Archives of Pathology & Laboratory Medicine
Waldenstrom'sMacroglobulinemia, Angioimmunoblastic T-cell Lymphoma
A 73-year-old woman presented with generalized lymphadenopathy, atypical peripheral blood lymphocytosis, and Coombs-positive hemolytic anemia. She was diagnosed with Waldenstrom macroglobulinemia (WM)/lymphoplasmacytic lymphoma (LPL), on the basis of IgM monoclonal gammopathy, significant lymphoplasmacytic infiltrate in bone marrow, circulating plasmacytoid lymphocytes in peripheral blood, monoclonal B lymphocytes with LPL-compatible immunophenotype by flow cytometry in bone marrow and peripheral blood, and clonal immunoglobulin heavy-chain gene rearrangement by polymerase chain reaction in peripheral blood. Molecular study results for MYB deletion and MYD88 mutation were negative. Following brief chemotherapy for WM, the patient's IgM monoclonal gammopathy and hemolytic anemia improved and the number of circulating monoclonal B lymphocytes decreased; however, she was noted to have polyclonal hypergammaglobulinemia and worsening generalized lymphadenopathy. An axillary lymph node was biopsied and showed no evidence of WM/LPL, but showed characteristic morphologic, immunohistochemical, and molecular features of angioimmunoblastic T-cell lymphoma (AITL), with Epstein-Barr virus (EBV) positivity in small B lymphocytes and clonal T-cell receptor gene rearrangement. This is the first reported case of concurrent presentation of WM and AITL. Because of the known association of AITL with B-lymphocyte abnormalities, including polyclonal hypergammaglobulinemia, Coombs-positive hemolytic anemia, and diffuse large B-cell lymphoma, the patient's WM is proposed to be pathogenetically related to AITL. Many of the B-cell abnormalities seen with AITL are thought to be related to immune dysregulation and poor control of EBV associated with AITL. This case illustrates a previously unreported form of B-cell lymphoma in association with AITL and suggests a possible pathogenetic connection to this T-cell lymphoproliferative disorder.
Jariwala, A., Alakeel, F., Lee, E., & Karcher, D. S. (2016). Concurrent Presentation Of Waldenstrom's Macroglobulinemia and Angioimmunoblastic T-cell Lymphoma. Archives of Pathology & Laboratory Medicine, 141 (9). Retrieved from https://hsrc.himmelfarb.gwu.edu/smhs_path_facpubs/179