Perrotta Flashcards

(56 cards)

1
Q

diseases associated with ovalocytes

A

hereditary ovalocytosis

iron deficiency

thalassemia

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2
Q

diseases associated with spherocytes

A

hereditary spherocytosis

WAHA

cold agglutinate disease

paroxysmal cold hemoglobinuria

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3
Q

pelger huet cells associated w/

A

myelodysplastic syndromes

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4
Q

erythropoiesis occurs in what organs

A

yolk sac

liver

spleen

bone marrow

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5
Q

extramedullar hematopoiesis organs

A

spelen

liver

lymph nodes

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6
Q

heme is made up of

A

1 Fe2+ and porphyrin ring

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7
Q

low to normal MCV with high MCHC

A

hereditary spherocytosis

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8
Q

defects in alpha spectrin cause

A

hereditary elliptocytosis

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9
Q

severe form of hereditary elliptocytosis is called

A

pyropoikilocytosis

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10
Q

defect in ankyrin

A

hereditary spherocytosis

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11
Q

target cells are present in

A

Hb SC

thalassemia (esp beta)

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12
Q

increased RDW (anisocytosis)

A

iron def anemias

macrocytic anemias

hemolytic anemias

(most anemias)

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13
Q

most common mutation in beta thal

A

point mutation

  • splicing mutation for beta+
  • chain terminator mutations for beta 0
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14
Q

Cooley’s anemia same as

A

beta thal major

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15
Q

crewcut appearance

A

beta thal major

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16
Q

tx for beta thal major

A

blood transfusions

BMT

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17
Q

dx PNH

A

flow cytometry

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18
Q

treat PNH

A

BMT

growth factors

anti-complement Ab (eculizumab)

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19
Q

treat WAHA

A

1st line: steroids

splenectomy

Rituximab

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20
Q

mechanism of alpha methyldopa causing WAHA

A

autoantibody (IgG)

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21
Q

mechanism of penicillin causing WAHA

A

IgG against drug/membrane complex “happen” mechanism

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22
Q

mechanism of cephalosporins causing WAHA

A

IgG against drug/membrane complex “happen” mechanism

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23
Q

mechanism of quinidine causing WAHA

A

“immune complex” / “ternary complex” IgM formed

24
Q

drug related WAHA: positive for

25
what test for paroxysmal cold hemoglobinuria
Donath-Landsteiner test (IgG anti- P Ab binds at low temp and complement lyses at higher temp)
26
causes of ineffective erythropoiesis
B12 and folate deficiency thalassemia myelodysplastic syndrome iron overload
27
pernicious anemia has increased risk of what cancer
gastric cancer (so do endoscopy)
28
what disease causes degeneration of myelin in dorsal and lateral tracts "subacute combined degeneration"
pernicious anemia
29
test to distinguish pernicious anemia vs other causes of B12 deficiency
Schilling test
30
after start rx for folate deficiency, should see what in blood
reticulocytosis
31
TIBC equation
TIBC = transferrin x 1.4
32
transferrin saturation equation
transferrin saturation = Fe / TIBC
33
acute phase reactant
ferritin
34
2 effects of increased hepcidin
decreased iron form macrophages to erythroid precursors suppress EPO
35
what is Fanconi's anemia
rare autosomal recessive disorder in proteins required for DNA repair
36
pathophys of aplastic anemia
damaged stem cell expresses a new antigen, which causes T cell to attack it damaged stem cell can't proliferate normally
37
treatment for aplastic anemia (child and adult)
child: BMT adult: antithymocyte globulin and cyclosporine
38
pure red cell aplasia can be secondary to
parvovirus B19 thymoma
39
chronic kidney failure causes what type of anemia
normocytic, normochromic
40
most common infectious cause of acute chest syndrome in sickle cell disease
Chlamydia pneumoniae
41
best screening test for pulmonary artery hypertension in SCD
echo for tricuspid regurg jet velocity
42
treatment goal of stroke in SCD
reduce HbS percentage
43
biggest indication for chronic transfusion in SCD
stroke prevention
44
autoantibody that recognizes platelet surface glycoproteins (GPIIb/IIa, etc) and platelets removed by macrophages
immune thrombocytopenic purpura
45
first line treatment for chronic ITP
corticosteroids IVIG WinRho
46
HPA-1a antibody
neonatal alloimmune thrombocytopenia
47
treatment for NAT
IVIG maybe corticosteroids maybe plt transfusion from mom
48
tx for HIT
direct thrombin inhibitors
49
serotonin release assay tests for
HIT
50
reversal of coumadin
fresh frozen plasma
51
use fresh frozen plasma for
reversal of coumadin liver disease DIC massive transfusion TTP
52
liver disease causes decreased synthesis of
vit K cep factors (2,7,9,10, C, S) antiplasmin (so increased fibrinolysis)
53
treatments for vWD
DDAVP vWF anti fibrinolytics
54
cryoprecipitate is used in
hypofibrinogenemia uremic bleeding
55
what is used to guide FFP transfusion
PT and PTT
56
transfusion related graft vs host disease is mediated by which lymphocyte
T lymphocyte