Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Granulocytic sarcoma is an extramedullary tumor consisting of immature cells of the granulocytic series known to occur in patients with myelodysplastic syndrome, chronic myelogenous leukemia, or acute myelogenous leukemia. This tumor may involve nodes, cervix, bone and periosteum, and infrequently the small intestine. Granulocytic sarcoma rarely occurs in the colon and has not been previously described endoscopically. We encountered a 73-year-old man with myelodysplastic syndrome who presented with fever, diarrhea, and abdominal pain. Colonoscopic evaluation (focal ulceration, friability, and nodularity) was compatible with Crohn's disease, although histology showed a dense myeloid cell infiltrate characteristic of granulocytic sarcoma. In patients with myelodysplastic syndrome or acute or chronic myelogenous leukemia presenting with diarrhea, abdominal pain, and/or fever, colonoscopy and biopsy are indicated to determine if the colon is affected by granulocytic sarcoma.
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PMID:Granulocytic sarcoma of the colon. 198 52

Crohn's disease (CD) has been occasionally reported to be associated with myelodysplastic syndromes (MDS) or acute myeloid leukaemia (AML). From October 1991 to June 1993, four AML patients with a previous history of CD but no history of MDS were referred to our institution. Three presented AML with a monoblastic proliferation (one AML4 and two AML5). Cytogenetic analysis revealed a normal karyotype in two patients, an inv(16) in another and a del(11)(q24-25) in the fourth. Interestingly, the three patients with AML4/AML5 had not received high doses of immunosuppressive agents, prior CD therapy ranging from 1 to 4 months sulfasalazine with additional azathioprine in one case.
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PMID:De novo acute myeloid leukemia in patients with Crohn's disease. 756 36

A 28-year-old man developed Crohn's disease and myelodysplastic syndrome concurrently. Chromosomal analysis of the bone marrow revealed a normal male karyotype. Subsequently, the myelodysplastic syndrome progressed to acute myelocytic leukemia. Several causes, including the medical treatment for Crohn's disease, chromosomal abnormalities, and a common underlying immune dysfunction, have been proposed as pathogenetic factors in the association with Crohn's disease of hematologic malignancies. This case suggests that neither medical treatment for Crohn's disease nor chromosomal abnormalities are inevitable causes of the development of hematologic malignancies associated with Crohn's disease. At present, the cause of the association remains unclear, although the idea of a common immune dysfunction is attractive.
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PMID:Simultaneous development of Crohn's disease and myelodysplastic syndrome progressing to acute myelocytic leukemia in a patient with a normal karyotype. 884 86

Somatic mutations of the adenomatous polyposis coli (APC) gene have been frequently found in sporadic colorectal tumors, and the frequency of such mutations remain constant as tumors progress from benign adenomas to malignant cancers. Thus the mutations of the APC gene may have a major role in the early development of sporadic colorectal tumors. Whether inactivation of the APC gene accounts for other types of primary tumors is still being investigated. We investigated for APC mutations within the mutation cluster region (a 684-bp region containing most of the mutations found in colorectal tumors) in 317 samples from a wide variety of human malignant and premalignant tissues, including 40 lung cancers, 47 renal cell carcinomas, 41 osteosarcomas and 21 other types of sarcomas, 45 acute lymphoid leukemias/lymphomas, 33 acute myeloid leukemias, 27 myelodysplastic syndrome samples, and 20 chronic colitis (ulcerative colitis and Crohn's disease) associated cancers and dysplasias, and 43 human malignant cell lines. We used single-strand conformation polymorphism assay following polymerase chain reaction. Samples with abnormal assay results were reamplified and analyzed by the direct DNA sequencing method. We detected a total of two cases with a base substitution. A silent mutation was detected in a case of myelodysplastic syndrome, and a novel nonsense mutation was discovered in a colorectal cancer cell line, SW837. In summary, we did not detect any functional mutations of the APC gene in a wide variety of tumors except for a colon cancer cell line, suggesting that alterations of the APC gene do not have a major role in the development of lung and renal cancers, various types of sarcomas, or hematological malignancies.
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PMID:Molecular analysis of the adenomatous polyposis coli gene in sarcomas, hematological malignancies and noncolonic, neoplastic tissues. 908 31

Familial Mediterranean fever (FMF) is an inherited disorder characterized by recurrent self-limiting attacks of joint, chest and abdominal pain associated with fever. The most serious complication in FMF is the development of amyloidosis, which usually leads to death from renal failure within a year. The use of colchicine has dramatically reduced this complication. We describe a 56-yr-old female patient with FMF in whom the arthropathy became the dominant clinical feature, resulting in the development of an erosive large and small joint arthritis during the course of the disease. The patient was treated with colchicine, but despite this, later developed amyloidosis confirmed on rectal biopsy, and chlorambucil was added to her treatment. For 10 yr, she also suffered intermittent abdominal pain and had terminal ileal changes suggestive of Crohn's disease. However, she was found to have ischaemic colitis at post mortem secondary to amyloidosis. Ischaemic bowel disease is an extremely unusual event in FMF. Other factors which may have contributed to the terminal ischaemia in this patient include anaemia secondary to blood loss and a drug-induced myelodysplasia, as well as hypotension during the final septicaemic illness. Clinicians should consider an ischaemic colitis as a possible differential diagnosis of abdominal pain in patients with FMF even in the absence of other clinical evidence of systemic amyloidosis.
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PMID:An unusual case of familial Mediterranean fever. 937 32

We report seven patients with both myelodysplastic syndrome (MDS) and inflammatory bowel disease (IBD): Crohn's disease in six cases, ulcerative colitis in one case. We describe their characteristics, and those of 10 previously published similar cases are presented here. Median age at diagnosis of IBD (61 years) was high, as compared to the usual age at diagnosis of IBD. IBD was diagnosed first in nine cases, MDS first in one patient, and both diseases were diagnosed simultaneously in seven cases. Concerning IBD, there was a strong predominance of Crohn's disease (15/17 cases), with an unusually high frequency of colonic involvement (11/15 cases). MDS, in 12/17 cases, showed no excess of marrow blasts. Cytogenetic analysis was abnormal in five of the 13 evaluable cases. These observations suggest that the association between MDS and IBD may not be fortuitous in some cases, and that, in particular, patients with IBD and anemia of nonobvious origin should be evaluated for MDS. The pathogenesis of those associations, however, remains unclear.
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PMID:Association between myelodysplastic syndromes and inflammatory bowel diseases. Report of seven new cases and review of the literature. 969 96

Besides cytopenia related to treatment, several hematological disorders such as anemia, abnormal platelet activity, thrombosis, presence of anticardiolipin or anti-neutrophil antibodies, cyclic neutropenia, and myelodysplasia, have been reported in patients with Crohn's disease (CD). The case we report here is the first one documenting the association of idiopathic thrombocytopenic purpura (ITP) with CD.
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PMID:Idiopathic thrombocytopenic purpura associated with Crohn's disease. 954 29

We report 3 patients who developed Crohn disease and myelodysplastic syndrome concurrently and review 9 previously reported cases of this association. Demographic and clinical features, treatment, and outcome are presented from previous reports and our own 3 cases. Of the 12 patients, 8 were men, and the mean age was 68.8 years (range, 28-83 yr). The geographic origin was heterogeneous. Pancytopenia was found in 5 patients, anemia in 5, and bicytopenia in 2. The classification types of myelodysplastic syndrome were variable. The marrow karyotype was reported in 10 patients: it was abnormal in 5, with chromosome 20 abnormalities in 3. Crohn disease and myelodysplasia were diagnosed simultaneously in 5 patients, whereas Crohn disease antedated myelodysplasia in 4 patients (interval, 8 mo-30 yr) and myelodysplasia antedated Crohn disease in 3 patients (interval, 2 mo-4 yr). Signs and symptoms of Crohn disease improved in most patients upon specific medical treatment. However, the outcome of myelodysplastic syndrome was not favorable in 10 of 11 patients in whom it was reported, with 4 deaths due to myelodysplasia-related complications. Notably, the activity of myelodysplasia in 1 of our own patients paralleled the clinical activity of Crohn disease, and hematologic abnormalities of the myelodysplasia resolved upon successful treatment of the Crohn disease, thus strengthening the hypothesis of a pathogenetic link between the disorders. The possibility of myelodysplastic syndrome should be considered in patients with Crohn disease over the age of 50 years who have peripheral blood cytopenias. Likewise, a diagnosis of Crohn disease should be considered in patients with myelodysplastic syndrome who develop gastrointestinal symptoms such as chronic and/or bloody diarrhea. In patients with such an association, the possibility of improvement of the myelodysplasia upon successful therapy of Crohn disease should not be ruled out.
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PMID:The association between Crohn disease and the myelodysplastic syndromes. Report of 3 cases and review of the literature. 985 99

Myelodysplastic syndromes (MDS) may be associated with inflammatory bowel disease (IBD). We characterized the clinical features and outcomes of patients with concurrent IBD and MDS. Using a diagnostic index, we identified all patients with both IBD and MDS at our center between 1976 and 1997. We also calculated an incidence rate of MDS in IBD using population-based data from Olmsted County, Minnesota, between 1950 and 1993. Among approximately 15,000 IBD patients seen, 25 (approximately 0.17%) were diagnosed with MDS. Fourteen had Crohn's disease and 11 had ulcerative colitis. The median age at diagnosis of IBD, particularly Crohn's disease, was higher than expected. Median age at diagnosis of MDS was typical. All but one ulcerative colitis patient was diagnosed before the diagnosis of MDS, while one-half of Crohn's disease patients were diagnosed with both ailments simultaneously. Five patients who had been diagnosed with IBD first were persistently anemic for at least 1 year prior to diagnosis of MDS. Two Crohn's disease patients had received purine analogs in the past. Median follow-up after MDS diagnosis was 1 year. Seven patients died, including two who progressed to acute myeloid leukemia. The incidence rate of MDS in IBD based on Olmsted County data was 0 cases per 100,000 person-years (95% CI, 0-55.2). The seemingly high frequency of myelodysplastic syndromes in a large referral-based group of patients with IBD suggests an association; however, an increased risk of MDS was not observed in a small regional cohort of IBD patients. Patients with MDS are diagnosed with concurrent IBD at an age older than expected. Simultaneous diagnoses were made in one-half of Crohn's disease patients. MDS should be considered in the differential diagnosis of anemia in IBD patients.
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PMID:Concurrent inflammatory bowel disease and myelodysplastic syndromes. 1033 78

An attempt has been made in this article to summarize the state-of-the-art clinical experience with the use of anti-TNF therapies in four diseased states with special emphasis on myelodysplastic syndromes. Given the central role of TNF-alpha in initiating and perpetuating the chronic damage produced in the diseased organs by controlling a cascade of pro-inflammatory cytokines, as well as its acute role in sepsis, theoretically speaking, neutralization of this peptide was a natural therapeutic choice. Results of the initial clinical trials appear encouraging and sometimes dramatic in their efficacy. The mechanism of response however, is interesting in that even when TNF-alpha is directly targeted by a monoclonal antibody, the resulting benefits can frequently not be attributed to TNF suppression alone. Rather, it appears that a more general effect on the T-lymphocytes is also contributing to the responses being seen. This raises the new possibility of combining anti-cytokine and anti-T-cell strategies to treat at least the more chronic diseases such as Crohn's disease and myelodysplastic syndromes. Continued clinical trials testing these strategies are clearly warranted.
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PMID:Anti-TNF therapies in rheumatoid arthritis, Crohn's disease, sepsis, and myelodysplastic syndromes. 1089 88


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