Heme/Onc SBSI Flashcards

(117 cards)

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
skin changes, cholestatic liver dysfunction, obstructive lung disease, or GI problems after transplant
graft v host
26
transplantation, 5 days to 3 months, increase GGT, alk phos, LDH, BUN, or Cr
acute rejection
27
vascular thrombi or tissue ischemia after transplant
hyperacute rejection
28
young, white pt, \<45 yo with personal and family hx of thrombosis
Factor V Leiden
29
hyperhomocysteinemia
MTHFR gene mutation
30
test for factor V Leiden
activated protein C resistance test
31
test for HIT
platelet factor 4 antibody or serotonin release assay
32
tx for factor V Leiden
warfarin for 6 months, avoid OCPs
33
tx of HIT
d/c heparin. start direct thrombin inhibitor (fondaparinux, argatroban, bivalirudin) followed by warfarin
34
tx antiphospholipid antibody syndrome
give heparin and warfarin
35
what is contraindicated in ITP and TTP
platelets
36
deficiency of vWF-cleaving enzyme (ADAMTS-13)
TTP
37
suspect TTP if 3/5 of these are present
Fever Anemia thrombocytopenia renal issue neuro symptoms
38
tx for TTP
plasma exchange
39
lab major difference TTP and HUS
HUS has much higher Cr
40
IgG antibodies formed against platelets, and then complex is destroyed by the spleen
ITP
41
abrupt onset of hemorrhagic complications following a viral illness with sudden, self-limiting purpura
ITP
42
tx of ITP, plts \>30k and no bleeding
no tx
43
tx ITP, plt \<30k or clinically significant bleeding
corticosteroids or IVIG
44
tx ITP with failed prior tx
splenectomy + rituximab + TPO receptor agonist
45
bone marrow production of platelets is increased with increased megakaryocytes in the marrow
ITP
46
glossitis, conjunctival pallor, cheilosis, koilonychia
fe deficiency anemia
47
initial test of Fe deficiency anemia will show
decreased MCV, decreased MCH, decreased MCHC decreased ferritin increased RDW increased TIBC decreased serum Fe
48
tx of Fe deficiency anemia
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
what will be labs in anemia of chronic disease?
ferritin increased decreased serum Fe decreased TIBC
50
tx of anemia associated with ESRD
epo
51
hx of chloramphenicol, INH, alcohol use, exposure to lead, collagen vascular disease and some myelodysplastic syndromes can cause
sideroblastic anemia
52
tx of sideroblastic anemia?
remove offending agent, transfuse as necessary, may also consider pyroxidine
53
neruopathy and subacute combined degeneration of the spinal cord
vit B12 deficiency
54
inability to generate glutathione reductase
G6PD deficiency
55
CD55 and 59 inhibited attachment
PNH
56
tx for PNH
prednisone is best initial therapy allogenic bone marrow transplant is curative eculizumab for hemolysis and thrombosis
57
extravascular hemolytic anemia with splenomegaly and jaundice
hereditary spherocytosis
58
best test for spherocytosis
eosin- 5- maleimide flow cytometry or acidified glycerol lysis test
59
tx for hereditary spherocytosis
splenectomy and chronic folic acid replacement
60
pancytopenia on labs, pallor, fatigue, weakness, tendency to infxn, petechiae, bruising and bleeding
aplastic anemia
61
drugs that can cause aplastic anemia
PTU, methimazole, carbamazepine
62
only form of hereditary aplastic anemia
Fanconi anemia
63
severe case of aplastic anemia but can't do stem cell transplant, tx?
cyclosporine (or tacrolimus), antithymocyte globulin (ATG), eltrombopag
64
PCV can convert to
AML in small portion of pts
65
why would PCV cause pruritis after hot bath?
increased histamine release
66
what happens to EPO in PCV?
EPO is decreased
67
tx for polycythemias
phlebotomy ASA if they have thrombosis allopurinol/rasburicase prevents an increase in uric acid hydroxyurea reduces cell counts
68
antibody response against donor plasma proteins causing urticaria, pruritis, wheezing, fever
allergic reaction
69
cytokine formation during storage of blood host antibodies against the donor HLA antigens and WBCs Type II hypersensitivity reaction
febrile nonhemolytic reaction
70
fever, HA, chills, flushing, rigors, and malaise 1-6 hours after transfusion
febrile nonhemolytic reaction
71
tx of febrile nonhemolytic transfusion reaction
stop transfusion, give acetaminophen leukoreduction of donor blood
72
DIC picture s/p transfusion
hemolytic transfusion reaction type II hypersensitivity
73
AML and ALL will have signs of
pancytopenia and RAPID onset and progression
74
DIC can be seen in what leukemia?
APML
75
Auer rods will be found in
AML
76
WBC is typically low in AML/ALL, if it very high, this is a risk of
leukostasis -\> DIC
77
tx of AML or ALL
chemo, or BMT if not responsive
78
how might you prevent Tumor Lysis syndrome
hydration and rasburicase to decrease uric acid level as renal protection
79
MC childhood malignancy
ALL
80
Down syndrome is associated with what leukemia?
ALL
81
t(15:17)
APML
82
tx for APL
all trans retinoic acid
83
what do you use to reverse factor Xa inhibitors?
andexanet alfa
84
reverse warfarin with
activated prothrombin complex concentrate
85
how do you tx tPa toxicity
aminocaproic acid
86
malignant clonal proliferation of functionally incompetent lymphocytes
CLL
87
cells that have a large nucleus, immature chromatin, a prominent nucleolus, scant cytoplasm and few or no cytoplasmic granules is
a blast cell seen in acute leukemias
88
blast cell that is myeloperoxidase positive
myeloblast
89
Terminal deoxynucleotidyltransferase (TdT) positive
lymphoblast
90
antidote/reversal to dabigatran
idarucizumab
91
malignancies that go with tuberous sclerosis
astrocytoma and cardiac rhabdomyoma
92
clonal proliferation of functionally incompetent lymphocytes that accumulate in the BM, peripheral blood, LN, spleen, and liver
CLL
93
fatigue, malaise, infxn, LAD, hepatomegaly, and splenomegaly in an older individual
CLL
94
initial tx of CLL
fludarabine and chlormabucil palliative and withheld until symptomatic
95
flow cytometry in CLL shows
CD5 marker on B cells
96
splenomegaly, LUQ pain, early satiety, fatigue, malaise
CML
97
basophilia
CML
98
labs elevated in CML
WBC \>100k, granulocytes in all stages of maturity LDH, uric acid, and B12
99
CML chromosome abnl
philadelphia chr. t(9:22)
100
tx of CML
tyrosine kinase inhibitors (imatinib)
101
tx of blast crisis in CML
2nd gen tyrosine kinase inhibitors (dasatinib, nilotinib) and hemopoietic stem cell transplantation
102
well differentiated B lymphocyte disorder
hairy cell leukemia
103
initial tx of hairy cell leukemia
cladribine
104
labs of tumor lysis syndrome
hyperK, hyperuricemia, hyperphosphatemia, hypocalcemia
105
painless wax and wane of adenopathy
follicular lymphoma (B cell)
106
MC NHL in adults, single rapidly growing mass, high cure rate with R CHOP
diffuse large B cell lymphoma
107
elderly male, CD5+ cells, NHL
mantle cell lymphoma
108
high grade neoplasm that can progress to ALL, cutaneous lesions caused by HTLV and a/w IVDA
Adult T cell lymphoma
109
cutaneous eczema like lesions and pruritis with 'cerebriform' lymphoid cellsmy
mycosis fungioides and can lead to Sezary syndrome
110
pt has long standing inflammatory disease/ or infection and is presenting with kidney, liver, and GI involvement
amyloidosis
111
if amyloidosis progresses rapidly, tx with
corticosteroids, melphalan
112
chemo type when standard chemo fails
salvage
113
chemo type that is given in addition to standard therapy
adjuvant
114
chemo type that is given before the standard therapy
neoadjuvant
115
chemo type that is the initial dose of treatment
induction
116
type of chemo given after induction therapy to reduce the tumor burden
consolidation
117
type of chemo you give after induction and consolidation
maintenance