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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
secondary to EMV, HIV, EBV, mycoplasma
CAIHA
25
caused by malnutrition or antibiotics
vitamin K deficiency (bleeding)
26
aspirin, NSAIDS, ADPris, EtOH cause
poisoned platelets (won't stick: bleeding)
27
manage with washed RBCs if severe, prophylax with antihistamine
mild urticaria
28
minimize with leukodepleted products
HLA sensitization
29
hepatic + K
2,7,9,10, C,S
30
hepatic, no K
11, antithrombin
31
source: endothelial cells
vWF, 8
32
measure intrinsic pathway
aPTT
33
measure extrinsic pathway
PT
34
normal aPTT, PT
platelet disorders, 13 deficiency, hyperfibrinolysis
35
increases aPTT
intrinsic: hemophilia, VW, Heparin, Antiphospholipid Ab (Lupus anticoag)
36
increases PT
extrinsic: vitamin K deficiency, warfarin
37
increases aPTT and PT
DIC, tons of warfarin, rare common pathway deficiency
38
transports iron
transferritin | ferritin stores iron in heart and liver
39
iron is only excreted by
desquamination of epithelium
40
spoon nails, blue sclera, esophageal web, chlorosis
iron deficiency!
41
looks like iron deficient anemia, but iron does not help
thalassemia
42
vertigo, hair loss, memory loss, tiny little testicles
hemachromotosis
43
genetic defect in hepcidin causes
hemachromotosis
44
increased hepcidin
caused by inflammation, causes iron sequestration and anemia of chronic disease
45
uncommon, idiopathic, Hep C, autoimmune and lymphoma
WAIHA
46
rare, not idiopathic, mycoplasma, EBV/CMV mono, lymphoma
CAIHA
47
infection, fava beans, DM ketoacidosis, anti-malarials, sulfas, aspirin, methylene blue
G6PD deficiency
48
Fever Anemia Thrombocytopenia Renal failure Neuro dysfunction
TTP
49
LDH, reticulocytes, haptoglobin
dx hemolysis
50
d-dimer elevation
fibrinolysis, meaning excess coagulation or renal failure
51
howell-jolie bodies
in SC, e.g., mean absent spleen function
52
encapsulated bacteria that are happy when you don't have a spleen
s. pneumoniae, h. flu, n. meningitidis, salmonella
53
increase fetal hemoglobin, decrease neutrophils and adhesion molecules, macrocytosis and increased hydration, NO production
hydroxyurea effects (SC)
54
MBA, glossitis/stomatitis, rare GI malabsorption secondary to impaired epithelium
folate deficiency
55
required for folate metabolism, succinyl CoA production, mylein methylation
B12
56
why not treat MBA with folate only?
B12 deficiency will trash you neurologically!
57
binds collagen and platelets and factor 8
VWf
58
Increased PT, aPTT and d-dimer decreased platelets and fibrinogen +/- schistocytes
DIC
59
inflammation, nephrotic syndrome, DIC, TTP, PNH, DM
some hypercoaguable states
60
inflammation leads to increased IL1 and TNF, which lead to
decreased thrombomodulin, | so thrombin is procoagulant instead of anti
61
extrinsic pathway
T, 7, 10, 5, 2, 1
62
intrinsic pathway
11, 9, 8, 10, 5, 2, 1
63
thrombin activates
5, 8, 11, 13
64
antithrombin prevents activation of
10, 2
65
TFPI prevents activation of
7, 10
66
C and S prevent activation of
8, 5
67
XLR (4)
G6PD d, Hemophilia A and B, agammaglobinemia
68
XLR/AR (2)
chronic granulomatosis, SCIDS
69
AR (2)
Chediak-Higashi | Hemophilia C
70
AD (6)
VW, hemochromatosis, elliptocytosis, spherocytosis, pelger-huet, may-hegglin
71
transfusion, chelation and bone marrow transplant
thalassemia, sickle cell
72
transfusion, iron and folate, anti-coagulants and eculizamab
PNH
73
transfusions, folate, splenectomy
sphero, elliptocytosis
74
no transfusions, unless angina or hemolytic instability
megaloblastic anemia
75
B12 + folate
megaloblastic anemia
76
Iron + C
Fe deficient anemia
77
prophylactic factor replacement
hemophilia A & B
78
fresh frozen plasma
hemophilia C
79
plasma exchange, steroids, rituximab, splenectomy
TTP
80
plasma exchange, rituximab
CAIHA
81
steroids, rituximab, splenectomy, IV Ig, anti-D, thrombopoetin mimics
ITP
82
steroids, rituximab, splenectomy, anticoagulation
WAIHA
83
cryoprecipitate, heparin
DIC
84
cryoprecpitate, aminocap, humate-P, desmopression
VW
85
desmopressin, dialysis
uremic platelet dysfunction
86
phlebotomy, chelation, liver transplant
hemochromatosis
87
supportive therapy only
G6DP deficiency

Pulm & Heme (6 decks)

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