Other Flashcards
1
Q
Management of acute GVHD affecting the GI tract
A
- nonabsorbable oral steroid (budesonide)
+ systemic glucocorticoid
2
Q
Management of erythrocytosis in patient with transplanted kidney
A
ACE inhibitor
3
Q
Most common mutations in CMML
A
- TET2
- SRSF2
- ASXL1
4
Q
CMML treatment
A
IF high risk, HMA bridge to transplant
IF low risk, observation
Once needing treatment HMA vs. hydrea
5
Q
Pathophysiology of cerebellar neurotoxicity with cytarabine
A
Widespread loss of purkinje fibers
6
Q
clinical benefit of early vs. delayed transplant in myeloma
A
PFS alone
7
Q
DOAC management perioperatively
A
- IF urgent surgery, reverse DOAC
- IF low/moderate bleeding risk, hold for one day before
- IF high bleeding risk, omit 2 days before
8
Q
Definition of GVHD overlap syndrome
A
simultaneous features of both chronic and acute GVHD
9
Q
Advanted of BuCy conditioning vs. BuFlu
A
- reduced time to t cell engraftment
10
Q
Burr cells on peripheral smear
A
see photo
11
Q
Burr cell differential
A
- ESRD
- Liver disease
- Pyruvate kinase deficiency
12
Q
PKD inheritance
A
autosomal recessive
13
Q
drug contrandicated with methotrexate
A
NSAIDS (reduce renal clearance)
14
Q
Site of hematopoiesis in gestation at 5/6 months
A
l*iver
bone marrow
15
Q
Peripheral smear of hereditary stomatocytosis
A
- see photo online
16
Q
Management of patient on ganciclovir for CMV and levels are uptrending
A
Continue (takes 1-2 weeks for levels to decline after starting treatment)
17
Q
Management of acquired VWF deficiency
A
recombinant VWF
18
Q
What is idecabtagene?
A
idecabtagene vicleucel (ide-cel)
19
Q
Least important antigen for hemolytic transfusion reaction
A
Lewis antigen
20
Q
Etiology of CLL patient treated with FCR and presenting with dry cough, fever, hypoxia
A
PJP oneumonia
21
Q
Other SE of IVIG to be aware of
A
aseptic meningitis
22
Q
What to do if you’ve caused hyperviscosity in LPL patient with rituximab
A
PLEX
23
Q
What to think with prolonged thrombin time
A
low fibrinogen
24
Q
Gaucher disease treatment
A
IF cytopenias or significant splenomegaly, enzyme replacement therapy
25
idelalisib mechanism
- inhibits phosphorylated MTOR
26
Letermovir SE's
- AKI
- pneumonia
27
Preferred systemic therapy for NLPHL
R-CHOP
28
Sweet syndrome clinical features
abrupt appearance of painful, edematous, and erythematous papules, plaques, or nodules on the skin + fever + neutrophilia
29
WHIM syndrome
Warts, Hypogammaglobulinemia, Infections, Myelokathexis
- severe neutropenia
30
Management of symptomatic gastric MALT that is negative for h pylori
rituximab
31
Management of cirrhosis from iron overload
liver transplantation
32
Management of hemolytic anemia post liver transplant
- transfuse donor type RBCs
33
Function of hepcidin
- degrades ferroportin
34
Next step if patient develops thrombocytopenia post ritiximab
- check EBV/HCV serologies
35
Second line for FL
- if early relapse/aggressive disease, CAR-T
- if late relapse and BR frontline, R-CHOP
36
Post transfusion purpura presentation
Severe thrombocytopenia after a platelet transfusion
37
Next steps if patient has detectable HPA-1a antibodies in the serum
- only transfuse HPA-1a negative RBCs
- *washed and resuspended RBCs (you need to remove any platelets that are HPA-1a positive and may have contaminated RBCs)
38
Preferred CAR-T products for ALL
IF age >25, Brexi-cell (Zuma-3)
IF age up to 25 years, tisa-cel (approval)
39
Other poor prognosticator in AML
- monosomal karyotype
40
Polymorphic PTLD management
- Single agent rituximab + reduction of immunosuppression
41
Monomorphic PTLD management
IF minimally symptomatic/more indolent lymphoma, single agent rituxan
IF DLBCL/more aggressive lymphatic, R-CHOP
42
Factor VII deficiency perioperative treatment for procedure with significant bleeding risk
recombinant activated factor VII (novoseven) 15-30 mcg/kg q12h
43
Factor XII deficiency coags
- prolonged apTT
44
Factor deficiencies with prolonged aPTT
8,9,11 and 12
45
prekallikrein and HMWK deficiency bleeding phenotype
No bleeding diasthesis,
just prolonged aPTT
46
Bleeding phenotype of hemophilia carriers
- can have bleeding, depends on level
47
peripartum management of hemophilia carrier
- depends on baseline factor level
- IF <50 need factor
48
Enzyme involved in Xa inhibitor metabolism
CYP3A4
49
Result of mixing study in VWD
corrects (it's factor VIII deficiency that causes the aPTT prolongation)
50
Other causes of protein s deficiency
- Liver disease (synthesized by hepatocytes) →
- Acute thrombosis →
- Pregnancy → nearly all pregnant people have decreased, physiologic protein s
- OCP’s/estrogen treatment
***Any comorbid illness that causes an acute phase response can lower the level of free protein S →
- DIC →
Anticoagulants: Warfarin, NOAC treatment →
*Nephrotic syndrome →
*HIV
51
Other causes of antithrombin deficiency
Liver disease →
Nephrotic syndrome (protein loss) →
Surgery and trauma → Modest fall in AT levels + nadir around post-op D3
Meds → asparaginase
Heparin therapy → up to 30% reduction
*OCP or estrogen treatment
52
Other clinical presentations of HITT aside from clotting
- skin necrosis at the site of heparin injections
- venous limb gangrene (eg patient presents with swollen and ischemic hand after cardiac surgery)
53
Cardiac surgery for patient with history of HITT
- IF ELISA is negative, rechallenge
- IF ELISA is positive, PLEX before
54
Blood type of mother with greatest risk of hemolytic disease of newborn if father is A+
- 0+
55
Additional antigens sickle cell patients need matching for
C, E, and Kell
56
How to differentiate alpha thal trait vs. beta thal trait
beta thal = A2 greater than 3.5
57
hemoglobin S trait on hgb electrophoresis
S of about 30%
58
Drugs precipitating G6PD
- sulfonylureas
- rasburicase
- phenazypyridine (pyridium)
*fluoroquinolones
*dabrafenib
59
Copper deficiency clinical features
*ringed sideroblasts on marrow
- cytopenias
- neurologic deficits
60
PKD clinical features
- *echinocytes on peripheral smear
- congenital hemolysis
*Pyruvate kinase can be low normal
*elevated hexokinase
61
Labs for PKD
- increased bisphosphoglyceric acid (2,3 BPG)
62
Treatment of AIP refractory to medical therapy
Liver transplant
63
function of erythroferrone
Decreases hepcidin production
64
Physiology of iron overload in MDS and other states of ineffective erythropoeis
- body senses inadequate iron so so increases erythroferrone production, which downregulates hepcidin to increase iron absorption
65
Ferroportin mutation is seen in what
hemochromatosis
66
AICD physiology
increased IL-6 production, which increases hepcidin production
67
Clinical features of congenital erythropoietic porphyria
bald + skin is peeling off + peeing red + brown teeth/Alopecia + friable skin + red urine + pink to brown pigmented teeth. Huge vat of urine to his right/very high levels of uroporphyrin.
68
Congenital erythropoietic porphyria (CEP) treatment
Bone marrow transplant
69
CNL clinical features
-neutrophil count can be very high, but is typically in the range of 20,000 to 35,000/microL
- otherwise normal blood counts
- no dysplasia in marrow and blast count 0-5%
70
Bone marrow in systemic mastocytosis + immunophenotype
- spindle shaped mast cells
- CD117+, CD2+, CD25+
71
management of hypereosinophilia with strongyloides travel hx
- empiric abx
- strongyloides antibody
- bone marrow biopsy
- pred 1 mg/kg (mitigate end organ damage from hypereosinophilia)
72
Gaucher disease physiology + gene mutation
- lysosomal storage disease
- glucocerebroside (a sphingolipid, also known as glucosylceramide) accumulates in cells and certain organs.
73
Managmeent of gaucher patient on imiglucerase with hypersensitivity reaction
Discontinue and start eliglustat
74
Upshaw shulman management
- recombinant ADAMTS13
- IF not available, plasma infusion
75
Management of pregnant Upshaw shulman patient
regularly scheduled plasma infusion
76
Relapsed aplastic anemia management
*alternative donor*-HSCT
77
Luspatercept indications in low risk MDS
- SF3B1
*ringed sideroblasts
78
why do AYA patients do better with pediatric intensive regimens?
- higher doses of nonmyelosuppressive drugs (asparaginase isn't typically in adult regimens like Hyper-CVAD and isn't very myelosuppressive)
79
Unfavorable risk HL management
- ABVD not XRT
80
Advanced stage NPHL
- rituximab based combination chemo
- R-CHOP
81
Relapsed ALL management
- IF treatment refractory, blinatumomab or inotzumab if CD22 bridget to allo-HSCT
- If treatment refractory, CAR-T
82
blinatumomab target
CD19
83
Consolidation for Ph- ALL who is MRD-
Blinatumomab alone (Preferred -- Transplant related mortality offsets survival advantage)
Blinatumomab + chemo
84
bexarotene SE's to know
- hypothyroidism
- hyperlipidemia
85
Brentuximab SE's to know
- peripheral neuropathy
- pneumonitis
86
Where does somatic hypermutation occur?
Germinal center of B-cell follices
87
Defining high risk smoldering myeloma
- 20/2/20
*also cytogenetics (same as for myeloma)
88
ET risk stratification
- age >60
- prior VTE
- CV RF's
*JAK2 V617
89
POEMS management with limited sclerotic lesions
Radiation
90
CNL treatment
Jak inhibitors
91
treatment for systemic POEMS
auto-HSCT
92
Pathogen associated with chronic ibrutinib
Aspergillus
93
CRS management
*remember that if mild (fever without hypotension or hypoxia), just supportive care without tocilizumab
94
Most common BCR/ABL fusion protein in CML
p210
(picture matt in a net)
95
Clinical features of T cell histiocyte-rich large b cell lymphoma
- larger degree of infiltrating reactive T cells and macrophages
- tend to be young with liver and bony involvement
96
Copper deficiency MCV
microcytic
97
Recommended duration of prophylactic lovenox for cancer patients
28 days of LMWH
98
MYH9 inclusion bodies on peripheral smear
- see photo online
99
May Hegglin anomaly association
MYH9
100
target hemoglobin level with red cell exchange in SCD
hgb of 10
101
characteristic mixing study pattern of acquired hemophilia
prolongs at 1 hour and hten worsens at 2 hrs
102
Management of pt with protein s deficiency determined during pregnancy
antepartum vigilance, postpartum prophylaxis
103
Management of pregnant homozygous FVL or prothrombin patient
antepartum and postpartum prophylaxis
104
Bridging key point with warfarin bridging
*You always need at least 5 days of overlap. If INR bumps, don't hold
105
Whole blood contains
Anti A and anti B
106
Hereditary thrombophilia pts who need antepartum ppx
- antithrombin deficiency
- prothrombin gene mutation
- homozygous FVL
107
Monomorphic PTLD consistent w/ DLBCL management
upfront rituximab
