Should we suppress the antigenic stimulus in IPSID for lifelong?

Çelık, Aykut Ferhat; Pamuk, Gülsüm Emel; Pamuk, Ömer Nurı; Uzunismail, Hülya; Oktay, Ergun; Dogusoy, Gulen

doi:10.1016/s0002-9270(00)02111-0

Cited by 6 publications

(5 citation statements)

References 10 publications

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“…Life-long suppression of antigenic stimulus has been proposed. 65 Response to antibiotic diminishes with increasing dysplasia of the lymphoplasmacytic cells and with tumorous infiltration of the bowel wall and the mesenteric lymph nodes. In addition to marked clinical improvement and histologic tumor regression by serial jejunal biopsies, response can be roughly quantitated by estimating the serum level of ␣HC protein.…”

Section: Treatment and Outcomementioning

confidence: 99%

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

Al‐Saleem

Al‐Mondhiry

2005

Blood

180

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: Treatment and Outcomementioning

confidence: 99%

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

Al‐Saleem

Al‐Mondhiry

2005

Blood

180

135

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“…The importance of supportive therapies, such as the intravenous infusion of minerals and albumin or even total parenteral nutrition [56,70], has also been stressed. A prolonged conservative treatment with tetracycline is also advised as a means to avoid recurrences [71]. In spite of these efforts, in general the prognosis is poor and the morbidity is high [72,73].…”

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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“…Helicobacter pylori 5 (6.8%) [7,17,18,22,26] Campylobacter jejuni 3 (3.9%) [7,19,20] Vibrio fluvialis 1 (1.3%) [7] Escherichia coli 1 (1.3%) [7] Staphylococcus albus 1 (1.3%) [16] Calcium Hypocalcemia 2 (2.6%) [15,22] Upper gastrointestinal endoscopy Atrophic nodular mucosa 3 (3.9%) [7,18,20] Thickening of intestinal folds 2 (2.6%) [18,25] Edema 2 (2.6%) [17,25] Barium X-ray Intestinal mucosal fold thickening 3 (3.9%) [15,18,26] Small intestine dilation 2 (2.6%) [10,15] Intestinal fold flattening 1 (1.3%) [20] Capsule endoscopy Mucosal edema / villi swelling in small intestine 1 (1.3%) [27] Abdominal X-ray Lymphadenopathy 1 (1.3%) [20] Colonoscopy Nodular mucosa 1 (1.3%) [7] Abdominal ultrasonography Enlarged mesenteric lymph nodes 1 (1.3%) [15] Thickening of small intestinal wall 1 (1.3%) [24] https://doi.org/10.1371/journal.pone.0253695.t003…”

Section: Microbiology Testingmentioning

confidence: 99%

Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)

et al. 2021

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Immunoproliferative Small Intestinal Disease (IPSID) is a disease characterized by extra-nodal marginal zone B-cell lymphoma with villous atrophy in the small intestine, causing chronic intermittent non-bloody diarrhea. Although originally associated with the Mediterranean region, this disease is present in many countries worldwide and may have been underreported due to its complicated diagnosis and scarce scientific literature, especially in regards to treatment. This study aims to review IPSID clinical features, therapeutic options, and treatment outcomes to help physicians identify and treat IPSID. Using PRISMA guidelines, a systematic review of articles was conducted on PubMed database with search terms including IPSID, therapy, treatment, and outcomes. Inclusion and exclusion criteria were used to select 33 English language articles published from the year 2000–2020 that included relevant clinical information about IPSID treatment. Data were extracted independently by at least two authors to reduce the introduction of potential bias. There were 22 case reports, 7 reviews, 1 research article, 1 prospective study, 1 letter to the editor and 1 memoriam in which 76 patients were identified. Epidemiological analysis showed a mean patient age of 32 years old, 2.4:1 mal to female ratio and heterogeneous ethnicities, with 16 Europeans (43.2%) and 12 Asians (32.4%). Chief symptoms included chronic diarrhea (53/76, 69.7%), weight loss (49/76, 64.4%), malabsorption (38/76, 50%), abdominal pain (32/76, 42.1%), and finger clubbing (24/76, 31.6%). Patients stratified into the early disease stage (Galian A) were treated with tetracycline antibiotics, corticosteroids, and non-pharmacological supplements with mostly with complete or partial remission. Late stages (Galian B or C), were treated mostly with anthracycline-based chemotherapy, and occasionally surgery, radiotherapy, or rituximab. This work offers a targeted approach to diagnosing and treating IPSID to aid physicians and serve as a treatment guideline recommendation for future public policies and clinical studies.

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Should we suppress the antigenic stimulus in IPSID for lifelong?

Cited by 6 publications

References 10 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

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

Treating chronic diarrhea: A systematic review on Immunoproliferative Small Intestinal Disease (IPSID)

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