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Heme Midterm 1 > Hemolysis Information > Flashcards

Hemolysis Information Flashcards

1
Q

is a patient always anemic with a hemolytic disorder?

A

no…marrow compensation can make up for the loss of RBCs

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2
Q

list 5 clinical features of hemolytic anemias

A

jaundice, dark urine, pigmented gallstones, chronic ankle ulcers, splenomegaly

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3
Q

parvovirus B19 affect on RBC production?

A

for about a week the virus will shut down RBC production in bone marrow….for normal individual this is okay but for sickle cell patient this is not

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4
Q

scleral icterus

A

yellowing of sclera in eyes

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5
Q

what 3 organisms do you vaccinate against prior to splenectomy?

A

haemophilus B, meningitis, pneumococcus

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6
Q

blood smear findings after splenectomy?

A

howelljolly bodies…small DNA remnants

if these are gone…then spleen may be coming back

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7
Q

aplastic crisis

A

reticulocyte and hemoglobin concentrations can plummit due to marrow being turned off for 7 days from the non encapsulated parvovirus B19

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8
Q

does parvovirus have a capsid or envelope?

A

NOOOOOOO..will not respond to solvents against membrane of viruses

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9
Q

extravascular hemolysis

A

macrophages in spleen and liver and marrow remove damaged or antibody coated RBCs

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10
Q

intravascular hemolysis

A

RBCs rupture within the vasculature

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11
Q

three modes of evidence for RBC production

A

elevated reticulocyte count

erythroid hyperplasia

deformation of long bones and skull

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12
Q

erythroid hyperplasia definition

A

more RBC precursors and way less myeloid precursors

usually more myeloid precursors but now more erythroid

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13
Q

two main pieces of evidence showing hemolysis

A

elevated LDH levels from increase in lysis

elevated bilirubin levels

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14
Q

why is LDH elevated in hemolytic issues?

A

LDH is a biproduct of lysis of cells

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15
Q

what type of bilirubin is elevated in hemolysis?

A

unconjugated…normally the liver is able to conjugate bilirubin but in case of hemolysis the liver is overwhelmed and we see more unconjugated bilirubin

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16
Q

When does reduced serum haptoglobin occur?

A

when hemolysis is occurring intravascularly

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17
Q

Why is haptoglobin lowered in intravascular hemolysis?

A

haptoglobin is made in liver and has longer half life…but when hemoglobin is available to bind the half life is shortened to minutes and leads to huge decrease

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18
Q

What is the problem with measuring hemoglobin A1C in a patient with high blood sugar and hemolytic anemia?

A

hemoglobin molecules dont hang around long enough to become glycosylated…so A1C will be falsely low!

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19
Q

hereditary spherocytosis mode of inheritance

A

autosomal dominant

20
Q

hereditary spherocytosis problem

A

defect in a membrane protein and the lipid bilayer doesnt get tacked down outside of the RBC…the lipid microvesicles are then pinched off in the spleen

can have issue with parvovirus B19

21
Q

what is common molecule leading to hereditary spherocytosis?

A

ankyrinin

22
Q

CBC sign for hereditary spherocytosis

A

MCHC is elevated…only disease this changes

23
Q

main mode of diagnosis of hereditary spherocytosis

A

eosin-5maleimide flow cytometry staining…when staining drops it is indicative of the disorder

24
Q

osmotic fragility

A

a test for hereditary spherocytosis that is not done anymore…lysis occurs at higher tonicities

25
Q

treatment for hereditary spherocytosis

A

folate supplement and splenectomy

26
Q

importance of glucose 6 phosphate and G6PD enzyme for RBCs?

A

these molecules are part of the pentose pathway shunt in aerobic metabolism and help detoxify metabolites of oxidative stress….they protect RBCs from oxidative damage

27
Q

what happens if you have low G6PD?

A

the oxidizing agents can act on hemoglobin and will lead to methemoglobin..denaturing the Hb

28
Q

What is seen on smear with low G6PD?

A

heinz bodies lead to bite cells form macros biting off part off the heinz body from the RBCs

29
Q

mode of inheritance of G6PD deficiency?

A

X linked, common in african americans, mediterranean variant is more severe with baseline levels

30
Q

things to avoid if have G6PD deficiency?

A

fava beans, sulfa drugs like bactrim and septra, anti malarials, vitamin K, dapsone, mothballs

31
Q

what to expect following a hemolytic episode from deficient G6PD?

A

G6PD levels in african americans may be normal since mature cells were lysed and young cells with regular G6PD will be left

in mediterranean since have baseline levels of deficiency you will see immediately following hemolytci episode

32
Q

warm antibody autoimmune hemolytic anemia…antibody type and agglutination or not?

A

mediated by IgG antibodies…do not lead to agglutination

33
Q

cold antibody autoimmune hemolytic anemia…antibody type and agglutination or not?

A

mediated by IgM and does lead to agglutination

34
Q

what test to do for warm autoimmune hemolytic anemias?

A

direct coombs test

35
Q

How does a direct coombs test work?

A

a patients RBCs will either be coated with antibodies or C3 from complement…coombs test has IgG antibodies for both of these that will bind them and then lead to clumping seen in test tube…must follow with specific test to see if it is IgG antibody or complement bound to the RBCz

36
Q

process of disease in warm autoimmune hemolytic anemia?

A

the IgGs coat the RBCs, with or without C3…they then signal macrophages to eat because of macros binding the Fc receptor of the IgG…leads to spherocytes in blood

37
Q

how to acquire warm antibody hemolytic anemia?

A

drugs…other autoimmune diseases like lupus…malignancy

38
Q

clinical features of warm antibody hemolytic anemia?

A

splenomegaly, can have severe anemia, jaundice, positive coombs test and spherocytes

39
Q

warm antibody hemolytic anemia treatments

A

corticosteroids first then splenectomy or rituximab if steroids dont work

40
Q

process of disease in cold antibody hemolytic anemia

A

IgM will bind RBCs in extremities and then fix complement…as RBCs move to internal organs the IgMs fall off but complement still fixed…complement can then signal for direct lysis in blood or go to liver for macrophage to destroy

so have both intravascular and extrvascular hemolysis

41
Q

IgM agglutination in cold antibody hemolytic anemia can lead to what?

A

cyanosis and ischemia in extremities because blockage by agglutination

42
Q

two common causes of IgM mediated cold antibody hemolytic anemia?

A

infection with mycoplasma or mononuclesosis

lymphoproliferative disease called Waldenstroms macroglobulinemia

43
Q

treatment for cold antibody hemolytic anemia

A

steroids and splenectomy do not work

keep patient warm and may need rituximab

44
Q

microangiopathic hemolytic anemia mechanism and marker in smear

A

shear damage to RBCs as a result of endothelial cell activation

schistocytes in smear

45
Q

microangiopathic hemolytic anemia cause

A

TTP/HUS and DIC

Heme Midterm 1 (11 decks)

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