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Pathology > Anemias Of Decreased RBC SUrvival: Hemolytic Anemias > Flashcards

Anemias Of Decreased RBC SUrvival: Hemolytic Anemias Flashcards

1
Q

What are clinical and lab findings in extravascular hemolysis?

A

Anemia
Jaundice
Splenomegaly
Normal/ mildly decreased plasma hapyoglobin

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2
Q

What are the clinical and lab findings in intravascular hemolysis?

A 
Anemia
Jaundice
Hemoglobinuria
Hemoglobinemia
Hemosiderinuria
Decreased/ absent plasm haptoglobin
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3
Q

How does the bone marrow and blood changes in hemolytic anemias?

A

Anemia and hypoxia trigger renal EPO
EPO in blood binds t committed bone marrow progenitor cellsIncreased numbers of erythroid precursors in marrow= erythroid hyperplasia
Increased numbers of reticulocytes= polychromasia on wright giemsa and reticulocytosis on new methylene blue

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4
Q

What does the reticulocyte count indicated?

A

Low count= decreased production of RBCs, decreased EPO or no marrow response
High count= decreased survival or RBCs ( hemorrhage or hemolysis)

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5
Q

What is the diagnostic approach to hemolytic anemia?

A

Establish if hemolysis occurring
Order tests: serum total bilirubin, unconjugated bilirubin, UA,
Determine underlying cause: H and PE, ROS

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6
Q

What are thalassemia ?

A

Inherited disorders caused by mutations resulting in reduced synthesis of normally structured alpha or beta chains
Alpha results from gene deletions
Beta from point mutations
Prevalent in malarial infections area

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7
Q

What are the causes of the pathology with thalassemia ?

A

Effects of chronic hemolytic anemia
Toxic effects of excess globin chains
Iron overload
Expansion of hematopoietic compartment

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8
Q

What are the disorders associated with alpha thalassemia ?

A

Lost of 4 genes- hydrops fetalis Hb Barts
Lost of 3 genes- HBH disease
Lost of 2 genes- mild anemia
Lost of 1 gene- asymptomatic

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9
Q

What are the disorder associated with beta thalassemia ?

A

Major- lost of both genes or lost of one and reduction of the other- require frequent transfusions
Intermedia- lost of one or reduction in synthesis of both- moderate severity
Minor- lost of one or reduction of one- asymptomatic

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10
Q

What are the clinical features of beta thalassemia major?

A 
Severe microcytic hypochromic anemia
HbF or HbA2 elevated
Lifespan is short unless transfused
Risk of iron overload with frequent transfusions
Crew cut appearance on X ray
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11
Q

What are the lab findings in thalassemia ?

A

Increased serum iron and % saturation
Decreased TIBC
Increased or normal bone marrow iron

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12
Q

What are the characteristics of Sickle cell anemia?

A

Single point mutation in beta globin
Valine substitution for glutamate at position 6
Severe disease if homozygous
Sickling with hypoxia or dehydration
Heterozygosity confer protection against plasmodium falciparum malaria

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13
Q

What are the clinical features of sickle cell disease?

A

Repeated infarcts result in functional asplenia
Susceptible to infection with encapsulated organisms
Osteomyelitis caused by salmonella species

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14
Q

How to diagnose sickle cell anemia?

A

Neonatal screening
Rapid screen
HPLC- definitive diagnosis

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15
Q

What are the characteristics of Hereditary spherocytosis?

A

Cytoskeletal defect
Spherocytes, loss of membrane
Diagnosed with osmotic fragility test

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16
Q

What are the characteristics of hemolysis due to G6PD deficiency ?

A

X linked recessive trait
Protect against plasmodium falciparum
Defect in reducing NADP to NADPH
Oxidative injury leads to Heinz bodies and bite cells
Hemolysis is episodic only after exposure ti an oxidant stress

17
Q

What are the immune hemolytic anemia?

A

RBCs survival decreased due to deposition of immune globulin and /or complement on the RBC membrane

18
Q

What are the 3 groups of antibodies in immune hemolytic anemia?

A

Autoantibodies- cold and warm autoantibodies, cold agglutinin disease
Alloantibodies- transfusion related
Drug related antibodies

19
Q

What is paroxysmal nocturnal hemoglobinuria ?

A

Only acquired hemolytic anemia associated with an intrinsic defect of the RBC membrane
Called the great impersonator
RBCs susceptible to complement mediated hemolysis

20
Q

How to diagnose PNH?

A 
Flow cytometry: loss of CD55 and CD59
Increased LDH
Low/ absent haptoglobin
Hams test
Complement lysis sensitivity test
Sucrose lysis test

Pathology (9 decks)

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