title: Composite peripheral T-cell lymphoma not otherwise specified, and B-cell small lymphocytic lymphoma presenting with hemophagocytic lymphohistiocytosis.
abstract: We report a case of a 68-year-old female patient who developed hemophagocytic lymphohistiocytosis (HLH) secondary to peripheral T-cell lymphoma (PTCL) not otherwise specified (NOS) that developed in the setting of treatment-resistant B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL). The patient's B-cell lymphoma had a good initial response to chemotherapy for 4 years, after which it became less responsive and was thought to have undergone transition to a higher-grade lymphoma. Different regimens of chemoradiotherapy were then tried with modest response until the patient presented 3 years later with signs and symptoms of HLH. The patient died 1 month later, and an autopsy was performed. Significant para-aortic lymphadenopathy and splenomegaly were found. Microscopic, immunohistochemical and molecular evaluations confirmed the presence of composite B-cell and T-cell lymphoma in the para-aortic enlarged lymph nodes. Bone marrow examination showed hemophagocytosis, and the liver demonstrated infiltration by activated macrophages with hepatocellular necrosis. This report highlights the importance of searching for a possible underlying T-cell lymphoma in light of HLH. Different theories have been proposed to explain the rare occurrence of concurrent B- and T-cell lymphomas, but the development of HLH in this patient highlights the importance of immune dysregulation as a proposed mechanism to explain some cases of composite lymphomas. A review of the literature and discussion of the relative merits of these hypotheses are presented in the context of this case.
authors.affiliation: Yale-New Haven Hospital, New Haven, CT, USA.
authors.forename: Ahmed
authors.initials: A
authors.lastname: Alomari
authors.forename: Pei
authors.initials: P
authors.lastname: Hui
authors.forename: Mina
authors.initials: M
authors.lastname: Xu
mesh_terms: D000368:Aged; D015897:Comorbidity; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D016411:Lymphoma, T-Cell, Peripheral / Q000150:complications* / Q000453:epidemiology / Q000473:pathology
keywords: lymph node pathology