Gamma heavy chain disease simulating alpha chain disease.

Bender, S. W.; Danon, F; Preud’homme, Jean-Louis; Posselt, H.G.; Roettger, P; Séligmann, M

doi:10.1136/gut.19.12.1148

Cited by 17 publications

(5 citation statements)

References 16 publications

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“…8 For the purpose of this review, the terms IPSID and ␣ heavy chain disease (␣HCD) are used synonymously to refer to the secretory form, a relatively well-defined clinicopathologic entity. This is not withstanding the fact that there are rare patients with ␥ rather than ␣ heavy chain IPSID 9 and the rare colonic, gastric, or pulmonary ␣HCD. [10][11][12] IPSID is a unique mature B-cell neoplasm regarding its epidemiology, clinical features, morphology, and molecular pathogenesis.…”

Section: Introductionmentioning

confidence: 99%

Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms

Al‐Saleem

Al‐Mondhiry

2005

Blood

178

135

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Immunoproliferative small intestinal disease (IPSID) was recently added to the growing list of infectious pathogen-associated human lymphomas. Molecular and immunohistochemical studies demonstrated an association with Campylobacter jejuni. IPSID is a variant of the B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), which involves mainly the proximal small intestine resulting in malabsorption, diarrhea, and abdominal pain. Geographically, IPSID is most prevalent in the Middle East and Africa. IPSID lymphomas reveal excessive plasma cell differentiation and produce truncated alpha heavy chain proteins lacking the light chains as well as the first constant domain. The corresponding mRNA lacks the variable heavy chain (V(H)) and the constant heavy chain 1 (C(H)1) sequences and contains deletions as well as insertions of unknown origin. The encoding gene sequence reveals a deletion of V region and parts of C(H)1 domain. Cytogenetic studies demonstrated clonal rearrangements involving predominantly the heavy and light chain genes, including t(9;14) translocation involving the PAX5 gene. Early-stage IPSID responds to antibiotics (30%-70% complete remission). Most untreated IPSID patients progress to lymphoplasmacytic and immunoblastic lymphoma invading the intestinal wall and mesenteric lymph nodes, and may metastasize to a distant organ. IPSID lymphoma shares clinical, morphologic, and molecular features with MALT lymphoma, lymphoplasmacytic lymphoma, and plasma cell neoplasms.

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Section: Introductionmentioning

confidence: 99%

Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms

Al‐Saleem

Al‐Mondhiry

2005

Blood

178

135

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“…The vast majority of IPSID are characterized by the synthesis of Ig-α1 heavy chain without the light chain by the neoplastic cells, with occasional cases showing the production of Ig-γ heavy chain in absence of the light chain (Bender et al, 1978;Kopec et al, 1974). Sequence analyses of the Ig heavy chain gene of the neoplastic cells of IPSID show non-contiguous deletion that involves most of the VH/ JH (heavy chain variable/joining) segments and the switch/ CH (heavy chain constant region domain-1) sequence.…”

Section: Immunoproliferative Small-intestinal Diseasementioning

confidence: 96%

Gastrointestinal Lymphoma

Wotherspoon

Spencer

2015

Mucosal Immunology

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“…Protracted antigenic stimulation may lead to a variety of responses from the intes tinal immune system apart from a-CD or other monoclonal B cell proliferations [19,20], Rambaud et al [21] have described cases with diffuse follicular lymphoid hyper plasia and malabsorption simulating a-CD Baklien et al [22] have reported an unusual case with malabsorption and dense infiltra tion of the small intestine with immunocytes producing excessive polyclonal IgA. The present cases of non-a-CD ISPID represent further examples within the spectrum of these disorders.…”

Section: Discussionmentioning

confidence: 99%

Malabsorption Associated with Nonmalignant Immunoproliferative Small Intestinal Disease

Manousos¹,

Economidou²,

Papademetriou³

et al. 1987

Digestion

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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-α-chain disease benign IPSID.

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Gamma heavy chain disease simulating alpha chain disease.

Cited by 17 publications

References 16 publications

Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms

Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms

Gastrointestinal Lymphoma

Malabsorption Associated with Nonmalignant Immunoproliferative Small Intestinal Disease

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