Malignant lymphoma and extensive viral wart formation in a patient with intestinal lymphangiectasia and lymphocyte depletion

Ward, Mairi; Roux, Antoine; Small, W.; Sircus, W.

doi:10.1136/pgmj.53.626.753

Cited by 28 publications

(14 citation statements)

References 7 publications

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“…Among 50 protein-losing enteropathy cases, three patients were reported to complicate malignant lymphoma (29). Some of these cases have been reported in Japan (30,31), such as that by Kojima et al (31) who reported a very similar case to ours.…”

Section: Discussionsupporting

confidence: 75%

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Human Herpes Virus 8-negative Primary Effusion Lymphoma (PEL) in a Patient after Repeated Chylous Ascites and Chylothorax

et al. 2004

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We describe a case of malignant lymphoma which presented in the body cavities without identifiable tumor masses. Malignant lymphoma cells showed strong atypia with prominent nuclei and basophilic cytoplasm containing vacuoles. The chromosomes showed diploidy and complex abnormalities including translocations and deletions. We diagnosed this patient with primary effusion lymphoma (PEL), even though she tested negative for human herpes virus-8 (HHV-8) which has been suggested to be causally related to PEL. Interestingly, the patient also showed complicated protein-losing enteropathy, and PEL occurred after repeated chylous ascites and chylothorax. The possible pathogenesis of this rare disease is discussed here.

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Section: Discussionsupporting

confidence: 75%

“…Protein-losing enteropathy often complicates malignant lymphoma (28,29). Among 50 protein-losing enteropathy cases, three patients were reported to complicate malignant lymphoma (29).…”

Section: Discussionmentioning

confidence: 99%

Human Herpes Virus 8-negative Primary Effusion Lymphoma (PEL) in a Patient after Repeated Chylous Ascites and Chylothorax

et al. 2004

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“…A diagnosis of PIL was clearly suggested by several observations: (a) early onset, in childhood and adolescence, respectively, of symptoms directly related to protein losing enteropathy—that is, peripheral hypoalbuminaemic oedema (due to intestinal lymph loss), chylous peritoneal, and pleural effusions12—or to marked depression of cell mediated immunity (longstanding cutaneous warts of presumably viral origin). Indeed, such cutaneous viral warts have already been reported as a clinical presentation of PIL6 11; (b) anatomical demonstration in patient No 1 of enteromesenteric (pseudo lymphangiomatous) lymphangiectasia at the age of 11—that is, nearly 40 years before the onset of lymphoma; and (c) persistent small intestinal diffuse lymphangiectasia in both patients after successful treatment of the lymphoma.…”

Section: Discussionmentioning

confidence: 82%

Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts

Bouhnik

Etienney

Németh

et al. 2000

Gut

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As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They presented with a longstanding history of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in duodenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diVuse ileal infiltration. In both, histological examination showed centroblastic high grade B cell lymphoma associated with duodenojejuno-ileal mucosal and submucosal lymphangiectasia. They were subsequently successfully treated with surgery and postoperative chemotherapy (AVmCP: adriamycin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PACOB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone), respectively. A three year follow up in both cases showed persistent diVuse lymphangiectasia without evidence of lymphoma. The present findings support the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development.

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“…This possible pathophysiological mechanism raises the ques tion of the development of NLH in PIL towards malig nant lymphomas, as it is known that lymphomas may complicate PIL. Six cases have been hitherto reported [9,[19][20][21]. Only 2 out of 6 patients had intestinal lymphoma.…”

Section: Discussionmentioning

confidence: 99%

Immunological Study in Primary Intestinal Lymphangiectasia

Heresbach¹,

Raoul²,

Genetet³

et al. 1994

Digestion

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Primary intestinal lymphangiectasia is a rare congenital condition associated with protein-losing enteropathy. Hypogammaglobulinemia and lymphopenia secondary to this condition are frequent but infectious complications are not. So far few immunological studies have been made in these patients. We report here the results of such a study carried out in two adolescents. Both patients presented with a dramatic decrease in serum gammaglobulins, especially IgG and IgA, and in peripheral blood lymphocytes, especially CD4 T helper cells. From a functional standpoint, the proliferative response to certain mitogens was reduced. A decrease in in vitro production of immunoglobulins by B lymphocytes may be due to a faulty T/B cell cooperation. Histological examination of duodenal biopsy specimens revealed a decreased number of intraepithelial lymphocytes. Colonoscopy revealed nodular lymphoid hyperplasia in the terminal ileum, confirmed by endoscopic biopsy. The role of these abnormalities in the development of infectious complications and lymphoma is underscored.

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Malignant lymphoma and extensive viral wart formation in a patient with intestinal lymphangiectasia and lymphocyte depletion

Abstract: Summary A patient is described, the subject of a previous report (Ross et al., 1971), who subsequently developed gross peripheral mosaic warts and a malignant small bowel lymphoma in association with marked lymphocyte depletion.

Cited by 28 publications

References 7 publications

Human Herpes Virus 8-negative Primary Effusion Lymphoma (PEL) in a Patient after Repeated Chylous Ascites and Chylothorax

Human Herpes Virus 8-negative Primary Effusion Lymphoma (PEL) in a Patient after Repeated Chylous Ascites and Chylothorax

Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts

Immunological Study in Primary Intestinal Lymphangiectasia

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