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In APL, what markers are lost? Aberrantly expressed? Which aberrant markers have prognostic implications?
CD15 (normal promyelocyte is CD15+/-); CD2 (microgranular variant), CD64 and CD56; CD56 expression poor prognosis
What marker is characteristically expressed in megakaryocytes/megakaryoblasts in AML M7 (megakaryoblastic) and MDS?
CD34 (usually CD34+/- in megakaryoblasts)
What is required for a diagnosis of MPN/MDS?
Features of MDS (fits a category of MDS w/dysplasia and <20% blasts) + MPN (leukoerythroblastosis with proliferation of at least one lineage) + NO characteristic cytogenetic abnmlty (BCR-ABL, PDGFR, FGFR1, etc)
Which APL translocations are not sensitive to Trans-retinoic acid tx?
t(11;17)(q23;q11) with PLZF-RARα and t(5;17), also short bcr3 breakpoint which has features of M3v
What causes inclusions/phagocytosis in pts with APL? What else could cause this finding?
Trans-retinoic acid tx; possibly GCSF, which could push myelos to maturation in APL (could kill pt?)
What mutation is often seen in histiocytic malignancies?
What is the characteristic feature of anemia in RA-RS?
Normo or microcytic anemia
What is a negative prognostic indicator in inv16 AML (eosinophils)?
Kit mutation (also negative impact on incidence of relapse but not overall survival in t(8;21)
IHC profile of blastic plasmacytoid dendritic cell neoplasms (formerly aggressive NK)?
CD4+, CD56+, CD123+, TCL1+, CD33+, CD68+, sometimes CD7+ and TdT+
What is the most common cytogenetic abnormality in T-prolymphocytic leukemia? Other abnormalities?
Inversion of chromosome 14 with breakpoints in q11 and q32; 10% t(14;14)(q11;q32); chromosome 8 abnormalities: trisomy 8q or idic 8p11, or t(8;8)
What T-cell lymphoma has isochromosome 7q in most cases?
Hepatosplenic T-cell lymphoma
What is the prognosis for the following cytogenetic abnormalities in AML? How would a kit mutation affect prognosis?
All good prognosis; kit mutation associated with a poorer prognosis/early relapse
What is the prognosis for the following cytogenetic abnormalities:
A) monosomy 7
C) 11q23 abnormalities (except 9;11)
E) trisomy 8
F) normal cytogenetics
- A) monosomy 7 - poor
- B) inv(3) - poor
- C) 11q23 abnormalities - poor
- D) t(9;11) - intermediate
- E) trisomy 8 - intermediate
- F) normal cytogenetics - intermediate
All of the following cytogenetic abnormalities are not considered definitive evidence of MDS except:
A) trisomy 8
Which of the following CLL genomic aberrations (more than one may apply) have a less favorable or poor prognosis:
C) isolated del(13q14.3)
E) Trisomy 12
All except C
All of the following are chemotherapy agents involved in therapy related MDS/AML except:
Which agent usually doesn't have an MDS phase?
A) Topoisomerase inhibitors
B) Alkylating agents (cyclophosphamide, nitrogen mustard, cisplatin, carboplatin)
C) Ionizing radiation therapy
D) Antimetabolites (5-FU, 6-MP, Ara-C)
E) Antitubulin agents (vincristine, docetaxel)
D; A - topoisomerase inhibitors do not have an MDS phase
In AML t(8;21), what are the genes? What do they encode?
Chrom 8 - ETO gene; chrom 21 is AML1 gene, which encodes the alpha chain of core binding factor (CBFalpha)
Which AML has the CBFbeta gene?
AML with inv(16) or t(16;16)
Do NK cells express CD3?
What disease shows these findings?
Langerhans cell histiocytosis (eosinophilic granulomas)
What is the staining pattern for AML M0 with respect to sudan black (SBB), MPO, CAE, and NSE? Are Auer rods seen?
Negative for all; Auer rods are rare
What do SBB and CAE stain for?
SBB - myeloids and erythroids (but negative in AML M0 and M5 (monoblasts) and M7 (megas)
CAE - granulocytes (but negative in AML M0 and M1)
In ALL with t(4;11), what will you characteristically find in the immunophenotypic profile?
What is the common protein size for ALL with t(9;22)?
Variant translocations in PML? Treatment implications? What breakpoint is associated with the variant M3 (M3v)?
t(11;17) and t(5;17); resistant to ATRA; short breakpoint (bcr3, intron 3)
Common sites for extramedullary plasmacytoma? Plasmacytomas in bone? Do they develop MM?
Nasal cavity, oropharynx, larynx; vertebrae > ribs > pelvis; 75% of bone plasmacytomas develop MM within 10 yrs; extramedullary plasmacytomas do not develop MM
Diagnostic criteria for smoldering myeloma? MGUS?
- Smoldering: 10-30% plasma cells, no CRAB
- MGUS: <3.5g/dL gammopathy, <10% plasma cells, no CRAB
Most common form of heavy chain disease?
Alpha heavy chain
Where is the BCL6 gene located? Common translocation in DLBCL?
Chrom 3q27; t(3;14) involving heavy chain
What lymphomas have t(14;18)
90% follicular lymphoma, small minority in DLBCL; rare in other B-cell lymphomas
Translocations in extranodal MZL?
- 1) t(11;18) involving API2 and MALT1 genes, usually in stomach and lung
- 2) t(14;18) involving MALT1-IgH gene fusion, usually in ocular, parotid, cutaneous sites
What is the expression of cyclin D1 in hairy cell leukemia?
Pathognomonic finding in plasma cells of heavy chain disease?
All of the following are minor criteria for primary myelofibrosis except:
A) Platelets ≥ 450
B) Increased LDH
T/F: Essential thrombocythemia demonstrates BM with increased granulocytes with left-shift.
False - only PV and PMF have this
How can the vacuolated erythroid precursors in MDS be differentiated from other causes (ETOH, CDA, anti-seizure drugs)?
Vacuoles in MDS are poorly defined and sharply delineated in toxic alteration
In pediatric follicular lymphoma, how does it differ from adult form?
1) BCL2 negative, 2) usually grade 3 but good prognosis, 3) no t(14;18)
How does cutaneous follicle center lymphoma differ from nodal FL?
Usually BCL2 negative and no BCL2 rearrangements, CD10 positive only in follicular patterns not diffuse, overall good prognosis
What virus is associated with PTLD? How does the polymorphic differ from monomorphic type?
EBV; polymorphic - immunoblasts, PCs, small-intermediate lymphocytes efface LN
Monomorphic looks like another B-cell lymphoma (except follicular and MALT don't count!)
The majority of PTLD in solid organ transplants are of what origin - host or donor? What types of transplants are most common?
Host origin; liver and lung allograft
When does PTLD typically present? How is it treated?
Within 1st year of tx; calcineurin inhibitors (cyclosporine or tacrolimus)
Morphologic findings in early lesion PTLD?
- *No effacement of LN architecture
- 1) Plasmacytic hyperplasia (plasma cells, lymphocytes, and bland immunoblasts) or
- 2) infectious mononucleosis-like (paracortical expansion with numerous immunoblasts in a background of T-cells and plasma cells)
Genes involved in B-ALL with t(5;14)? What is the morphology? Prognosis?
IL3-IgH translocation; variable circulating eosinophilia; not different from other ALLs
Genes involved in B-ALL with t(1;19)? Prognosis? Any unique morphology?
E2A(TCF3) on chromosome 19 and PBX1 on chromosome 1; previously considered poor prognosis but now overcome with modern tx; no, looks like regular ALL
Most common genetic abnormality in B-ALL in infants <1yo? What's the immunophenotype again? Prognosis?
MLL (11q23) translocations, esp t(4;11); CD19+, CD10-, CD15+; poor
35 year-old female presents with superior vena cava syndrome. Dx?
Primary mediastinal large B-cell lymphoma
Typical IPT of T-LGL leukemia?
CD8+, CD4-, CD16+, CD57+, TIA1+, Granzyme B+, commonly have diminished or loss of expression of CD5 and/or CD7
What is the expression profile of mantle cell lymphoma with respect to IgM and IgD? Splenic marginal zone? Nodal MZL?
Both IgM+/IgD+; usually IgM+/IgD-
Mutation in PDGFRA? PDGFRB? How is it detected? Tx?
- PDGFRA - CHIC2 deletion detected by FISH due to cryptic del(4)(q12)
- PDGFRB - rearrangement of 5q31-33, usually t(5;12) with ETV6-PDGFRB fusion
- *Sensitive to tyrosine kinase inhibitors like Gleevec
Clinical findings in PDGFR cases?
- High serum tryptase (>12ng/mL) and vitamin B12
- M:F ratio of 17:1
- Endomyocardial fibrosis, hepatosplenomegaly, pulmonary fibrosis
High risk cytogenetics in myeloma? Good prognosis?
- Bad: t(4;14), t(14;16), t(14;20), deletion 17p (p53), deletion 13 or aneuploidy, hypodiploidy
- Good: t(11;14) or t(6;14) by FISH, hyperdiploidy, absence of unfavorable cytogenetics
What disease does LN in POEMS syndrome look like? What does POEMS stand for?
- polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes
IPT of Sezary syndrome?
CD4+/CD8-, CD2+, CD3+, CD5+, CD7-, CD26-
Distribution of malignant cells in liver and BM of hepatosplenic T-cell lymphoma?
Intrasinusoidal like spleen
Most common morphology in MTX-induced LPD?
Which ALL cytogenetic abnormality has the worst prognosis?
What is the IPT for lymphomatoid papulosis? Where do they typically present? Morphology?
CD3+, CD4+/CD8-, CD5+, CD30+, TIA1+; trunk and extremities of adults; wedge-shaped dermal infiltrate extending into subcutaneous fat
What clonal abnormalities are common in NK-cell leukemia? Extranodal NK/T-cell lymphoma?
7p-, 17p-, 1q+; 6q-
Virus driving hydroa vacciniforme-like lymphoma? IPT? Hypersensitivity?
EBV; CD8+, granzyme B+, TIA1+, subset CD30+; mosquito bites
All of the following B-cell lymphomas are driven by EBV except:
D) Primary mediastinal large B-cell lymphoma
E) Lymphomatoid granulomatosis
All of the following T-cell lymphomas are driven by EBV except:
A) Aggressive NK-cell leukemia
C) Hepatosplenic T-cell lymphoma
D) Lymphoepithelioid (Lennert) lymphoma