HTLV-1-Associated Lymphoma Presented as Massive Lymphadenopathy.

Adult T-cell leukemia/lymphoma is an aggressive T-cell malignancy caused by the long-term infection of human T-cell lymphotropic virus type 1 (HTLV-1). Our understanding of clinical features still largely relies on the Shimoyama classification developed 30 years ago, which described the 4 clinical subtypes (the smoldering, chronic, lymphoma, and acute types) based on the manifestations of lymphocytosis, elevated lactate dehydrogenase, hypercalcemia, lymphadenopathy, and involvement of the skin, lung, liver, spleen, central nervous system, bone, ascites, pleural effusion, and gastrointestinal tract. HTLV-1-associated lymphoma has a variety of presentations but the presentation of massive lymphadenopathy and compression symptoms is rare and has not been emphasized in the literature. In this article, we describe 2 cases of adult T-cell leukemia/lymphomas that presented with massive cervical nodes or mediastinal nodes with compressing symptoms as the major presenting clinical features. Clinicians should remain aware of this type of presentation by HTLV-1-associated lymphoma, especially in patients who came from endemic areas, even if not all clinical features are present and particularly with hypercalcemia and lytic bone lesions.

Aggressive Diffuse Intermediate Size B-Cell Lymphoma With P53 Mutation Presented as Primary Bone Marrow Lymphoma.

Primary bone marrow lymphoma (PBML) is a disease entity in which lymphoma primarily originates in the bone marrow without signs of involvement of lymph nodes, spleen, liver, or any other organs, and excludes leukemia/lymphoma. PBML has been a rare presentation of malignant lymphoma, and most of the cases have a poor prognosis and require rapid diagnoses and treatments. Among all PBMLs, diffuse large B-cell lymphoma (DLBCL) is the most common pathological subtype. Over 25 years and from 7 institutions, the International Extranodal Lymphoma Study Group retrospectively collected PBML cases and, in 2012, published these 21 cases, including 19 cases of B-cell lymphoma and 2 cases of peripheral T-cell lymphoma. Among the B-cell types, DLBCL accounted for 79% and follicular lymphoma (FL) for 21%. DLBCLs were characterized by the existence of large cells. In this article, we present a rare case of high-grade aggressive type with P53 mutation, intermediate-sized B-cell lymphoma, excluded FL by the absence of FL lymphoma markers, presented as PBML. Our patient had rapid progression and succumbed to the disease shortly after diagnosis. Upon literature review, 62 B-cell lymphoma cases were identified that presented as PBML (51 high-grade and 11 low-grade)-mostly case reports. Among these, only one case was reported as intermediate-sized DLBCL-like lymphoma but not with aggressive features. Our case represents the first case of aggressive intermediate-sized lymphoma, not a FL, with P53 mutation, highly elevated lactate dehydrogenase, and Ki-67 presented as PBML. Such a profile would need to be quickly recognized and aggressive treatment applied, such as CART (chimeric antigen receptor T-cells) therapy or DA-EPOCH-R (dose-adjusted EPOCH [etoposide-prednisone-oncovin-cyclophosphamide-hydroxydaunorubicin] and rituximab) with or without venetoclax.