Hemolytic Anemia Flashcards

1
Q

Hemolytic Anemia

A

-Shortened RBC lifespan
-Hemolysis: Destruction of RBCs
-Bone marrow production cannot keep up

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2
Q

Hemolytic Anemia: Clinical Findings

A

-Clinical findings: Fatigue, malaise, pallor, jaundice, splenomegaly, hepatomegaly

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3
Q

Hemolytic increases the risk of:

A

Gallstones

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4
Q

Hemolytic Anemia: Laboratory Tests

A
  1. Lactate dehydrogenase (LDH): Increased
    -LDH-1 found in heart and RBCs
    -Destruction of RBC: Increased LDH-1 and total LDH
  2. Serum unconjugated bilirubin: Increased
    -Often elevated in hemolysis
  3. Reticulocyte Count (Immature RBC): Increased
    -Often elevated in hemolysis
  4. Serum Haptoglobin
    -Finds feee hemoglobin released from RBC destruction
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5
Q

Hereditary Spherocytosis: Etiology

A

Defect in RBC membrane
-Spherocytes
-Destroyed by spleen

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6
Q

Hereditary Spherocytosis: CBC

A

-Mild to moderate anemia: >8g/dL
-Peripheral Blood Smear: Spherocytes
-Additional Tests: Osmotic fragility test (Increases)

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7
Q

Hereditary Spherocytosis: Management

A

-Blood transfusions
-Splenectomy
-Folate Supplementation

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8
Q

G6PD: Etiology

A

-X-linked genetic abnormality of the enzyme
-Triggers: Certain drugs/chemicals, infections (viral & bacterial), stressors, certain foods (fava beans)

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9
Q

G6PD: Clinical Findings

A

-Difficulty fighting infections
-Typical signs of hemolytic anemia

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10
Q

G6PD: Lab Findings

A

-CBC: Moderate anemia
-Peripheral blood smear: Bite cell

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11
Q

Sickle Cell Anemia: Heterozygous vs. Homozygous

A

-Genetic hemoglobinopathy
-Homozygous (S/S): Sickle Cell anemia (more severe)
-Heterozygous (A/S): Sickle Cell trait, usually asymptomatic w/o anemia, exertional sickling (PA, dehydration, high altitude, pain, fatigue)

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12
Q

Sickle Cell Anemia: Etiology

A

-Inflexible
-Odd shape, rigidity
-Increased blood viscosity
-Stasis
-Obstruction of arterioles/capillaries
-Ischemia
-Fragile->hemolysis
-Vaso-occlusive crisis
-Progressive organ failure possible

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13
Q

Sickle Cell Anemia: Clinical Findings

A

-After 6 months of age (HbF->HbA)
-Hemolytic anemia symptoms: Pallor, fatigue & jaundice (severe)

Vaso-occlusive crises
-Symmetrical painful swelling of hands and feet
-Functional asplenia by 5-6 years of age (loss of function of the spleen)
-Increased infection risk
-Delayed physical/sexual maturation
-Painful crises-bones, joints, abdomen, back, viscera
-Chronic abdominal pain
-Lung and kidney problems

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14
Q

Sickle Cell Anemia: Lab Findings

A

-CBC: Moderate to severe anemia, RBC indices are usually normal, In crisis: Leukocytosis, Thrombocytosis
-Peripheral Blood Smear: Sickle Cells
-Miscellaneous: Decreased ESR

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15
Q

Sickle Cell Anemia: Special Tests

A

-Test: Hb electrophoresis (SCA: 80-100% Hb S (no Hb A)/SCT: 20-40% Hb S; 60-80% HbA1 (HbS>60%)
-Screening Tests: “SickleDex”

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16
Q

Sickle Cell Anemia: Treatment/Prognosis

A

-Anemia is lifelong
-In 2nd decade in life, fewer crises, complications more frequent
-Crises start at 1-2 years
-Life expectancy 40-50 years: Infections, thrombosis, pulmonary emboli, pulmonary hypertension, renal failure

17
Q

Thalassemias

A

-Genetic disorder: Impacts rate (slow) of synthesis of adult hemoglobin (Hb A):
Alpha/Beta Thalassemia
-Inadequate Hb and unbalanced accumulation of globin chains: hypochromic, microcytic red cells & hemolytic anemia

18
Q

Thalassemias are more prevalent in:

A

Mediterranean, Middle East, Africa, SE Asia

19
Q

Types of Thalassemia

A

-Thalassemia Trait/Minor (alpha or beta): mild anemia, hypochromic, microcytic
-B-Thalassemia intermedia
-B-Thalassemia major (Cooley’s anemia): severe anemia; Transfusions necessary to sustain life

20
Q

Thalassemia Major: Etiology

A

-Homozygous
-Defective production rates of B-globin polypeptide

21
Q

Thalassemia Major: Management/Treatment

A

-Regular transfusions: Hb>9.3 g/dL
-Folate supplementation
-Treat bacterial infections promptly
-Splenectomy
-Iron management: Avoid iron rich foods/Reduce absorption with certain foods
-Iron overload causes most morbidity & mortality (Average lifespan-30 years of age)

22
Q

Thalassemia Major: Clinical Findings

A

-Severe anemia
-Jaundice
-Splenomegaly
-Hemolytic faces: maxillary hypertrophy, prominent forehead

23
Q

Thalassemia Major: Lab Findings

A

-CBC: Severe anemia
-Peripheral smear: Target cells
-Special Tests: Hb ELP (hemoglobin electrophoresis)->Significant reduction or absent HbA, increased HbF (>50%), increased HbA2

24
Q

Thalassemia Minor: Etiology

A

-Heterozygous
-Defective rate of production of beta globulin chains

25
Q

Thalassemia Minor: Management

A

-No treatment, live healthy
-NL iron panels
-No iron supplement unless warranted

26
Q

Thalassemia Minor: Clinical Findings

A

-Asymptomatic to mild symptoms of anemia

27
Q

Thalassemia Minor: Lab Findings

A

-CBC: Mild anemia (Hb>10g/dL), Slight elevation in RBC count, MCV/MCH/MCHC decrease, RDW: NL
-Peripheral blood smear: Hypo/micro RBCs, Target Cell (may be present)

28
Q

Thalassemia Minor: Special Tests

A

Reticulocytosis (~3%), Hb electrophoresis (Hb ELP), Calculate Mentzer index (MCV/RBC count): <13 thalassemia more likely/>13 iron deficiency more likely

29
Q

Traumatic Hemolytic Anemia: Etiology

A

-March hemoglobinuria
-Karate
-Bongo playing
-Prosthetic heart valves

30
Q

Traumatic Hemolytic Anemia: Lab Findings

A

-Low MCV, high RDW, anemia
-Peripheral blood smear: Schistocytes

31
Q

Hemolytic anemia d/t infectious agents: Etiology

A

-Malaria
-Bacterial toxins-clostridium perfrigens, streptococci, meningococci

32
Q

Hemolytic Anemia d/t immunologic abnormalities

A

-Transfusion of incompatible blood
-Hemolytic disease of the new born: May be d/t ABC incompatibility (more common/less severe) or Rh incompatibility (More severe/less commo

33
Q

HA d/t immunologic abnormalities: Clinical Findings

A

-Anemic newborn
-Jaundice
-Pathological: W/I first 24 hours
-Physiological: After 24 hours after birth

34
Q

HA d/t immunologic findings: Lab Findings

A

-CBC: Anemia
-Special Tests: + Coombs Test (Direct antiglobin test)

35
Q

HA d/t Autoimmune (AIHA)

A

-Acquired anemia induced by binding autoantibodies to RBC -membrane
-Main types defined by maximal binding temperature of antibodies:

36
Q

HA d/t Autoimmune: Warm vs. Cold

A

-Warm (98.6 F) reacting IgG antibody
-Cold (32-39F) reacting IgM antibody (Cold exposure, Elderly, Acrocyanosis-blue discoloration of distal extremities, Raynaud’s Phenomenon

37
Q

HA d/t autoimmune: Risk Factors
(MMAP)

A

-Malignancy
-Autoimmune disorders (Warm IgG)
-Medications
-Prior blood transfusion, hematopoietic cell transplant

38
Q

HA d/t autoimmune: Lab Findings

A

-Direct Coombs’ (DAT-direct antiglobulin tests): Positive test indicates presence of antibodies or complement on RBC surface. Warm (IgG antibodies)/Cold (IgM antibodies)
-CBC: Anemia (normochromic, normocytic)

39
Q

Thalassemia Minor vs. IDA

A

-RDW: Thalassemia Minor (Normal)/IDA (Elevated)
-RBC Count: T Minor (Slight increase)/IDA (Decreased)
-Serum Ferritin: T Minor (Normal)/IDA (decreased)
-Serum Iron: T Minor (normal)/IDA (decreased)
-Target Cells: BOTH may be present
-Mentzer Index: T Minor <13/IDA >13