Document Detail

Composite Peripheral T-cell Lymphoma, Not Otherwise Specified and B-cell Small Lymphocytic Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis.
MedLine Citation:
PMID:  23129836     Owner:  NLM     Status:  Publisher    
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.
Ahmed Alomari; Pei Hui; Mina Xu
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-4
Journal Detail:
Title:  International journal of surgical pathology     Volume:  -     ISSN:  1940-2465     ISO Abbreviation:  Int. J. Surg. Pathol.     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-6     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9314927     Medline TA:  Int J Surg Pathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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