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ANEMIA

Classified by cause
1. Decreased red cell production
a. Stem cell damage - neutrophils, platelets often
affected also
b. Defective red cell maturation
2. Increased red cell destruction (hemolysis)
a. Intrinsic defect in red cell leading to
shortened lifespan
b. External factors in blood or blood vessels
destroy red cells
3. Blood loss
HEMOLYTIC ANEMIA

 Increased rate of red cell destruction

 Increased rate of production - increased


reticulocytes

 Red cell destruction causes increased bilirubin


production and jaundice

 Most red cell destruction occurs in spleen


• splenectomy may cause improvement
Hemolytic anemia: low hematocrit, plasma too yellow due to high bilirubin
INHERITED HEMOLYTIC ANEMIA
Sickle cell anemia
 Mutation changes structure of hemoglobin
 Mutant hemoglobin (deoxy form) polymerizes in cells
and damages cell membranes
• membrane damage causes hemolysis
• hemoglobin crystals change cell shape to "sickle"
• sickled cells are rigid and block small blood vessels,
causing tissue damage O
2

“Sickled” cell

 Genetics: mainly affects those of African and Middle


Eastern descent; recessive inheritance (carriers
partially protected from malaria)
SICKLE CELL ANEMIA

Sickle cell Normal


Sickle Cell Normal red cell

Sickle cells inflexible,


can’t do this
Sickle cell anemia - pathophysiology
Retinal vessel occlusion in sickle disease
IMMUNE HEMOLYTIC ANEMIA

 Production of "autoantibodies" against


one's own red cells
 Antibodies coat cells and lead to
destruction in spleen and liver
 Positive Coombs test (detects antibodies
on red cells) in most cases
 Treatment: corticosteroids, splenectomy,
i.v. gamma globulin
TRANSFUSION REACTION
 Giving a person blood of the wrong type may cause
destruction of the transfused cells (hemolysis) by antibodies
in the recipient's blood

 The most serious reactions occur with blood mismatched for


antigens in the ABO system:
• giving O patient A, B, or AB blood
• giving A patient B or AB blood
• giving B patient A or AB blood

 In such instances there may be very rapid hemolysis


accompanied by shock, kidney failure, bleeding, and death
HEMOLYTIC DISEASE OF THE NEWBORN
 Caused by maternal antibodies against antigens on
fetal red cells (usually Rh antigens); mother usually
exposed (sensitized) to Rh antigen during prior
pregnancy

 These antibodies cross the placenta and cause


destruction of fetal red cells

 Infant liver unable to properly metabolize


hemoglobin breakdown products (bilirubin)

 Stillbirth or anemia, jaundice, and brain damage


may result

 Prevention: prevent sensitization by giving


antibody against Rh factor (Rhogam) to Rh-negative
woman soon after delivery of Rh-positive child
POLYCYTHEMIA

 Definition: increased total red cell volume


• high hematocrit
• thick blood can cause thrombosis, other circulatory
disorders

 Polycythemia vera: increased, unregulated red cell


production
• Most cases due to an acquired mutation in marrow
cells that makes red cell precursors much more
sensitive to erythropoietin

 Secondary polycythemia: increased erythropoietin


production due to decreased oxygen delivery to kidney
• Often due to low levels of oxygen in the blood
NEUTROPHIL DISORDERS
 Neutropenia (decreased neutrophils)
• Decreased production (bone marrow failure,
cancer chemotherapy)
• Increased consumption (some infections, enlarged
spleen, autoimmune)
 Increased risk of infection when neutrophil count
low

 Neutrophilia (increased neutrophils)


• Increased production due to physiologic stimuli
(e.g., infection)
• Increased production due to bone marrow
neoplasm
10/31/97 11/7/97 2/12/98

neutrophils 0 neutrophils 19,000 neutrophils 1200


CANCERS OF THE BLOOD
AND LYMPHATIC SYSTEMS
LEUKEMIA
 Malignant proliferation of white cells and/or their
precursors (blasts)

 Myelogenous (neutrophil precursors)


• Acute myelogenous leukemia (AML)
• Chronic myelogenous leukemia (CML)
 Lymphocytic
• Acute lymphocytic leukemia (ALL)
• Chronic lymphocytic leukemia (CLL)

 Chronic leukemias: more mature cells, slow-growing


 Acute leukemias: immature cells (blasts), fast-
growing
PATHOPHYSIOLOGY OF LEUKEMIA

 Bone marrow failure (marrow fills with


leukemic cells)
• anemia
• neutropenia (infections)
• thrombocytopenia (bleeding)
 Leukemic cells in blood may impair
circulation
 Leukemic cells in other organs
• spleen, lymph nodes
• skin
• brain
 Toxic substances from leukemic cells
• uric acid (gout, kidney failure)
• proteolytic enzymes (tissue damage,
bleeding)
White cells

Leukemia
GUM INFILTRATION IN ACUTE LEUKEMIA
SKIN INFILTRATION IN ACUTE LEUKEMIA
CEREBRAL HEMORRHAGE
IN ACUTE LEUKEMIA
PURPURA IN LEUKEMIA
DISSEMINATED FUNGAL INFECTION
IN ACUTE LEUKEMIA
ACUTE LEUKEMIAS
 Acute myelogenous leukemia (AML)
• adults > children
• fatal if untreated
• remission, occasional cure possible with intensive
chemotherapy
• sometimes curable with bone marrow transplant

 Acute lymphocytic leukemia (ALL)


• children and adults (most common childhood leukemia)
• fatal if untreated
• curable with chemotherapy or bone marrow
transplantation
• Cure rates in children > 75%
DIFFERENTIATION OF
NEUTROPHILS AND RED CELLS

Cells capable of division Cells cannot divide


Bone marrow in acute leukemia

Normal AML
Immature cells (blasts) in
Mature lymphocytes in
acute myelogenous
chronic lymphocytic leukemia
leukemia
CHRONIC LEUKEMIAS
 Chronic myelogenous leukemia (CML)
• rare in children
• treatable but often fatal within 5-10 years
 Newer treatments will probably improve the prognosis
• may be curable with bone marrow transplantation

 Chronic lymphocytic leukemia (CLL)


• almost all patients middle-aged and older
• treatable but incurable
• not all patients need treatment, many live > 10
years
Enlarged lymph
nodes
(lymphadenopathy)
in chronic
lymphocytic
leukemia
LYMPHOMAS
 Cancer of lymphocytes or their precursors
 Forms tumors in lymph nodes, spleen, bone marrow,
other organs
• Sometimes in blood - overlap with lymphoid leukemia
 Many different kinds - spectrum of severity
• Hodgkin's vs non-Hodgkin's
• B-cell vs T-cell
• Mature vs immature lymphoid cells
• Fast vs slow-growing
 Usually treatable, sometimes curable (chemotherapy,
radiation, marrow transplantation)
NON-HODGKIN'S LYMPHOMAS
Many types, complex classification scheme
 LOW GRADE
• Cells appear mature
• Good news: slow-growing - patients may live many years with
disease
• Bad news: treatable but generally incurable
• Usually affect older people
 HIGH GRADE
• Cells less mature
• The most aggressive forms resemble acute lymphoblastic leukemia
• Good news: often curable with chemotherapy
• Bad news: faster-growing, fatal in months if not treated or
treatment doesn't work
• Some varieties occur in children
• Example: Burkitt lymphoma (resembles acute leukemia)

 Rule of thumb: the less mature the cancer cell, the


faster growing the lymphoma, and the more likely the
disease is to affect younger people and to be curable
HODGKIN'S DISEASE
 Probably a cancer of lymphocytes (lymphoma)
 Almost always begins in lymph nodes
 Spreads gradually to other lymph nodes & organs
stage = extent of spread
 Relatively common in young adults
 Curable in many cases with radiation or
chemotherapy

“Reed-Sternberg” cell
characteristic of
Hodgkin’s disease
HODGKIN’S DISEASE
Staging

• Stage I: single lymph node or contiguous group of nodes


• Stage II: more than one node group, same side of diaphragm
• Stage III: confined to nodes (and/or spleen) but present on
both sides of diaphragm
• Stage IV: spread outside nodes (liver, bone marrow, lung,
etc)

• Presence of symptoms (fever, weight loss, night sweats)


designated by "B" after stage (no symptoms = "A")

• Lower stage disease often treated with radiotherapy; higher


stage disease with chemotherapy
HODGKIN’S DISEASE
Response to treatment

Before treatment After 6 months After 7 years


(Treatment
completed)
MULTIPLE MYELOMA

 Cancer of plasma cells (antibody-producing


cells)

 Most patients have monoclonal immunoglobulin


(antibody-like protein) in blood or urine
• This protein may damage kidneys, other
organs

 Bone destruction, bone marrow failure common


MULTIPLE MYELOMA

Red cells Plasma cell


stuck
together by
abnormal
protein
(rouleaux)
BONE LESIONS IN MYELOMA

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