
Spur Cells
(acanthocytes)
Liver disease
Howell-Jolly bodies
Hypersegmented
PMNS
schistocytes
Burr cells
Spur cells
Target cells
Spherocytes
THE END!
References:
MKSAP Hematology-Oncology.