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Pulm & Heme > Heme Extras > Flashcards

Heme Extras Flashcards

0
Q

albanism, photophobia, lysosome dysfunction

A

chediak-higashi

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1
Q

large platelets, seen in May Heggin

A

macrothrombocytopenia

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2
Q

LDH

A

enzyme released in tissue damage, high in TTP

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3
Q

gallstones (cholelithiasis) seen in

A

spherical and elliptical RBC syndromes

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4
Q

Heinz bodies

A

round inclusions of denatured hemoglobin in RBCs

G6PD deficiency

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5
Q

misshaped hemoglobin due to beta-delta crossover

A

Hb lepore (unstable)

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6
Q

lysis in liver or spleen

A

extravascular hemolysis. intra is complement or mechanical

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7
Q

regulates complement system, low level means RBC lysis

A

CD55/59 (PIGA mutation means PNH)

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8
Q

complement-inhibiting Ab, treats PHN

A

ecuzimab

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9
Q

phototherapy

A

oxidizes bilrubin, prevent kernicterus in HDN

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10
Q

CD20 Ab, gets rid of B cells, treats TTP, ITP, C/WAIHA

A

Rituximab

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11
Q

Donath-Landstiener +

A

PCH

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12
Q

complexes w/5, 9, VWf for platelet adhesion

A

Gp1b

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13
Q

increases in inflammation, decreases S

A

Cb4, procoagulant

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14
Q

ADAM doesn’t chop up VWf

A

small clots form (TTP)

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15
Q

only sign is increased PT, appears during surgery

A

Hemophilia C

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16
Q

Comes with clotting and renal disease

A

PNH

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17
Q

huge platelets and petechiae

A

ITP

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18
Q

HIV, EBV/CMV, steroids, immunocompromise, chemo, hodgkins

A

lymphocytopenia

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19
Q

penicillin, EtOH, chemo, immunocompromise

A

med-induced thrombocytopenia

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20
Q

CVD, IBD, infection, cancer, thyroid disease, renal failure, familial mediterranean fever

A

anemia of chronic disease

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21
Q

rare cold Ig

A

PCH

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22
Q

PIGA mutation

A

PNH

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23
Q

false + RPR (syphilis)

A

Antiphospholipid Antibody Syndrome

aquired clotting disorder

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24
Q

secondary to EMV, HIV, EBV, mycoplasma

A

CAIHA

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25
Q

caused by malnutrition or antibiotics

A

vitamin K deficiency (bleeding)

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26
Q

aspirin, NSAIDS, ADPris, EtOH cause

A

poisoned platelets (won’t stick: bleeding)

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27
Q

manage with washed RBCs if severe, prophylax with antihistamine

A

mild urticaria

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28
Q

minimize with leukodepleted products

A

HLA sensitization

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29
Q

hepatic + K

A

2,7,9,10, C,S

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30
Q

hepatic, no K

A

11, antithrombin

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31
Q

source: endothelial cells

A

vWF, 8

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32
Q

measure intrinsic pathway

A

aPTT

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33
Q

measure extrinsic pathway

A

PT

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34
Q

normal aPTT, PT

A

platelet disorders, 13 deficiency, hyperfibrinolysis

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35
Q

increases aPTT

A

intrinsic: hemophilia, VW, Heparin, Antiphospholipid Ab (Lupus anticoag)

36
Q

increases PT

A

extrinsic: vitamin K deficiency, warfarin

37
Q

increases aPTT and PT

A

DIC, tons of warfarin, rare common pathway deficiency

38
Q

transports iron

A

transferritin

ferritin stores iron in heart and liver

39
Q

iron is only excreted by

A

desquamination of epithelium

40
Q

spoon nails, blue sclera, esophageal web, chlorosis

A

iron deficiency!

41
Q

looks like iron deficient anemia, but iron does not help

A

thalassemia

42
Q

vertigo, hair loss, memory loss, tiny little testicles

A

hemachromotosis

43
Q

genetic defect in hepcidin causes

A

hemachromotosis

44
Q

increased hepcidin

A

caused by inflammation, causes iron sequestration and anemia of chronic disease

45
Q

uncommon, idiopathic, Hep C, autoimmune and lymphoma

46
Q

rare, not idiopathic, mycoplasma, EBV/CMV mono, lymphoma

47
Q

infection, fava beans, DM ketoacidosis, anti-malarials, sulfas, aspirin, methylene blue

A

G6PD deficiency

48
Q

Fever Anemia Thrombocytopenia Renal failure Neuro dysfunction

49
Q

LDH, reticulocytes, haptoglobin

A

dx hemolysis

50
Q

d-dimer elevation

A

fibrinolysis, meaning excess coagulation or renal failure

51
Q

howell-jolie bodies

A

in SC, e.g., mean absent spleen function

52
Q

encapsulated bacteria that are happy when you don’t have a spleen

A

s. pneumoniae, h. flu, n. meningitidis, salmonella

53
Q

increase fetal hemoglobin, decrease neutrophils and adhesion molecules, macrocytosis and increased hydration, NO production

A

hydroxyurea effects (SC)

54
Q

MBA, glossitis/stomatitis, rare GI malabsorption secondary to impaired epithelium

A

folate deficiency

55
Q

required for folate metabolism, succinyl CoA production, mylein methylation

56
Q

why not treat MBA with folate only?

A

B12 deficiency will trash you neurologically!

57
Q

binds collagen and platelets and factor 8

58
Q

Increased PT, aPTT and d-dimer
decreased platelets and fibrinogen
+/- schistocytes

59
Q

inflammation, nephrotic syndrome, DIC, TTP, PNH, DM

A

some hypercoaguable states

60
Q

inflammation leads to increased IL1 and TNF, which lead to

A

decreased thrombomodulin,

so thrombin is procoagulant instead of anti

61
Q

extrinsic pathway

A

T, 7, 10, 5, 2, 1

62
Q

intrinsic pathway

A

11, 9, 8, 10, 5, 2, 1

63
Q

thrombin activates

A

5, 8, 11, 13

64
Q

antithrombin prevents activation of

65
Q

TFPI prevents activation of

66
Q

C and S prevent activation of

67
Q

XLR (4)

A

G6PD d, Hemophilia A and B, agammaglobinemia

68
Q

XLR/AR (2)

A

chronic granulomatosis, SCIDS

69
Q

AR (2)

A

Chediak-Higashi

Hemophilia C

70
Q

AD (6)

A

VW, hemochromatosis, elliptocytosis, spherocytosis, pelger-huet, may-hegglin

71
Q

transfusion, chelation and bone marrow transplant

A

thalassemia, sickle cell

72
Q

transfusion, iron and folate, anti-coagulants and eculizamab

73
Q

transfusions, folate, splenectomy

A

sphero, elliptocytosis

74
Q

no transfusions, unless angina or hemolytic instability

A

megaloblastic anemia

75
Q

B12 + folate

A

megaloblastic anemia

76
Q

Iron + C

A

Fe deficient anemia

77
Q

prophylactic factor replacement

A

hemophilia A & B

78
Q

fresh frozen plasma

A

hemophilia C

79
Q

plasma exchange, steroids, rituximab, splenectomy

80
Q

plasma exchange, rituximab

81
Q

steroids, rituximab, splenectomy, IV Ig, anti-D, thrombopoetin mimics

82
Q

steroids, rituximab, splenectomy, anticoagulation

83
Q

cryoprecipitate, heparin

84
Q

cryoprecpitate, aminocap, humate-P, desmopression

85
Q

desmopressin, dialysis

A

uremic platelet dysfunction

86
Q

phlebotomy, chelation, liver transplant

A

hemochromatosis

87
Q

supportive therapy only

A

G6DP deficiency

Pulm & Heme (6 decks)

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