Alpha heavy chain disease developing in an Asian resident in the United Kingdom and responding to antibiotics

Ross, I. N.; Thompson, Rohan; Asquith, P.

doi:10.1136/pgmj.59.693.443

Cited by 3 publications

(1 citation statement)

References 7 publications

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“…This fact may be related to the improvement in the socio-economic status and the sanitary conditions in developing countries. However, the disease still exists and it is interesting that in developed countries of the west new cases appear as a result of the ongoing influx of 'Third World' residents [34,[54][55][56].…”

Section: Discussionmentioning

confidence: 99%

Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania

Economidou¹,

Manousos²,

Triantafillidis³

et al. 2006

European Journal of Gastroenterology & Hepatology

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The patients were categorized in three subgroups of IPSID: alpha-chain disease (n=8), non-alpha chain disease with other monoclonal immunoglobulins (n=3), and polyclonal 'non-malignant' IPSID (n=2). In several patients the disease had unusual features, and this in some cases delayed the diagnosis. In suspected cases it is thus of the utmost importance to proceed to an exploratory laparatomy. Patients with stage C disease had a short survival, whereas two patients with stage A alpha-chain disease responded to treatment with cyclophosphamide, vincristine and prednisolone, and cyclophosphamide, doxorubicine, vincristine and prednisolone, respectively, have a disease-free long survival of 35 and 12 years, and appear to be cured.

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Section: Discussionmentioning

confidence: 99%

Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania

Economidou¹,

Manousos²,

Triantafillidis³

et al. 2006

European Journal of Gastroenterology & Hepatology

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Alpha-chain disease. Report of a case from taiwan

Shih¹,

Liaw²,

Hsueh³

et al. 1987

Cancer

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The occurrence of alpha-chain disease (a-CD) in a Chinese patient, a resident of Taiwan, is reported. The patient's clinical features were characterized by severe malabsorption and ran a rapidly deteriorated course. Duodenal biopsy showed total villous atrophy with a heavy infiltration of the lamina propria by lymphoplasmacytic cells. Colonic biopsy revealed immunoblastic lymphoma. a-CD protein was demonstrated in the patient's serum and concentrated urine by the immunoselection method, and also detected in the cellular infiltrate by immunoperoxidase technique. Postmortem examination disclosed that the lymphoma involved not only the entire gastrointestinal tract and mesenteric nodes, but also the retroper-itoneal and peripheral lymph nodes. Immunoperoxidase studies support the concept of a common clonal origin of the lymphoplasmacytic proliferation and immunoblastic lymphoma in a-CD. Cancer 59545-548, 1987 LPHA-CHAIN DISEASE (a-CD), first described by Se

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Leukemic manifestation in a case of alpha‐chain disease with multiple polypoid intestinal lymphocytic lymphoma

et al. 1992

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We describe a female aborigine from Taiwan with alpha-chain disease associated with multiple polypoid intestinal lymphocytic lymphoma and leukemic manifestation. Initially, the patient experienced intermittent diarrhea, abdominal pain, and leukemic manifestation. No evidence of bone marrow involvement was found. Alpha-chain protein was demonstrated in the serum. Gastroendoscopy and a series of radiographs of the small intestine revealed multiple polypoid tumors involving the entire length of the small intestine. Duodenal biopsy showed diffuse lymphocytic lymphoma. Immunohistochemical staining of tumor samples revealed features typical of alpha-chain disease. Cytogenetic analysis showed the same abnormal karyotypes of neoplastic clones in intestinal tumor cells and in circulating leukemic cells. The data suggest that alpha-chain disease can present initially with intestinal multiple polypoid lymphocytic lymphoma and leukemic manifestation without evidence of bone marrow involvement. The data also support the homing theory of lymphomas from mucosa-associated lymphoid tissue.

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Alpha heavy chain disease developing in an Asian resident in the United Kingdom and responding to antibiotics

Cited by 3 publications

References 7 publications

Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania

Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania

Alpha-chain disease. Report of a case from taiwan

Leukemic manifestation in a case of alpha‐chain disease with multiple polypoid intestinal lymphocytic lymphoma

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