Hem Test 1: Hemolytic Anemias

Front 1

A group of disorders characterized by decreased red cell lifespan

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hemolytic anemias

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Clinical feature of hemolytic anemia

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jaundice
dark urine (tea-colored or red)
pigmented gallstones
ankle ulcers
splenomegaly
aplastic crises w/ Parvovirus B19
inc requirement for folate

Front 3

two signs of jaundice

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scleral icterus
yellow discoloration of skin

Front 4

post-splenectomy blood findings

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target cells
acanthocytes
schistocytes
nucleated red cell
Howell-Jolly bodies

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3 classifications of heolytic anemia

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sites of RBC destruction
acquired v. congenital
mechanism of red cell damage

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Differentiate between extravascular v. intravascular hemolysis

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extra has macrophages in spleen, liver, and marrow remove damaned RBC
intra has RBCs rupture within vasculature, releasing free Hb into circulation

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What would you see in blood for inc red cell production?

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elevated retic ct

Front 8

What would you see in bone marrow for inc red cell production?

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erythroid hyperplasia, reduced M/E ratio

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What would you see in bone for inc red cell production?

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deforming changes in skull and long bones (frontal bossing)

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Biochem consequences of hemolysis in general

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elevated LDH levels (of any cell lysis)
elevated unconjugated bilirubin

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Biochem consequences of intravascular hemolysis

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reduced serum haptoglobin (binding to free Hb)

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Most common defect leading to anemia

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hereditary spherocytosis (dominant)

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What pinches off in hereditary spherocytosis? What is the defect causing this?

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lipid microvesicles pinch off;
defect is in proteins of membrane skeleton

Front 14

How is hereditary spherocytosis dx?

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inc osmotic fragility (lysis occurs at higher tonicity)

Front 15

What is the response with splenectomy in hereditary spherocytosis?

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Stops hemolysis, but spherocytes will persist

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2 tx options for hereditary spherocytosis

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folate supplementation
splenectomy

Front 17

What test can be beneficial to spherocytosis?

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MCHC

Front 18

What is rate-limiting enzyme in pentose-phosphate shunt?

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G6PD

Front 19

In absence of G6PD, what do oxidized Hb to metHb precipitate as? What other path presents with this?

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Heinz bodies; also seen in Thalassemias; spleen pinches off Heinz bodies, leaving bite cell

Front 20

Genetic transmission of G6PD deficiency

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X-linked (10-14% of AA men)

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Agents to avoid in G6PD deficiency

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anti-malarials
sulfa drugs
dapsone
Vitamin K
fava beans
Naphtha (mothballs)

Front 22

How can African pts have normal G6PD levels while having G6PD deficiency?

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its maximal about 7-10d after exposue, but the body compensates by making reticulocytes, which have normal G6PD levels in younger cells

Front 23

Two coatings for autoimmune extravascular hemolysis

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IgG (leads to spherocyte formation) or C3

Front 24

Describe characteristics of warm antibodies.

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React best at 37C, do not agglutinate, IgG

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Describe characteristics of cold antibodies.

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best at <32, do cause RBC agglutination, in nose, fingers

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hallmark of autoimmune hemolytic anemia

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positive Coomb's test

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What does Coomb's reagent consist of?

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antibodies to human IgG and C3

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Smear finding in warm-antibody hemolytic anemias

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spherocytes

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Causes of warm-antibody hemolytic anemia

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primary or secondary to another disorder
can be induced by drugs
can be associated with immune platelet destruction (Evan's syndrome)

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warm-antibody hemolytic anemia clinical features

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can have symptoms of anemia, symptoms based on rate of fall of Hb

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Lab findings of warm-antibody hemolytic anemia

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1. inc retic, inc bili, inc LDH
2. positive Coomb's test (both direct and indirect)
3. Spherocytes

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tx of warm-antibody hemolytic anemia

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immunosuppression is mainstay (corticosteroids, then splenectomy, rituximab as third-line), can transfuse if immediate

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3 mechanisms of drug-induced immune hemolysis

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innocent bystander (quinine, quinidine, isoniazide)
hapten (penicillin and cephalosporin)
true autoimmune (alpha-methyldopa, L-DOPA, procainamide)

Front 34

Cold agglutinin disease antibody?

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IgM

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After clumping, what can cold agglutinin present as?

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distal occlusion

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Cold agglutinin disease therapy infection associations

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mycoplasma or mononucleosis

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Tx of Cold agglutinin disease

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keep pt warm, can use immunosuppression, but steroids and splenectomy usually doesn't help

Front 38

Hallmark of microangiopathic hemolytic anemia

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presence of schistocytes on peripheral smear

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Causes of microangiopathic hemolytic anemia

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TTP/HUS
DIC
malignant HTN
Ecclampsia, HELLP syndrome, acute fatty liver of pregnancy
vasculitis