Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 29-year-old man, a known heroin addict until 1984 in whom HIV antibodies had been first demonstrated in 1985, was hospitalized because of fever, nocturnal sweating, weight loss and treatment-resistant diarrhoea. An opportunistic infection of the gastrointestinal tract was excluded microbiologically and serologically. Coloscopy and biopsy revealed a highly malignant gastrointestinal B-cell lymphoma, which had caused a spontaneous rectosigmoid-ileum fistula. Lymphoma infiltrations were also found in the duodenum, jejunum, left lung and brain. Because the underlying disease was far progressed (CD4/CD8 ratio: 0.04) and the patient was in a poor general condition neither surgery nor chemotherapy was undertaken. He died of cerebral lymphoma involvement. Gastrointestinal lymphoma should be included in the differential diagnosis of chronic diarrhoea in HIV-positive patients.
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PMID:[A fistula between the rectosigmoid junction and the ileum as a complication of highly malignant AIDS-associated lymphoma]. 174 69

This is the case report of a 4-year-old white boy who was diagnosed as having acute lymphoblastic leukemia (ALL) in November 1985. While in remission and on maintenance chemotherapy, he developed a primary Epstein-Barr virus (EBV) respiratory infection in October 1986. On October 27, 1986 a plain abdominal radiograph taken for abdominal distention showed free air. At celiotomy, multiple nodules were noted to stud the small bowel. Central necrosis of these nodules with perforations were present in the distal small bowel. Resections and end-to-end anastomoses were performed. Three days later the patient again had a similar acute abdominal episode. At reexploration, similar lesions in the liver, kidney, duodenum, proximal jejunum, and colon were found. Liver biopsy as well as intestinal resections and end-to-end anastomoses were performed, along with a loop ileostomy. Polymorphic B-cell lymphoma positive for EBV was found in the specimens. After cessation of chemotherapy and institution of abdominal radiotherapy, the hepatic and renal lesions were seen to resolve on computed tomography scan. The patient's course was complicated by the development of cervical and mediastinal abscesses that were drained, and E coli sepsis accompanied by chronic diarrhea requiring intravenous hyperalimentation. By January 1988, he appeared to be recovering. His ileostomy was closed in March 1988. Despite cessation of chemotherapy since October 1986, the patient is now well and in complete remission.
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PMID:Multiple small bowel perforations in leukemia secondary to Epstein-Barr virus lymphoma with survival: a case report. 217 5

Twenty-five children or adolescents with relapsed or refractory non-Hodgkin's lymphoma (NHL) were included in this phase II study of the combination of cytarabine (ARA-C) 50 mg/m2/d by 12 hours continuous infusion day 1 to day 5, ARA-C 3 g/m2/d in 3 hours day 1 to day 4, and etoposide (VP 16) 200 mg/m2 daily from day 1 to day 4. Twelve patients had B-cell, 12 T-cell, and one non-T, non-B-cell lymphoma; according to Murphy's staging system, 15 had stage III and nine stage IV disease with bone marrow involvement at diagnosis. All had previously received ARA-C by push or continuous infusion. Two patients had received epipodophyllotoxins. At the time of the study, three children had initial refractory disease, 18 were in first relapse (14 on therapy), two in first refractory relapse, and two in second relapse (on therapy). The overall response rate (RR) was 60%: eight complete responses (CRs), seven partial responses (PRs) (two became CRs after a second course). The RR was 66% (four CRs plus four PRs) in B-cell and 54% (four CRs, three PRs) in non-B-cell NHL. It was 20% (one PR per five patients) in initial or relapsed refractory disease. In four patients with measurable CNS disease, there were three CRs. Duration of response was nonassessable since all the responding patients received high-dose polychemotherapy followed by autologous bone marrow transplantation (ABMT) (five are alive with long follow-up [FU]). Toxicity was marked mostly by pancytopenia for 2 weeks, and half the patients encountered a grade-3 infection. One severe diarrhea was observed. In conclusion, high-dose ARA-C (HD-ARA-C) and VP 16 are an effective regimen in relapsed NHL, especially with CNS disease, and its toxicity is acceptable with regards to the prognosis of the disease.
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PMID:Phase II study of cytarabine and etoposide in children with refractory or relapsed non-Hodgkin's lymphoma: a study of the French Society of Pediatric Oncology. 231 35

Primary lymphoma of colon is rare in clinical practice and sometimes difficult to diagnose. We described a case of primary, low grade B-cell lymphoma of colon, whose clinical pictures were similar to inflammatory bowel disease. Although the colon mucosa showed diffuse infiltration of lymphocytes with lymphoepithelial lesions on endoscopic biopsy, immunohistochemical stains for B- and T-cell markers were negative. However, the diagnosis of lymphoma was established by positive findings on rearrangement of antigen receptor gene. Clinical symptom of blood-tinged diarrhea subsided after six courses of chemotherapy. Follow-up colonoscopy revealed normal colon mucosa. However, biopsy of colon mucosa still revealed mild proliferation of small lymphocytes. This case has drawn our attention that primary colonic lymphoma may clinically simulate inflammatory bowel disease, and gene rearrangement analysis might be helpful in the diagnosis of malignant lymphoma when traditional histological and immunohistochemical studies fail to provide a definite diagnosis.
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PMID:Primary low grade B-cell lymphoma of colon mimicking inflammatory bowel disease: a case report. 929 17

Twenty-five patients with relapsed or refractory non-Hodgkin's lymphoma were treated by combination chemotherapy with irinotecan hydrochloride (CPT-11) and adriamycin (ADM): CPT-11, 25 mg/m2 on days 1 and 2; ADM, 40 mg/m2 on day 3. Nine (36%) of twenty-five patients achieved CR. Fairly good responses were seen in relapsed B-cell lymphomas (4 of 8 in diffuse large B-cell lymphoma and 2 of 2 in follicular lymphoma grade 1), and substantial responses in T-cell lymphomas (1 of 4 in peripheral T-cell lymphoma and 2 of 7 in adult T-cell leukemia/lymphoma). Leukopenia was frequent but tolerable, and diarrhea minimal. Combination chemotherapy with a reduced dose CPT-11 and ADM was useful in the treatment of relapsed non-Hodgkin's lymphoma.
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PMID:Combination chemotherapy with irinotecan and adriamycin for refractory and relapsed non-Hodgkin's lymphoma. 1069 Apr

Irinotecan hydrochloride (CPT-11), a DNA topoisomerase-I inhibitor, is now widely used in the treatment of various solid tumors, including colorectal, gastric, breast, lung, and ovarian cancer. Despite the good response shown in the late phase-II study, CPT-11 was not often employed in the treatment of malignant lymphoma, mainly because of severe leukopenia and diarrhea caused by the recommended schedule: 40 mg/m2 of CPT-11 on days 1 to 3, 8 to 10, 15 to 17, then discontinued for at least 2 weeks. In clinical use, administration of CPT-11 had to be ceased on days 15 to 17 in almost all cases, and on days 8 to 10 in a considerable number of patients. Subsequently, a lower dose schedule (less than 40 mg/m2) was developed. Our phase II trial employing a reduced dose of CPT-11 on days 1 and 2, plus ADM on day 3 with 3-week interval in patients with refractory and relapsed NHL showed a fairly good response of relapsed B-cell lymphoma and a substantial response of T-cell lymphoma with acceptable toxicity. The combination of a topoisomerase-I inhibitor (CPT-11) and a topoisomerase-II inhibitor is an interesting concept for the treatment of NHL. Another phase II trial in combination with CPT-11 and other anti-cancer drugs, particularly cisplatin or topoisomerase-II inhibitors, is warranted. A superior salvage chemotherapy regimen could be found in the future by investigating combinations of low-dose CPT-11 and cisplatin or topoisomerase-II inhibitors.
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PMID:Chemotherapy with irinotecan (CPT-11), a topoisomerase-I inhibitor, for refractory and relapsed non-Hodgkin's lymphoma. 1169 85

We report the case of a 33-year-old man who presented with a large B-cell non Hodgkin's lymphoma presenting as acute pancreatitis. Abdominal CT showed diffuse swelling of the pancreas, with two distinct masses in the corpus and the tail. Thoracic CT showed a markedly enlarged mediastinum, with a voluminous mass in the middle mediastinum. Direct biopsy of this mass revealed a large B-cell lymphoma. Chemotherapy followed by peripheral blood cell autotransplantation led to complete disappearance of the pancreatic and mediastinal masses. Fatty diarrhea occurred after chemotherapy, probably owing to gland destruction by lymphomatous infiltration. Twenty-six months later, the patient is disease-free but continues to require pancreatic enzyme supplements.
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PMID:Secondary pancreatic involvement by diffuse large B-cell lymphoma presenting as acute pancreatitis: treatment and outcome. 1213 34

Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by protein-losing enteropathy occurring more frequently in childhood. Chronic diarrhea and diffuse edema are the main clinical manifestations. Peripheral lymphedema may also be associated. Lymphedema is usually present at the time of diagnosis or appears later in the course of the disease. We report the observation of a 31-year-old man suffering from an upper, lower limb and genital lymphedema many years before diagnosis of primary intestinal lymphangiectasia was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and hypogammaglobulinemia were also noted. Treatment of lymphedema included low stretch bandaging and elastic stocking. No dietary management with a low-fat diet was added. Search for primary intestinal lymphangiectasia with biological parameters would be useful when primary lymphedema is present. Especially since primary intestinal lymphangiectasia may be complicated by occurrence of B cell lymphoma.
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PMID:[Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)]. 1522 6

We conducted a phase II study to evaluate the efficacy and safety of cladribine (2-chlorodeoxyadenosine [2-CdA]) for patients with refractory or relapsed indolent B-cell lymphoma or mycosis fungoides. Forty-five patients were enrolled, and 43 patients, including 34 with follicular lymphoma, were eligible. 2-CdA was given by continuous intravenous infusion at a dose of 0.09 mg/kg daily for 7 consecutive days, and this schedule was repeated every 4 weeks up to a maximum of 6 cycles. The overall and complete response rates were 58.1% (25/43; 90% confidence interval, 44.5%-70.9%) and 14.0% (6/43), respectively. The disease progression-free proportions of all 43 eligible and all 25 responding patients at 2 years were 30.3% and 48.1%, respectively. Neutropenia and thrombocytopenia of grade 3 or 4 were observed in 53.3% and 37.8% of patients, respectively, with prolonged cytopenia observed in patients with increased numbers of treatment cycles. Nonhematologic toxicities of grade 3 or greater included diarrhea, arrhythmia, malaise, and gastrointestinal bleeding in 1 patient each, an increase in glutamic-pyruvic transaminase level in 2 patients, and infection in 5 patients. Two treatment-related deaths were observed. Four patients developed myelodysplastic syndrome (MDS) at 13 months to 2 years after completion of the 2-CdA treatments. 2-CdA is an active agent with acceptable toxicity for refractory or relapsed indolent lymphoma; however, prolonged myelosuppression and the potential development of MDS should be carefully monitored.
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PMID:Durable response but prolonged cytopenia after cladribine treatment in relapsed patients with indolent non-Hodgkin's lymphomas: results of a Japanese phase II study. 1554 Sep 3

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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PMID:Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms. 1554 84


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