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HEMA 337 > Module 5: Hemolytic Anemias > Flashcards

Module 5: Hemolytic Anemias Flashcards

1
Q

Defects in RBC membranes causing hemolytic anemias:

A

Hereditary spherocytosis
Hereditary elliptocytosis
Hereditary acanthocytosis/abetalipoproteinemia
Hereditary stomatocytosis

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2
Q

Enzyme defects causing hemolytic anemias:

A

Glucose 6 phosphate dehydrogenase deficiency

Pyruvate kinase deficiency

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3
Q

Abnormal hemoglobins causing hemolytic anemias:

A 
Sickle cell trait
Sickle cell anemia
Alpha thalassemia
Beta thalassemia minor
Beta thalassemia major (Cooley anemia)
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4
Q

Acquired intracorpuscular defect causing hemolytic anemia:

A

Paroxysmal nocturnal hemoglobinuria

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5
Q

Immune hemolytic anemias:

A

Due to alloantibodies (HDN, transfusion reactions)
Autoimmune hemolytic anemia (warm, cold)
Due to complement activation (paroxysmal cold hemoglobinuria)

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6
Q

What is hereditary spherocytosis?

A

Autosomal dominant intracorpuscular RBC membrane defect
Deficiency/abnormality of membrane proteins
Decreased SA:volume ratio
Cells become rigid

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7
Q

Hereditary spherocytosis: intra/exravascular hemolysis?

A

Extravascular

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8
Q

Major lab findings in hereditary spherocytosis:

A 
Microspherocytes
Polychromasia
Increased osmotic fragility
DAT negative
Increased autohemolysis corrected with glucose or ATP
Increased MCHC
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9
Q

What is hereditary elliptocytosis?

A

Autosomal dominant intracorpuscular RBC membrane defect

Deficiency of membrane cytoskeletal proteins

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10
Q

Hereditary elliptoocytosis: intra/exravascular hemolysis?

A

Extravascular

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11
Q

Major lab findings in hereditary elliptocytosis:

A

Enlarged spleen
Hyperbilirubinemia
25-95% elliptocytes
Spherocytes and polychromasia

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12
Q

General lab findings in hemolytic anemias

A 
Hb decreased
Hct decreased
RBC count decreased
Relic count increased
Blood film: polychromasia, poikilocytosis
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13
Q

What is hereditary acanthocytosis/abetalipoproteinemia?

A

Intracorpuscular RBC membrane defect
Deficiency of beta lipoprotein
Sphingomyelin/lecithin ratio is increased

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14
Q

Hereditary acanthocytosis/abetalipoproteinemia: intra/exravascular hemolysis?

A

Extravascular

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15
Q

Major lab findings in hereditary acanthocytosis/abetalipoproteinemia:

A

Acanthocytes
Low ESR
Mechanical fragility increased
Cholesterol and triglycerides usually decreased

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16
Q

What is hereditary stomatocytosis?

A

Intracorpuscular RBC membrane defect

Increased passive permeability of Na and K causes net swelling of the cell (gain Na, lose K)

17
Q

Hereditary stomatocytosis: intra/exravascular hemolysis?

A

Intravascular

18
Q

Major lab findings in hereditary stomatocytosis:

A

10-30% stomatocytes
No spherocytes
Autohemolysis increased, partly corrected with ATP and glucose
Osmotic fragility increased

19
Q

What is glucose 6 phosphate dehydrogenase deficiency?

A

Most common enzyme defect

Decreased G6PD –> increased NADPH –> decreased glutathione –>increased methemoglobin (oxidized hemoglobin)

20
Q

Glucose 6 phosphate dehydrogenase deficiency: intra/exravascular hemolysis?

A

Intravascular

21
Q

Major lab findings in glucose 6 phosphate dehydrogenase deficiency:

A 
Bite cells and schistocytes
Heinz bodies
Polychromasia
Decreased haptoglobin
Autohemolysis increased, corrected with glucose
Enzyme assay
22
Q

What is pyruvate kinase deficiency?

A

Causes chronic anemia

Deceased ATP production, hemolysis due to failure of active transport and other ATP dependent reactions

23
Q

Hereditary pyruvate kinase deficiency: intra/exravascular hemolysis?

A

Extravascular

24
Q

Major lab findings in pyruvate kinase deficiency:

A 
Increased 2,3 BPG
Enlarged spleen
Autohemolysis increased, corrected with ATP but not glucose
No characteristic morphology
Enzyme assay
25
Q

What is sickle cell trait?

A 
Hemoglobin A 55-85%
Hemoglobin S 20-45%
Heterozygous A and S
On chromosome 11
Resistance to plasmodium falciparum
26
Q

Hereditary sickle cell trait: intra/exravascular hemolysis?

A

Extravascular

27
Q

Major lab findings in sickle cell trait:

A 
Usually asymptomatic
May see hypo chromic/microcytic anemia
Target cells
Decreased oxygen: crisis (sickle cell anemia)
Hemoglobin electrophoresis

HEMA 337 (7 decks)

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