Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Non-Hodgkin lymphoma may occasionally contain large transformed cells resembling Hodgkin and Reed-Sternberg cells (HRS cells). We report a 63-year-old man with HRS cells in a recurrent mantle cell lymphoma (MCL). The patient initially presented with orbital MCL and recurred after 8 years with widespread involvement. The HRS cells were present in the recurrent disease but not in the initial orbital lesions, suggesting a transformed event after a prolonged disease course. Morphologically, the HRS cells were single cells and small clusters among the MCL cells and were frequently accompanied by histiocytes but without eosinophils or other inflammatory cells. The HRS cells showed a phenotype of classic Hodgkin lymphoma (cHL). The HRS cells were clonally related to the MCL, which was demonstrated by the presence of identical t(11;14) that resulted in productive cyclin D1 expression in both cell types. Review of the literature identified 7 additional MCL cases that showed a spectrum of clinical and pathologic features ranging from scattered HRS cells to true composite MCL and cHL. The HRS cells were clonally related to MCL in 4 cases (including the current case) and unrelated in 2 cases. These findings suggest MCL with HRS cells is a heterogeneous group that may represent a spectrum of transformation at the various stages. Proof of clonal relationship between HRS cells and MCL is useful to distinguish these cases from true composite MCL and cHL.
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PMID:Mantle Cell Lymphoma With Hodgkin and Reed-Sternberg Cells: Review With Illustrative Case. 2854 34

Mantle cell lymphoma is a rare and aggressive subtype of B-cell non-Hodgkin lymphomas. Mantle cell lymphoma frequently involves extranodal sites, and gastrointestinal tract is involved microscopically and macroscopically in more than 80$ of cases. We present two cases of recurrent mantle cell lymphoma presenting with lower and upper gastrointestinal bleeding, respectively. A 58-year-old woman with a history of recurrent mantle cell lymphoma treated with chemotherapy and stem cell transplantation presented with left-sided abdominal pain and hematochezia. Colonoscopy showed a mass-like lesion in the ascending colon, polyps in the ascending colon, and splenic flexure. A 68-year-old man with a history of mantle cell lymphoma treated with chemotherapy presented with epigastric pain and melena. Esophagogastroduodenoscopy showed a large polypoidal ulcerated mass with oozing in the duodenal bulb. Biopsies in both patients were suggestive of mantle cell lymphoma. Patients with mantle cell lymphoma could be asymptomatic or may present with abdominal pain, obstruction, diarrhea, or gastrointestinal bleeding. In patients presenting with gastrointestinal symptoms, endoscopy must be pursued and biopsies must be taken for any suspicious lesions as well as normal mucosa to exclude mantle cell lymphoma as an etiology for the lesion or symptoms. Even though there are no standard guidelines for endoscopic screening of gastrointestinal tract in asymptomatic patients, one should be aware of involvement of gastrointestinal tract in the early course of disease or recurrent disease. Although mantle cell lymphoma is initially responsive to chemotherapy, it eventually becomes refractory with a median survival of 3-5 years.
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PMID:Recurrent Mantle Cell Lymphoma Presenting as Gastrointestinal Bleeding. 3018 88