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What antigens are lost in the following T-cell LPDs?
1) Adult T-cell Leukemia
2) Peripheral T NOS
- 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
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
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
Which BCL marker is often seen in T-cell malignancies?
What is the DDx for enteropathy-associated T-cell neoplasms?
Peripheral T NOS Enteropathy associatedT-cell with gamma/delta, CD56+, CD3+ALK+ ALCL (rare!)
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)
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
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-)
All of the following T-cell lymphomas are CD4+/CD8- except:
A) Peripheral T-cell lymphoma
B) Adult T-cell leukemia
C) Mycosis fungoides
All of the following are CD8+/CD4- except:
B) Blastic plasmacytoid dendritic cell neoplasm
C) Hepatosplenic TCL
D) Subcutaneous panniculitis-like TCL
E) Primary Cutaneous γ/δ TCL