Abstract
Peripheral T cell lymphomas (PTCLs) are uncommon and less well studied. Most PTCLs present as systemic disease and often involve bone marrow. Bone marrow involvement by PTCLs may damage the normal hematopoiesis and bring more challenge to clinical management of these patients. This study focuses on the clinical pathological features and clinical outcomes in 13 patients with nodal peripheral T cell lymphoma and subsequent bone marrow biopsy positive for the same lymphoma. Eight patients were diagnosed of peripheral T cell lymphoma - not otherwise specified (PTCL-NOS), 3 angioimmunoblastic T cell lymphoma (AITL), 1 anaplastic large cell lymphoma (ALCL), and 1 T cell lymphoma of gamma-delta origin (TCL-gamma/delta). All patients had peripheral blood abnormalities: 11 anemia, 7 thrombocytopenia, 5 neutropenia, and 2 lymphocytosis. Of interest, the CD4/CD8 phenotype of PTCL was shown correlating with abnormal peripheral blood findings: CD4+/CD8- phenotype was more often associated with anemia and thrombocytopenia, and CD4-/CD8+ phenotype was associated with lymphocytosis. Chemotherapy remains the choice of first line treatment for these patients with or without stem cell transplantation. 12 patients had treatment and follow up data available for review, 5 were in remission or free of disease during the follow up period; 5 patients were alive or in hospice with persistent disease and 2 died of disease due to multiple complications. The outcome of PTCL treated with the standard chemotherapy has been less favorable compared with B cell lymphomas. Majority of the patients with nodal PTCL have bone marrow involvement at the time of initial diagnosis, which has significant impact on normal hematopoiesis and may be a significant factor in the overall unfavorable prognosis for these patients. Further investigation with better knowledge about this disease will be helpful in the development of more efficient therapy and improve the disease free survival and life quality for these patients.
Highlights
Peripheral T cell lymphomas (PTCLs) are uncommon diseases, composing about 10% to 15% of non-Hodgkin lymphoma and about 6% of all lymphomas [1, 2]
The original diagnosis of PTCL from lymph node biopsies was listed in Table 1, including 8 PTCL-NOS, 3 angioimmunoblastic T cell lymphoma (AITL), 1 anaplastic large cell lymphoma (ALCL) and 1 TCL of gamma-delta origin
Less common and not well studied, PTCLs often present as systemic disease with bone marrow involvement
Summary
Peripheral T cell lymphomas (PTCLs) are uncommon diseases, composing about 10% to 15% of non-Hodgkin lymphoma and about 6% of all lymphomas [1, 2]. PTCLs are divided into nodal, extranodal, cutaneous and leukemic based on the clinical presentation and organ system involvement [3, 4]. Nodal PTCLs are the most common and usually present as late stage disease at diagnosis with spread to other organ systems including bone marrow [5, 6]. PTCL involving bone marrow is an indication for late stage disease, and impact the normal hematopoietic functions and may bring more challenges for the treatment and management of these patients [7]. The treatment of PTCLs has been traditionally similar to diffuse large B cell lymphoma [8, 9]. The study on PTCLs is still limited and the clinical and pathological features of PTCL involving bone marrow are not well illustrated. This study is focused on the pathological features of these patients with clinical correlation and follow up studies
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