Neoplastic T-cell Processes_Fellowship

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Neoplastic T-cell Processes_Fellowship
2014-09-18 22:48:18
Neoplastic cell Processes

Neoplastic T-cell Processes
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  1. What antigens are lost in the following T-cell LPDs?
    1) Adult T-cell Leukemia
    2) Peripheral T NOS
    3) ALCL
    4) MF/Sezary
    • 1) Adult T-cell Leukemia - CD7 neg w/ strong CD25
    • 2) Peripheral T NOS - CD5 or CD7 neg
    • 3) ALCL/ALK+ = CD3 neg, often CD5 and CD7 neg, usually CD43+, strong CD25+
    • 4) ALCL/ALK- = CD3 neg, often lose T-cell marker
    • 4) MF/Sezary = loss CD7 or diminished CD2, CD5, or sometimes CD3
  2. What is the IPT profile of AITL?
    • T cells: CD4+, CD10, BCL6 with no loss pan-T markers, PD-1, CD10, CXCL13
    • FDM: disorganized & surrounds HEV
  3. What cells in AITL can mimic HD?
    EBV-driven large, often multinucleated R-S like cells as well as immunoblasts scattered throughout small to medium malignant T-cells with clear cytoplasm with minimal nuclear atypia
  4. Which BCL marker is often seen in T-cell malignancies?
  5. What is the DDx for enteropathy-associated T-cell neoplasms?
    Peripheral T NOS Enteropathy associatedT-cell with gamma/delta, CD56+, CD3+ALK+ ALCL (rare!)
  6. What is the IHC profile for enteropathy associated T-cell? What other aberrancy can be found? Chrom abnml?
    • Type I: CD3+, CD4-, CD8-, CD56-, CD103+, TCRbeta/gamma+, subset CD30+ in most cases
    • Type II (monomorphic): CD3+, CD4-, CD8+, CD56+, TCRbeta/gamma+
    • Type I - Gain 1q or 5q; Type II - MYC amplifications (8q24)
  7. What is the DDx in T-PLL? Chromosomal abnormalities? Molecular findings?
    Blastic mantle cell, atypical CLL, and B-PLL (very rare!); 1) 80%  inversion chrom 14 w/ brkpts @ q11 and q32,  2) 70-80% chromosome 8 abnormalities: idic 8p11, t(8;8)(p11-12;q12); TCRγ&β rearranged
  8. In T-prolymphocytic leukemia, what distinct clinical findings are notable? Morphology on a PB smear? Characteristic IHC profile?
    • HSM w/rash in 20% of cases, anemia, thrombocytopenia, ↑↑↑WBC (>100k
    • with 50% of cases >200k), minority show serous effusions, esp pleural; small-med lymphoid cells with irregular nuclear contours, prominent nucleolus; 25% co-express CD4+/CD8+ (but usually CD4+/CD8-)
  9. All of the following T-cell lymphomas are CD4+/CD8- except:
    A) Peripheral T-cell lymphoma
    B) Adult T-cell leukemia
    C) Mycosis fungoides
    D) EATL
    E) AITL
  10. All of the following are CD8+/CD4- except:
    A) EATL
    B) Blastic plasmacytoid dendritic cell neoplasm
    C) Hepatosplenic TCL
    D) Subcutaneous panniculitis-like TCL
    E) Primary Cutaneous γ/δ TCL