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Heme Onc > Heme Onc Board Prep > Flashcards

Heme Onc Board Prep Flashcards

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1
Q

Cytogenetics: t(8:21)
Molecular:
FAB:
Characteristics:

A

Cytogenetics: t (8:21)
Molecular: RUNX1
FAB: M2
Characteristics: Auer Roads, Chloromas, CNS+, good prognosis

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2
Q

Cytogenetics: inv 16
Molecular:
FAB:
Characteristics:

A

Cytogenetics: inv 16
Molecular: CBFB-MYH11
FAB: M4Eo
Characteristics: Eos with baso granules, chloromas, CNS+, good px

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3
Q

Cytogenetics: t (15;17)
Molecular:
FAB:
Characteristics:

A

Cytogenetics: t(15;17)
Molecular: PML-RARA (APL)
FAB: M3
Characteristics: Granules/Auer rods, DIC/bleeding, good px with ATRA

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4
Q

Cytogenetics: 11q23
Molecular:
FAB:
Characteristics:

A

Cytogenetics:11q23
Molecular: MLL(KMT2A)
FAB: M4/m5
Characteristics: infant, WBC/skin/CNS/gums, t-AML after topo II inhibitor

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5
Q

therapy related AML:
Topo II inhibitors, etop, anthracyclines, platinums

-Genetics?
- Treatment?

A

1-2 year onset
presents in fulminant AML
11q23 common but can be seen in 8;21, 15;17, 9;22, inv16
treat with chemo and HSCT

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6
Q

treatment related AML: alkylators and radiation

-genetics?
- Treatment?

A

5-7 onset
MDS precedes AML
-5, del5q, del 7q
Chemo and HSCT

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7
Q

leukemia rates in mono twins

A

increased risk, younger the first twin is at dx, the higher the risk of second twin (<1 year old). over 6 is minimal. between 1-6 is 20%

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8
Q

AML Favorable Characteristics:
(5)

A
  1. DS <4 yo
  2. APL t(15;17)
  3. inv 16, 8;21
  4. Molecular: NPM1, CEBPA
  5. MRD neg after course 1
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9
Q

Unfavorable characteristics of AML
(5)

A
  1. t-AML, MDS-AML
  2. monosomy -7, 5q, 3q
  3. FLT3/ITD molecular
  4. induction failure (>5% blasts after course 2)
  5. MRD positive after course 1
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10
Q

AML Induction regimen

A

Dauno, cytarabine, gemtuz +/- etop

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11
Q

CML originates from abnormal pluripotent HSC. it is PH+, 9;22 BCRABLE +. What are the 3 phases

A

Chronic: <10% in marrow and blood
Accelerated; 10-19%
Crisis: >20%

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12
Q

P210 is asstd with…
P190 is asstd with

A

CML
ALL

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13
Q

CML treatment

A

HU used sometimes
continue TKI indefinitely. COnsider HSCT in chronic phase
for refracgtory chronic you can do higher tki dose or change tiki
for accelerated phase or blast crisis try to induce remission with chemo + TKI then take to HSCT

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14
Q

incidence rate of AML and ALL

A

2-4 years (80 million cases/million
81% ALL
Whites 2x higher
Leading cause of cancer death <20 years

1970s CNS deemed a sanctuary site

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15
Q

genetic conditions increased risk for ALL

A

DS, ATM, nijmegan breakage syndrome, bloom (AML), constitutional mismatch repari, rasopathies, kleinfelter, li fraumene (hypodiploid), immunodeficiency

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16
Q

Which gene is overexpressed in DS with ALL

A

60% overexpress CRLF2, P2Ry8 most common. Favorable prognosis for DS with CRLF2 vs patients with CRLF2 without DS

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17
Q

ALL Risk stratification, SR vs HR

A

SR: 1-9.9yrs, WBC <50k
HR: 1-10 yrs with WBC >50, >10 years

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18
Q

T ALL slower to clear than B ALL. What time point is prognostic?

A

MRD at end of consolidation is most prognostic (vs at end of inductino like B ALL )

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19
Q

most important prognostic indicator for infant ALL

A

KMT2A most common is t(4;11)
poor prognosis especially under 90 days old, more often extramedullary sites

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20
Q

what kind of leukemia has eosinophils over 100,000?

A

eosinophilia can be reactive, not related to a specific type of

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21
Q

Hypercalcemia is associated with which translocation?

A

t(17;19)
TCF3:HLF fusion
poor prognosis

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22
Q

Which type of blasts express HLA-DR

A

almost all b precursors are DR+, most t all are DR-, some AML are DR+

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23
Q

myeloid expression like cd13 and 33 is common in ALL and has no prognostic significance (commonly seen with ETVRUNX1). When does it confer a poor prognosis

A

when myeloid and lymphoid features are on the same cell
Very poor prognosis when there are distinct populations of lymphoid and myeloid blasts (indicates a more primitive stem cell)

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24
Q

MPAL - AML or ALL therapy?

A

ALL therapy

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25
Cytogenetics/Gains/losses Hyperdiploidy: Low Hypodiploidy Haploidy Masked hypoloidy
Hyperdiploidy: gain of 4 and 10 (double trisomy) Low Hypodiploidy: modal number 32-39 chromosomes Haploidy: modal number <32 chromosomes Masked hypoloidy: can masquerade as hyperdiploidy
26
Ph like ALL deletion of
27
4 most valuable drugs in remission induction for ALL
steroids VCR PEG Anthracycline
28
SR ALL treatment overview
4 week induction 4 week consolidation with wkly IT (8 week for HR) 8 week interim maintenance with escalating IV mtx 8 week delayed intensification maybe 2nd interim maintenance (capizzi for HR) Maintenance
29
cranial radiation for CNS3 disease receives 1800 cGY which includes ...
posterior halves of the globes of eyes spinal radiation rarely used
30
why give HD MTX before cranial radiation for ALL
HD mtx after cxrt is associated with cns toxicity
31
which day of MRD is most significant predictor of outcome of ALL
day 29
32
targets: Inotuzumab blina car-t
Inotuzumab: CD22 conjugated to calicheamicin blina: bispecific CD3 and CD19 car-t: transduced with anticd19 receptor
33
why must you treat an ALL CNS relapse systemically?
very high chance of BM relapse if you dont treat systemically
34
When CD34+ levels is over ___ you collect stem cells for transplant
>20cells/uL
35
Hematopoietic cell sources: Autologous transplant: Allogenic transplant:
Autologous transplant: PBMC, quick engraftment Allogenic transplant: yet to be determined. bone marrow has intermediate rejection and GVHD. PBMC gives more t cells, rapid engraftment, but more chronic GVHD Bone marrow is preferred in peds for nonmalignant disorders. Used for high risk procedures: reduced intensity regimens, second transplant PBSC is preferred for graft manipulation (T cell depletion)
36
What is the mismatch limit for unrelated donor bone marrow/PBSC
single allele/antigen mismatch (7/8). If there's more than one mismatch, then need to use post-cy
37
who gets non myeloablative vs reduced intensity vs myeloablative conditioning in allogenic transplants?
Allogeneic: Myeloablative are superior to RIC for AML/MDS - Bu/Cy, Bu/Flu TBI-based are better for ALL RIC and non ablative better for nonmalignant disorders (RIC for HLH flu/mel/thio) SCID has no prep needed. Serotherapy (ATG, Campath ) used to reduce GVHD All autos are getting high dose, myeloablative therapy (NBL, CNS, Lymphoma)
38
9 mo with HLH s/p transplant with RIC regimen has falling chimerism at day 55+. what is the next step?
wean immune suppression rapidly and follow chimerism note that in aplastic anemia, bc it's an autoimmune reponse sometimes in partial chimerism you will increase immune suppression
39
Which type of T cell is most delayed to recover after transplant
CD4 Agents to prevent GVHD further compromise cd4 recovery: ATG, pred = lysis cyclosporine, tacro = inhibit TCR activation, thus early cytokine production MTX, MMF, siro (mtor inhibitor that inhibits cells cycle) = inhibit clonal expansion and proliferation
40
T cell absent T cell broken
SCID WAS, Hyper IGM, XLP, ALPS
41
B cell absent B cell broken
XLA (increased t cells) CVID (decrease b and t cells, normal CBC)
42
Neuts absent Neuts broken
SCN CGD
43
44
Definition of severe aplastic anemia
2/3 peripheral blood criteria: anc<500, plts <20k, retic <1% and 1/2 bone marrow criteria: <25% cellularity, or up to 50% cellularity with <30% hematopoietic cells Very severe AA: ANC<200
45
eosinophilic fascitis and hypogammaglobinemia can be seen with
secondary aplastic anemia
46
which type of hepatitis can be associated with aplastic anemia?
seronegative (non A-G)
47
what can you see 5-10 days after starting ATG for aplastic anemia?
serum sicknesss: fever, rash, myalgia, arthralgia, myocarditis, gi/cns/renal
48
when do you automically dose reduce eltrombopeg for aplastic anemia
Southeast asian ancestry
49
PNH is d/t acquired somatic mutations in PIG-A gene. How does PIGA function?
it creates an anchor called GPI which binds glycoproteins CD55/59 to cell membrane. If it does not bind cell can undergo complement mediated lysis
50
diagnosis of PNH
flow to quantitate % of GPI deficient (absent CD55/59) anchored protein on granulocytes and other cell lineages
51
missing radii but still have thumbs = missing radii but no thumbs =
TAR Fanconi anemia
52
what if you have a high suspicion of fanconi anemia but blood testing breakage analysis is equivocal
repeat breakage testing on cultured skin fibroblasts
53
you can treat FA with androgens. why do you need to follow LFTs
peliosis hepatis (blood lakes)
54
pulmonary fibrosis, early greying, dental, hyperhydrosis
dyskeratosis congeniti there are other features but pulm fibrosis and early greying are commonly tested on boards.
55
how often do you marrow SDS patients
yearly
56
ELA-2/ELANE gene
severe congenital neutropenia (ANC<200) heterozygous mutations with cyclic neutropenia
57
WHIM syndrome
neutropenia with warts, hypogammaglobulinemia, infections, myelokathexis AD CXCR4 mutation HPV susceptibility
58
age <1 year macrocytosis reticulocytopenia ADA deficiency mutations in rps and rpl ribosomal proteins
dBA
59
dba or TEC? early childhood presentation 1-5 yrs no physical anomalies some may have macrocytosis half will have high plt count
TEC dba presents under a year with macrocytosis
60
congenital megakaryocytic thrombocytopenia (CAMT) AR, c-MPL gene mutations (thrombopoietin receptor) which decreases BM megakaryocytes so thrombocytopenia noted at birth. How do you treat
transplant
61
RUSAT radioulnar synostosis with amegakaryocytic thrombocytopenia how does this differ from CAMT
strong predisposition to early marrow failure (sAA) screen bilateral forearms, FTT, renal US and echo
62
TAR when do you expect them to outgrow thrombocytopenia
by 1 year look for bilateral absence of radii with thumbs (in FA there is no thumbs)
63
Pearson Syndrome
refractory sideroblastic anemia by age 6 months -- mitochondrial DNA deletion **** marrow shows vauolated precursors/ringed sideroblasts can look like SDS bc also has pancreatic dysfunction
64
GATA2 spectrum disorders
monocytopenia, warts Monocytopenia and mycobacterial infection, monosomy 7 MDS
65
Most common infection among those with MPO deficiency and diabetes
disseminated Candida
66
How to diagnose specific granule deficiency
through a smear you will see lack of specific granules and bilobed nuclei then confirmed by electron microsopy and genetic sequencing. It is very rare and d/t defect in the myeloid specific transcription factor ccaat/enhancer biding protein epsilon (CEBPE)
67

Heme Onc (2 decks)

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