Two cases of nonmalignant immunoproliferative small intestinal disease (IPSID) presenting with severe malabsorption are described. The first patient had a lymphocytic infiltrate of the lamina propria and lymphoid hyperplasia of the mesenteric lymph nodes and responded to oral tetracycline. The second patient had a polyclonal plasmacytic infiltration of the lamina propria and of the mesenteric nodes and responded only to cytotoxic treatment with cyclophosphamide. These cases represent examples of non-alpha-chain disease benign IPSID.
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http://dx.doi.org/10.1159/000199416 | DOI Listing |
Blood
March 2011
Department of Laboratory Medicine, Warren Grant Magnuson Clinical Center, Bethesda, MD, USA.
Somatic gain-of-function mutations in members of the RAS subfamily of small guanosine triphosphatases are found in > 30% of all human cancers. We recently described a syndrome of chronic nonmalignant lymphadenopathy, splenomegaly, and autoimmunity associated with a mutation in NRAS affecting hematopoietic cells, and initially we classified the disease as a variant of the autoimmune lymphoproliferative syndrome. Here, we demonstrate that somatic mutations in the related KRAS gene can also be associated with a nonmalignant syndrome of autoimmunity and breakdown of leukocyte homeostasis.
View Article and Find Full Text PDFEur J Gastroenterol Hepatol
September 2006
Department of Immunology-Histocompatibility, Evangelismos General Hospital, Athens, Greece.
Objectives: Immunoproliferative small intestinal disease (IPSID) represents a spectrum of clinicopathological entities including alpha-chain disease and other types of lymphoplasmacytic proliferations of the lamina propria of the small intestine, presenting with severe malabsorption. IPSID has been described mainly in the Mediterranean, Middle East, and African countries. It occurs rarely in western countries.
View Article and Find Full Text PDFTwo cases of nonmalignant immunoproliferative small intestinal disease (IPSID) presenting with severe malabsorption are described. The first patient had a lymphocytic infiltrate of the lamina propria and lymphoid hyperplasia of the mesenteric lymph nodes and responded to oral tetracycline. The second patient had a polyclonal plasmacytic infiltration of the lamina propria and of the mesenteric nodes and responded only to cytotoxic treatment with cyclophosphamide.
View Article and Find Full Text PDFSchweiz Med Wochenschr
April 1979
Among 246 patients (49 with Hodgkin's disease, 29 with multiple myeloma, 75 with other lympho- and immunoproliferative syndromes, 70 with carcinomas and 23 with non-malignant affections) treated by cytostatic or immunosuppressive chemotherapy, 6 developed malignant hemopathy (acute myeloblastic leukemia, erythroleukemia and erythremia) apparently induced during the last 7 1/2 years. In addition, 2 carcinomas have been noted. All have received melphalan or chlorambucil, alone or associated with other cytostatic drugs.
View Article and Find Full Text PDFOne hundred and fifty-four patients with a monoclonal gammopathy, diagnosed between 1965-1974 in the Hadassah University Hospital are reviewed with special reference to the relative incidence of associated disorders. Most patients (63 per cent) had immunoproliferative disorders (multiple myeoloma, macroglobulinemia of Waldenstrom, chronic lymphocytic leukemia, and other non-Hodgkin lymphomata). A non-B-cell malignancy, either of blood-forming tissues or of epithelial origin, was found in 6.
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