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In what population is primary effusion lymphoma prevalent? Associated viruses? IHC profile? Molecular studies?
- HIV+; HHV8 and EBV; Negative for typical B-cell, T-cell, myeloid Ags but usu CD45+, CD30+, CD38+, CD138+, EMA+; southern blot shows IgH and LC clonal rearrangements
What form of lymphoma is usually seen in post-transplant LPDs? What other lymphomas can be observed in this setting?
conventional high-grade B-cell lymphoma; T-cell lymphoma & HD
Give the dx (NLPHL, TCBCL or HD) for the following IHC & histologic features:
1) CD45+, CD20+, EMA+, CD30-, CD15-; L&H popcorn cells, T-cell rosetting, absence of PMNs and eos
2) CD45-, CD20-, EMA-, CD30+, CD15+, PAX5+, fascin+, large "owl eyes" cells
3) CD20+, EMA-, CD30-, CD15-, no T-cell rosetting
What type of HD has this feature?
NLPHL - progressive transformation of GC with enlarged follicles (3-4x) showing inward migration of perifollicular and activated T cells into GCs, no L&H cells
What is the age distribution of classical HD?
Bimodal: peak at 15-35yo & late in life (60's)
Give the HD subtype for the following "factoids":
1) Common in underdeveloped world & HIV-associated
2) MC type in the western world
3) MC in elderly patients
4) Morph similar to NLPHD
5) Presents at a higher stage
6) In mediastinum > other types
- 1) MC
- 2) NS
- 3) LD
- 4) LR
- 5) LD
- 6) NS
Which HD does not commonly present in mediastinum but rather more frequently involves the abdomen? Which HD present in young females? Which type has highest % EBV+?
MC; NS & MC; MC
What is the growth pattern of MC type HD?
Interfollicular with no capsule thickening, some fibrosis but no broad bands, mixture of L, PMNs, eos, histios, and PC.
Which HD most frequently involves the BM? Which HD shows noncontiguous spread of lymphatic sites? Which type most often disseminates?
LD, HIV-associated (lowest in LP); LD; LD, MC and HIV-associated
Where are the R-S cells in the LN of LR HD? How do the GCs appear?
Found in mantle zone; regressed/atretic like Castleman's dz
Which B-cell lymphomas are CD11c+?
HCL, CLL, and mantle zone lymphoma
All of the following are T-cell lymphomas that can involve LNs except:
B) Peripheral TCL
C) Anaplastic large cell lymphoma
D) Blastic NK lymphoma
E) Subcutaneous panniculitic TCL
- E - in subQ soft tissue
- *Blastic NK involves the LN in some cases but is usually in the skin
All of the following are T-cell lymphomas that typically involve the blood/BM except:
A) T-cell PLL
B) T-cell LGL
C) Adult T-cell leukemia
D) Anaplastic large cell lymphoma
E) Aggressive NK cell leukemia
D - involves LNs
All of the following are extranodal T-cell lymphomas except:
A) Peripheral TCL
B) Subcutaneous panniculitis-like TCL
C) Enteropathy-type TCL
D) Mycosis fungoides
E) γδ cutaneous TCL
- A - primarily in LNs
- *But MF later may involve LNs!
For the following cell shapes, give the appropriate T-cell lymphoma:
1) Nuclear cloverleaf form
2) Cerebriform nuclei
3) Hallmark/horseshoe cells
- 1) Adult T-cell leukemia/lymphoma or Peripheral TCL
- 2) Mycosis fungoides
- 3) Anaplastic large cell lymphoma
What is the characteristic histology in PTCL? What variants exist?
Small or large cells with increased clear cytoplasm, sometimes cloverleaf nuclei with admixed inflammation composed of PCs, *eos and histiocytes; Lennert-like (lymphoepithelioid/lymphohistiocytic) and interfollicular (T-zone) variants
Which lymphomas typically have the following IHC profiles:
- 1) PTCL, ATCL, MF, AITCL (but can be mixed CD4/CD8), Blastic NK lymphoma
- 2) Precursor T ALL
- 3) LGL leukemia, hepatosplenic, Blastic NK
- 4) Precursor T ALL, hepatosplenic, primary cutaneous γδ T-cell lymphoma
This T-cell lymphoma variant frequently has trisomy 3.
Lennert-like PTCL variant (*T-cells frequently look like histiocytes)
60M p/w B symptoms, LAD, rash, and hepatosplenomegaly. Labs showed polyclonal hypergammaglobulinemia and DAT+ anemia. What T-cell process is most likely involved?
What is the classic histology and IHC profile of AITCL?
LN with arborizing HEVs surrounded by follicular dendritic cells, with either atretic, absent, or hyperplastic GCs early on; CD4+, CD8-, CD10+ (they are in GCs), CXCL13+, PD1+, sometimes EBER+
All of the following is true regarding ALCL except:
A) CD30+, CD15-, Alk+, EMA+, T-cell Ag+
B) No associated translocations
C) Large pleomorphic cells with sinusoidal growth pattern
D) Small cell variant (fried egg) is Alk+, mostly occur in children but prognosis is poor
E) Golgi staining pattern with clusterin highly specific for ALCL
- B - t(2;5)(p23;q35) in >95% of cases involving ALK gene on chrom 2 with NPM (nucleophosmin) gene on chrom 5, easily detected by PCR since on same intron
- *Other ALK rearrangements: t(1;2), t(2;3), and inv(2)
What is the most important prognostic factor in ALCL?
Nucleophosmin on chrom 5 is a protein that shuttles between the nucleus and cytoplasm, and is seen in t(2;5) ALCL. What staining pattern is seen with ALK in these tumors?
ALK+ in nucleus (other translocations show cytoplasmic staining)
Which lymphomas are EMA+?
- Primary effusion lymphoma
Is PCR or ALK IHC more sensitive in ALCL?
ALK IHC b/c variant translocations show cytoplasmic ALK staining and are negative by PCR
40yo Japanese female p/w LAD, splenomegaly, and a skin rash. Labs show leukocytosis and hypercalcemia. Xrays show lytic bone lesions. Dx? Viral cause? What is the IHC profile of this malignancy? What might the skin bx show in the chronic variant?
Adult T-cell leukemia/lymphoma (>50% have skin involvement); HTLV-1; CD2+, CD3+, CD5+, CD4+, FOXP3+, CD7-, CD8-, CD25+ (IL-2 receptor); elaboration of osteoclast activating factor; epidermitropism with Pautrier-like microabscesses
What lymphoma is associated with celiac? What is the IHC profile? Where in the GI does it often occur? Prognosis?
Enteropathy-associated TCL, often preceded by refractory sprue + mucosal ulceration; CD8+, CD103+, CD5-, CD30+/-, CD56+/-; jejunum; poor
20M p/w B sxs and hepatosplenomegaly but no LAD. Likely malignancy involved? What locations does this malignancy not involve? Mutation? What variant is more common in females?
hepatosplenic γδ TCL; peripheral blood or LNs; isochrome 7q or trisomy 8; αβ
What lymphoma has this IHC profile: CD4-/CD8+, Tia+, CD5-, Perforin-, ?
What splenic lymphomas involve the red pulp? White pulp?
- Red pulp: hepatosplenic, HCL, DLBCL
- White pulp: MZL
15yo Asian male p/w a huge midline facial tumor. Dx (give all possible names for this)? What does histology show? Viral association?
- Extranodal (nasal) NK/TCL or lethal midline granuloma or polymorphic reticulosis or angiocentric lymphoma; angiocentric + necrosis + hemophagocytosis, mixed inflamm (PC, eos, macs); EBV
What T-cell process has the following IHC profile: cCD3ε+, CD56+, CD57+, CD43+, TIA-1+, EBV+?
Extranodal T/NK cell lymphoma
Aggressive T-cell process with rimming of lobules by lymphocytes. Other areas show fat necrosis and karyorrhexis. Septa appear uninvolved. Dx?
SubQ panniculitis TCL
70M has long hx of skin rashes that are turning into plaques. Sections show tumor cells with cerebriform nuclei, halos show epidermotropism. Dx? Confirmatory stains?
MF; CD4+, CD8-, CD25- (helps differentiate from Adult TCL), often CD7-
In Sezary syndrome w/ peripheral blood & LN involvement, what location is typically uninvolved? Characteristic clinical feature?
BM; generalized exfoliative erythroderma
These cells were incidentally found in an enlarged LN. S100+, MART1+, weakly HMB-45+. What do these cells represent?
Incidental finding of nevus cells, benign cells are in the connective tissue of the capsule or trabeculae, never in the marginal sinus. There are no mitoses, no atypia.
How does Kimura manifest clinically? What are the LN histologic findings?
Chronic inflamm d/o of Asian males, have subdermal lesions in H&N, painless unilateral inflammation of LNs, peripheral eosinophilia; follicular hyperplasia with rx germinal ctrs, eosinophilic infiltrates involving the interfollicular areas, sinusoidal areas, perinodal soft tissue, and subQ, prolif of postcapillary venules
What are the histologic features of infectious mono in the LN?
- 1) marked sinusoidal and capsular/extranodal infiltration by immunoblasts, often w/ R-S like features and atypia
- 2) follicular hyperplasia
- 3) polymorphous inflammatory cells
What disease is also called giant cell lymph node hyperplasia? What is the most common subtype? What subtype is associated w/ HHV-8? What is multicentric dz associated with? In children?
Castleman's - they get really huge, up to 15cm; hyaline-vascular; plasma cell and can p/w fever, anemia, night sweats, wt loss; plasma cell variant and POEMS; multicentric dz w/ plasma cell variant in girls (75%), good response to steroids
How do solitary plasmacytomas present clinically? Where do they typically preset? Where do the extraskeletal tumors present? Progression to MM?
No CRAB, just a single bone lesion w/ monoclonal PC infiltrate; nasal cavity, nasopharynx, sinuses, oropharynx, lung or any other body site; usually, but in 10-20yrs
What is required to dx multiple myeloma?
- 1 major (marrow >30% or plasmacytoma on bx + signif M spike by >3.5g/dL serum or >1g/24hr urine)
- + 1 minor (plasmacytosis 10-30% or M spike but not as much)
- or 3 minor (must include first two criteria) - plasmacytosis in marrow, plasmacytoma + M spike, lytic lesions, Ig reduced to <50% normal
What is the cutoff for dx of plasma cell myeloma in the peripheral blood? MM in the marrow?
20% or absolute plasma cell count >2.0 x109 prolymphs/mL; 30%
What chromosomal mutations are involved in myeloma? What % of MM patients have 4+ abnormalities? Which translocations have the shortest survival?
t(14;16), t(4;14), t(11;14) (most common MM translocation), del 17p13.1, 13q14, 19p muts; >80%; 4;14, 14;16, or 17p13.1 del
Most common cause of lack of M-spike in a patient with MM? 2nd MCC?
light chain only myeloma; IgD (usually λ light chain)
What is the most sensitive marker for T-ALL? Most common site for T-ALL?
What MALT variant commonly shows plasmacytoid rather than monocytoid features?
Immunoproliferative small intestinal disease (IPSID) occurs in the Middle East, the Cape region of South Africa and a variety of other tropical and subtropical locations
What lymphoma shares the t(14;18) with FL?
DLBCL (good prognosis)
Most commonly involved location in plasmacytoma?
Vertebra > ribs > pelvis
What defines smoldering myeloma?
±10% BM + M protein(>30g/L) + no CRAB
Most common metastatic tumor forming granulomas in LNs?
SCC, seminoma, and thymoma
In nodular lymphocyte predominant hodgkin lymphoma (NLPHL), what are the major histologic and IHC findings?
CD4+/CD57+ T-cells rimming around EMA+, OCT2+/CD15-/CD30- neoplastic cells with expanded follicular dendritic meshwork (CD21+)