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Step 2 question review > Heme/Onc SBSI > Flashcards

Heme/Onc SBSI Flashcards

1
Q

complications of hemophilia

A

arthropathy, spontaneous intracerebral, renal, retroperitoneal, and GI hemorrhages

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2
Q

tx of severe hemophilia

A

immediate transfusion of missing factor or cryoprecipitate

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3
Q

initial test for hemophilia

A

mixing study, PTT corrects after mixing

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4
Q

what is prolonged in hemophilia?

A

PTT

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5
Q

what does cryoprecipitate consist of?

A

mainly Factor VIII and fibrinogen

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6
Q

tx of mild hemophilia

A

desmopressin, releases factor VIII from endothelial cells

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7
Q

MC inherited bleeding disorder?

A

von willebrand disease

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8
Q

child with prolonged or recurrent mucosal bleeding and bleeding after dental or sx (epistaxis or menorrhagia)

positive family hx

A

von willebrand disease

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9
Q

initial test for von willebrand disease

A

increased BT and increased PTT

PT and plts are normal

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10
Q

most accurate test for vWD

A

ristocetin cofactor assay of pt plasma

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11
Q

mild to moderate vWD tx

A

desmopressin

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12
Q

what will worsen vWD?

A

ASA, NSAIDs, plt function inhibitors

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13
Q

acute neutropenia, what bugs are more likely?

A

S. aureus, Pseudomonas, E. coli, proteus, and klebsiella

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14
Q

best initial test for neutropenia

A

CBC with smear

after looking at drugs they are on

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15
Q

if decreased platelets and anemia with neutropenia, what should you get

A

bone marrow biopsy and/or aspirate

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16
Q

neutropenic fever, tx?

A

emergency

broad spec abx, cefepime

tx suspected fungal as well

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17
Q

what can be given to shorten duration of neutropenia?

A

G-CSF

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18
Q

What cytokines overproduced can cause eosinophilia?

A

IL-3, IL-5, GM-CSF

or by chemokines

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19
Q

dx with eosinophilia starts with

A

CBC with diff

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20
Q

CSF showing eosinophilia is suggestive of

A

drug reaction or infxn with coccidio or helminth

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21
Q

hematuria with eosinophilia

A

schistosomiasis

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22
Q

tx of new onset cardiac findings, eosinophilia or drug rxn

A

steroids

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23
Q

transplantation between identical twins

A

syngenic transplantation

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24
Q

complication of allogenic bone marrow transplantation in which donated T cells attack host tissues, especially skin, liver, and GI tract

A

graft v host

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25
Q

skin changes, cholestatic liver dysfunction, obstructive lung disease, or GI problems after transplant

A

graft v host

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26
Q

transplantation, 5 days to 3 months, increase GGT, alk phos, LDH, BUN, or Cr

A

acute rejection

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27
Q

vascular thrombi or tissue ischemia after transplant

A

hyperacute rejection

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28
Q

young, white pt, <45 yo with personal and family hx of thrombosis

A

Factor V Leiden

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29
Q

hyperhomocysteinemia

A

MTHFR gene mutation

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30
Q

test for factor V Leiden

A

activated protein C resistance test

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31
Q

test for HIT

A

platelet factor 4 antibody or serotonin release assay

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32
Q

tx for factor V Leiden

A

warfarin for 6 months, avoid OCPs

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33
Q

tx of HIT

A

d/c heparin. start direct thrombin inhibitor (fondaparinux, argatroban, bivalirudin)

followed by warfarin

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34
Q

tx antiphospholipid antibody syndrome

A

give heparin and warfarin

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35
Q

what is contraindicated in ITP and TTP

A

platelets

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36
Q

deficiency of vWF-cleaving enzyme (ADAMTS-13)

A

TTP

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37
Q

suspect TTP if 3/5 of these are present

A

Fever

Anemia

thrombocytopenia

renal issue

neuro symptoms

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38
Q

tx for TTP

A

plasma exchange

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39
Q

lab major difference TTP and HUS

A

HUS has much higher Cr

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40
Q

IgG antibodies formed against platelets, and then complex is destroyed by the spleen

A

ITP

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41
Q

abrupt onset of hemorrhagic complications following a viral illness with sudden, self-limiting purpura

A

ITP

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42
Q

tx of ITP, plts >30k and no bleeding

A

no tx

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43
Q

tx ITP, plt <30k or clinically significant bleeding

A

corticosteroids or IVIG

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44
Q

tx ITP with failed prior tx

A

splenectomy + rituximab + TPO receptor agonist

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45
Q

bone marrow production of platelets is increased with increased megakaryocytes in the marrow

A

ITP

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46
Q

glossitis, conjunctival pallor, cheilosis, koilonychia

A

fe deficiency anemia

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47
Q

initial test of Fe deficiency anemia will show

A

decreased MCV, decreased MCH, decreased MCHC

decreased ferritin

increased RDW

increased TIBC

decreased serum Fe

48
Q

tx of Fe deficiency anemia

A

replace Fe orally until normal and for at least 4-6 months

IM Fe if oral not working

IV Fe for people with malabsorption issues

49
Q

what will be labs in anemia of chronic disease?

A

ferritin increased

decreased serum Fe

decreased TIBC

50
Q

tx of anemia associated with ESRD

A

epo

51
Q

hx of chloramphenicol, INH, alcohol use, exposure to lead, collagen vascular disease and some myelodysplastic syndromes can cause

A

sideroblastic anemia

52
Q

tx of sideroblastic anemia?

A

remove offending agent, transfuse as necessary, may also consider pyroxidine

53
Q

neruopathy and subacute combined degeneration of the spinal cord

A

vit B12 deficiency

54
Q

inability to generate glutathione reductase

A

G6PD deficiency

55
Q

CD55 and 59 inhibited attachment

A

PNH

56
Q

tx for PNH

A

prednisone is best initial therapy

allogenic bone marrow transplant is curative

eculizumab for hemolysis and thrombosis

57
Q

extravascular hemolytic anemia with splenomegaly and jaundice

A

hereditary spherocytosis

58
Q

best test for spherocytosis

A

eosin- 5- maleimide flow cytometry

or acidified glycerol lysis test

59
Q

tx for hereditary spherocytosis

A

splenectomy and chronic folic acid replacement

60
Q

pancytopenia on labs, pallor, fatigue, weakness, tendency to infxn, petechiae, bruising and bleeding

A

aplastic anemia

61
Q

drugs that can cause aplastic anemia

A

PTU, methimazole, carbamazepine

62
Q

only form of hereditary aplastic anemia

A

Fanconi anemia

63
Q

severe case of aplastic anemia but can’t do stem cell transplant, tx?

A

cyclosporine (or tacrolimus), antithymocyte globulin (ATG), eltrombopag

64
Q

PCV can convert to

A

AML in small portion of pts

65
Q

why would PCV cause pruritis after hot bath?

A

increased histamine release

66
Q

what happens to EPO in PCV?

A

EPO is decreased

67
Q

tx for polycythemias

A

phlebotomy

ASA if they have thrombosis

allopurinol/rasburicase prevents an increase in uric acid

hydroxyurea reduces cell counts

68
Q

antibody response against donor plasma proteins causing urticaria, pruritis, wheezing, fever

A

allergic reaction

69
Q

cytokine formation during storage of blood

host antibodies against the donor HLA antigens and WBCs

Type II hypersensitivity reaction

A

febrile nonhemolytic reaction

70
Q

fever, HA, chills, flushing, rigors, and malaise 1-6 hours after transfusion

A

febrile nonhemolytic reaction

71
Q

tx of febrile nonhemolytic transfusion reaction

A

stop transfusion, give acetaminophen

leukoreduction of donor blood

72
Q

DIC picture s/p transfusion

A

hemolytic transfusion reaction

type II hypersensitivity

73
Q

AML and ALL will have signs of

A

pancytopenia and RAPID onset and progression

74
Q

DIC can be seen in what leukemia?

A

APML

75
Q

Auer rods will be found in

A

AML

76
Q

WBC is typically low in AML/ALL, if it very high, this is a risk of

A

leukostasis -> DIC

77
Q

tx of AML or ALL

A

chemo, or BMT if not responsive

78
Q

how might you prevent Tumor Lysis syndrome

A

hydration and rasburicase to decrease uric acid level as renal protection

79
Q

MC childhood malignancy

A

ALL

80
Q

Down syndrome is associated with what leukemia?

A

ALL

81
Q

t(15:17)

A

APML

82
Q

tx for APL

A

all trans retinoic acid

83
Q

what do you use to reverse factor Xa inhibitors?

A

andexanet alfa

84
Q

reverse warfarin with

A

activated prothrombin complex concentrate

85
Q

how do you tx tPa toxicity

A

aminocaproic acid

86
Q

malignant clonal proliferation of functionally incompetent lymphocytes

A

CLL

87
Q

cells that have a large nucleus, immature chromatin, a prominent nucleolus, scant cytoplasm and few or no cytoplasmic granules is

A

a blast cell

seen in acute leukemias

88
Q

blast cell that is myeloperoxidase positive

A

myeloblast

89
Q

Terminal deoxynucleotidyltransferase (TdT) positive

A

lymphoblast

90
Q

antidote/reversal to dabigatran

A

idarucizumab

91
Q

malignancies that go with tuberous sclerosis

A

astrocytoma and cardiac rhabdomyoma

92
Q

clonal proliferation of functionally incompetent lymphocytes that accumulate in the BM, peripheral blood, LN, spleen, and liver

A

CLL

93
Q

fatigue, malaise, infxn, LAD, hepatomegaly, and splenomegaly in an older individual

A

CLL

94
Q

initial tx of CLL

A

fludarabine and chlormabucil

palliative and withheld until symptomatic

95
Q

flow cytometry in CLL shows

A

CD5 marker on B cells

96
Q

splenomegaly, LUQ pain, early satiety, fatigue, malaise

A

CML

97
Q

basophilia

A

CML

98
Q

labs elevated in CML

A

WBC >100k, granulocytes in all stages of maturity

LDH, uric acid, and B12

99
Q

CML chromosome abnl

A

philadelphia chr. t(9:22)

100
Q

tx of CML

A

tyrosine kinase inhibitors (imatinib)

101
Q

tx of blast crisis in CML

A

2nd gen tyrosine kinase inhibitors

(dasatinib, nilotinib)

and hemopoietic stem cell transplantation

102
Q

well differentiated B lymphocyte disorder

A

hairy cell leukemia

103
Q

initial tx of hairy cell leukemia

A

cladribine

104
Q

labs of tumor lysis syndrome

A

hyperK, hyperuricemia, hyperphosphatemia, hypocalcemia

105
Q

painless wax and wane of adenopathy

A

follicular lymphoma (B cell)

106
Q

MC NHL in adults, single rapidly growing mass, high cure rate with R CHOP

A

diffuse large B cell lymphoma

107
Q

elderly male, CD5+ cells, NHL

A

mantle cell lymphoma

108
Q

high grade neoplasm that can progress to ALL, cutaneous lesions

caused by HTLV and a/w IVDA

A

Adult T cell lymphoma

109
Q

cutaneous eczema like lesions and pruritis with ‘cerebriform’ lymphoid cellsmy

A

mycosis fungioides and can lead to Sezary syndrome

110
Q

pt has long standing inflammatory disease/ or infection and is presenting with kidney, liver, and GI involvement

A

amyloidosis

111
Q

if amyloidosis progresses rapidly, tx with

A

corticosteroids, melphalan

112
Q

chemo type when standard chemo fails

A

salvage

113
Q

chemo type that is given in addition to standard therapy

A

adjuvant

114
Q

chemo type that is given before the standard therapy

A

neoadjuvant

115
Q

chemo type that is the initial dose of treatment

A

induction

116
Q

type of chemo given after induction therapy to reduce the tumor burden

A

consolidation

117
Q

type of chemo you give after induction and consolidation

A

maintenance

Step 2 question review (9 decks)

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