Peripheral T-Cell Lymphomas with Cytotoxic Phenotype in Patients with Nodal SLL/CLL
Daniel F Boyer, Neal I Lindeman, Nancy L Harris, Judith A Ferry. Massachusetts General Hospital, Boston, MA; Brigham and Women's Hospital, Boston, MA
Background: Cases of patients with B- & T-cell lymphomas are uncommon, and cases in which patients with CLL develop peripheral T-cell lymphoma (PTCL) are extremely rare. Patients with CLL have expanded effector T-cell populations, including occasional circulating cytotoxic T-cell clones that could be the source of rare PTCLs. We evaluated 3 cases of PTCL in patients with SLL/CLL.
Design: 2 cases of ALK+ ALCL & 1 case of CD8+ PTCL in patients with SLL/CLL were identified in the consult files of the MGH Dept of Pathology. Immunophenotyping was performed by standard IHC & flow cytometry. 1 case of composite ALCL & SLL was further characterized by PCR-based analysis of IGH & TCR-gamma gene rearrangements and FISH for ALK gene rearrangement.
Results: Patient 1: A 56-yo previously healthy woman presented with lymphadenopathy. Inguinal lymph node biopsy showed multifocal involvement by ALK+ ALCL (CD30+ CD5+ perforin+ EMA+; other B & T-cell markers negative) and diffuse infiltration by CD5+ small B cells, consistent with SLL/CLL. ALK rearrangement was present in the ALCL cells but not in small lymphocytes. DNA was isolated from ALCL-enriched and SLL/CLL-enriched areas of the lymph node. Clonal TCR-gamma rearrangement was identified in the ALCL-enriched region but not in the SLL/CLL-enriched region. The same B-cell clone was present in both, but the amount of PCR product was lower in the ALCL-enriched region.
Patient 2: A 67-yo woman presented with cervical adenopathy. Biopsy of a cervical node showed SLL/CLL. Her adenopathy responded to chemotherapy, but she developed axillary adenopathy 8 months later. Biopsy showed ALK+ ALCL (CD30+ CD5+ granzymeB+ perforin+ CD4 & CD7 dim; other B & T-cell markers negative) with no residual SLL/CLL.
Patient 3: A 70-yo man with a 10-year history of nodal & marrow involvement by SLL/CLL followed an indolent course until onset of progressive thrombocytopenia and splenomegaly. Splenectomy showed diffuse infiltration of the red pulp by large atypical CD3+ CD2+ CD8+ CD5- CD30-/+ ALK- perforin+ granzymeB+ T cells, diagnosed as PTCL, NOS.
Conclusions: Our 3 patients are older adults with predominantly nodal involvement by SLL/CLL who also had T-cell lymphomas with cytotoxic immunophenotype, including 2 ALK+ ALCL, an uncommon PTCL in this age range. Our observations suggest that, in the setting of expanded cytotoxic T-cell populations in patients with SLL/CLL, ALK translocation may provide the trigger for T-cell oncogenesis in rare cases.
Monday, March 4, 2013 1:00 PM
Poster Session II # 216, Monday Afternoon