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HEME/LYMPH Exam 2 > RBC and Bleeding Disorders IV > Flashcards

RBC and Bleeding Disorders IV Flashcards

1
Q

PIGA mutation

A

in paroxysmal nocturnal hemoglobinuria

phosphatidylinositol glycan complementation group A gene

x-linked

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2
Q

paroxysmal nocturnal hemoglobinuria

A

mutation in PIGA
-rare

only hemolytic anemia caused by acquired genetic defect**

RBCs deficient in three GPI-linked proteins that regulate complement activity
-susceptible to lysis - intravascular hemolysis (MAC complex)

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3
Q

PNH at night

A

because blood pH slight decrease - increased activity of complement

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4
Q

death in PNH

A

venous thrombosis

-of hepatic, portal, or cerebral veins

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5
Q

progression of PNH

A

may go to AML or MDS

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6
Q

diagnosis of PNH

A

flow cytometry

-detects RBCs deficienct in CD59 and CD55

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7
Q

CD59

A

deficient in PNH

-detected with flow cytometry

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8
Q

tx of PNH

A

monoclonal antibody

  • eculizumab
  • inhibits conversion of C5
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9
Q

immunohemolytic anemias

A

Abs bind RBCs leading to premature destruction

aka autoimmune hemolytic anemias

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10
Q

warm Ab type

A

IgG antibodies active at 37C

primary
secondary, SLE, drugs, neoplasms

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11
Q

cold agglutinin type

A

IgM antibodies active below 37C

acute - mycoplasm infection, mono

chronic - idiopathic, neoplasms

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12
Q

cold hemolysin type

A

IgG antibodies active below 37C

rare - children with viral infections

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13
Q

children with viral infections

A

cold hemolysin type

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14
Q

mycoplasm and mono infection

A

cold agglutinin type

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15
Q

PCN and cephalosporin

A

drug induced warm-antibody type

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16
Q

alpha-methyldopa

A

telomerase breaking drug

lead to warm -AB type

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17
Q

paroxysmal cold hemoglobinuria

A

cold hemolysin type

IgG bunds P blood group antigens

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18
Q

mycoplasma pneumonia

A

cold agglutinin type of immunohemolytic anemias

IgM Abs bind RBC at low temperatures

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19
Q

direct coombs antiglobulin test

A

patient RBCs mixed with sera with Abs for Ig/complement

clumping

20
Q

indirect coombs antiglobulin test

A

patients serum tested for ability to agglutinate commercial RBCs

test used to characterize antigen target and temperature dependence of responsible antibody

quantitative test**

21
Q

to characterize antigen target and temperature dependence of responsible antibody

A

indirect coombs test

22
Q

cardiac valve prosthesis

A

hemolytic anemia from RBC trauma

23
Q

microangiopathic hemolytic anemia

A

seen with DIC, TTP, HUS, malignant HTN, and SLE, and disseminated cancers

microvascular lesion - luminal narrowing

deposition of fibrin and platelets

shear stress

24
Q

schistocytes, burr cells, helmet cells, triangle cells

A

in PBS with traumatic hemolytic anemia

25
Q

anemias with diminished erythropoiesis

A

nutritional deficiency, renal failure, chronic inflammation

less commonly bone marrow failure, aplastic anemia, neoplasm

26
Q

extrinsic causes of diminished erythropoiesis

A

more common and clinically important

nutritional deficiencies

27
Q

animal products

A

B12 higher

28
Q

B12 and folic acid

A

coenzymes for synthesis of thymidine - DNA base

deficiency - megaloblastic anemia

29
Q

intrinsic factor

A

binds B12 in stomach

30
Q

haptocorrin

A

binds B12 in saliva

31
Q

megaloblastic

A

impaired DNA synthesis

32
Q

pernicious anemia

A

decreased B12

-autoimmune gastritis impairs IF production

33
Q

cobalamin

A

B12

34
Q

cubillin

A

receptor for IF:B12 aborption in ileum

35
Q

hypersegmented PMNs

A

in megaloblastic anemia

also see giant metamyelocytes and band forms

36
Q

parietal cells

A

produce intrinsic factor

37
Q

spastic paresis, sensory ataxia, severe parathesias of lower limbs

A

neuro complications of B12 deficiency

38
Q

autoimmune gastritis impairing production of IG

A

pernicious anemia

39
Q

pernicious anemia clinical

A

scandinavian and other caucasian populations

disease of older adults

rare younger than 30

40
Q

dx of pernicious anemia

A

1 moderate megaloblastic anemia

2 leukopenia with hypersegmented granulocytes

3 low serum B12

4 elevated homocysteine and methylmalonic acid

41
Q

after tx with B12 in pernicious anemia

A

reticulocytosis and rising Hct 5 days after tx

42
Q

pernicious anemia pathology

A

autoantibodies diagnostic utility - not primary cause

thought to be an autoreactive T-cell response*** - initiates gastric mucosal injury

43
Q

type I pernicious anemia

A

block B12-IF binding

44
Q

type II pernicious anemia

A

block IF:B12 to ileal receptor binding

45
Q

type III pernicious anemia

A

85-90%

recognize alpha and beta subunits of gastric H+ pumps

HEME/LYMPH Exam 2 (16 decks)

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