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Heme Onc > Heme Onc Board Prep > Flashcards

Heme Onc Board Prep Flashcards

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
Q

Cytogenetics/Gains/losses
Hyperdiploidy:
Low Hypodiploidy
Haploidy
Masked hypoloidy

A

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

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26
Q

Ph like ALL deletion of

A
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27
Q

4 most valuable drugs in remission induction for ALL

A

steroids
VCR
PEG
Anthracycline

28
Q

SR ALL treatment overview

A

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
Q

cranial radiation for CNS3 disease receives 1800 cGY which includes …

A

posterior halves of the globes of eyes
spinal radiation rarely used

30
Q

why give HD MTX before cranial radiation for ALL

A

HD mtx after cxrt is associated with cns toxicity

31
Q

which day of MRD is most significant predictor of outcome of ALL

A

day 29

32
Q

targets:
Inotuzumab
blina
car-t

A

Inotuzumab: CD22 conjugated to calicheamicin
blina: bispecific CD3 and CD19
car-t: transduced with anticd19 receptor

33
Q

why must you treat an ALL CNS relapse systemically?

A

very high chance of BM relapse if you dont treat systemically

34
Q

When CD34+ levels is over ___ you collect stem cells for transplant

A

> 20cells/uL

35
Q

Hematopoietic cell sources:
Autologous transplant:
Allogenic transplant:

A

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
Q

What is the mismatch limit for unrelated donor bone marrow/PBSC

A

single allele/antigen mismatch (7/8). If there’s more than one mismatch, then need to use post-cy

37
Q

who gets non myeloablative vs reduced intensity vs myeloablative conditioning in allogenic transplants?

A

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
Q

9 mo with HLH s/p transplant with RIC regimen has falling chimerism at day 55+. what is the next step?

A

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
Q

Which type of T cell is most delayed to recover after transplant

A

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
Q

T cell absent
T cell broken

A

SCID
WAS, Hyper IGM, XLP, ALPS

41
Q

B cell absent
B cell broken

A

XLA (increased t cells)
CVID (decrease b and t cells, normal CBC)

42
Q

Neuts absent
Neuts broken

A

SCN
CGD

43
Q
A
44
Q

Definition of severe aplastic anemia

A

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
Q

eosinophilic fascitis and hypogammaglobinemia can be seen with

A

secondary aplastic anemia

46
Q

which type of hepatitis can be associated with aplastic anemia?

A

seronegative (non A-G)

47
Q

what can you see 5-10 days after starting ATG for aplastic anemia?

A

serum sicknesss: fever, rash, myalgia, arthralgia, myocarditis, gi/cns/renal

48
Q

when do you automically dose reduce eltrombopeg for aplastic anemia

A

Southeast asian ancestry

49
Q

PNH is d/t acquired somatic mutations in PIG-A gene. How does PIGA function?

A

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
Q

diagnosis of PNH

A

flow to quantitate % of GPI deficient (absent CD55/59) anchored protein on granulocytes and other cell lineages

51
Q

missing radii but still have thumbs =
missing radii but no thumbs =

A

TAR
Fanconi anemia

52
Q

what if you have a high suspicion of fanconi anemia but blood testing breakage analysis is equivocal

A

repeat breakage testing on cultured skin fibroblasts

53
Q

you can treat FA with androgens. why do you need to follow LFTs

A

peliosis hepatis (blood lakes)

54
Q

pulmonary fibrosis, early greying, dental, hyperhydrosis

A

dyskeratosis congeniti

there are other features but pulm fibrosis and early greying are commonly tested on boards.

55
Q

how often do you marrow SDS patients

A

yearly

56
Q

ELA-2/ELANE gene

A

severe congenital neutropenia (ANC<200)

heterozygous mutations with cyclic neutropenia

57
Q

WHIM syndrome

A

neutropenia with warts, hypogammaglobulinemia, infections, myelokathexis

AD CXCR4 mutation

HPV susceptibility

58
Q

age <1 year
macrocytosis
reticulocytopenia
ADA deficiency
mutations in rps and rpl ribosomal proteins

A

dBA

59
Q

dba or TEC?
early childhood presentation 1-5 yrs
no physical anomalies
some may have macrocytosis
half will have high plt count

A

TEC
dba presents under a year with macrocytosis

60
Q

congenital megakaryocytic thrombocytopenia (CAMT)
AR, c-MPL gene mutations (thrombopoietin receptor) which decreases BM megakaryocytes so thrombocytopenia noted at birth. How do you treat

A

transplant

61
Q

RUSAT
radioulnar synostosis with amegakaryocytic thrombocytopenia

how does this differ from CAMT

A

strong predisposition to early marrow failure (sAA)
screen bilateral forearms, FTT, renal US and echo

62
Q

TAR
when do you expect them to outgrow thrombocytopenia

A

by 1 year

look for bilateral absence of radii with thumbs (in FA there is no thumbs)

63
Q

Pearson Syndrome

A

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
Q

GATA2 spectrum disorders

A

monocytopenia, warts

Monocytopenia and mycobacterial infection, monosomy 7 MDS

65
Q

Most common infection among those with MPO deficiency and diabetes

A

disseminated Candida

66
Q

How to diagnose specific granule deficiency

A

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
Q
A

Heme Onc (2 decks)

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