1/14. Myelodysplastic syndrome with monosomy 7 after immunosuppressive therapy in Behcet's disease.Only few cases of Behcet's and hematological malignancies have been reported until now. We recently observed a 39-year-old female patient with Behcet's disease developing a myelodysplastic syndrome (MDS) FAB subtype refractory anemia with excess of blasts in transformation [RAEB-t] with a monosomy 7 after being treated with cyclosporin A and chlorambucil for several years. This case is reported and the occurrence of hematological malignancies and Behcet's disease is reviewed.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
2/14. Behcet's disease associated with myelodysplastic syndrome: a case report.A rare case of Behcet's disease associated with myelodysplastic syndrome (MDS) is described. A 50-year-old Korean female suffering recurrent oral ulcer, genital ulcer, fatigue, arthralgia in both knees and fever was diagnosed as Behcet's disease. The findings of bone marrow aspirates were consistent with refractory anemia, a subtype of myelodysplastic syndrome. Chromosomal analysis of bone marrow cells revealed 46,XX,-8,-20, der(8)t(8;20)(p23;p10), der(8) t(8;20)(p23;q10)[30]. The chromosomal changes found in this patient were different from those of previous reports, which mostly revealed trisomy 8. If anemia, low reticulocyte count and dyspoietic cells are sustained in Behcet's disease, physicians should be alert to the possibility of MDS with aberration in chromosome 8 and perform a bone marrow study for the proper diagnosis and treatment of the disease. We presented a case of Behcet's disease associated with MDS, which is the first Korean case.- - - - - - - - - - ranking = 6keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
3/14. Intestinal Behcet's disease associated with myelodysplastic syndrome with chromosomal trisomy 8--a report of two cases and a review of the literature.Two cases of intestinal Behcet's disease, which developed in the state of myelodysplastic syndrome with trisomy 8, are presented. Both cases are included in the incomplete type of Behcet's disease, with recurrent aphthous stomatitis, skin lesions, genital ulcers or vascular involvement and punched-out ulcers in the cecum, without ocular involvement. The chromosomal analyses revealed chromosomal abnormalities, including trisomy 8, in both cases. Chromosomal trisomy 8 was shown in all 6 cases with the intestinal Behcet's disease associated with myelodysplastic syndrome reported previously, including our patients. Their histories indicated that myelodysplastic syndrome might have started before the development of intestinal Becet's disease. Theses findings suggested that chromosomal trisomy 8 might play an important role in the pathogenesis, at least in some groups, of intestinal Behcet's disease.- - - - - - - - - - ranking = 7keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
4/14. Atypical Behcet's syndrome in a patient with myelodysplastic syndrome.We report the case of a 67-year-old man with myelodysplastic syndrome (MDS), who presented with fever, painless penile and groin ulcers, as well as oral and esophageal ulcerations, all of which were exquisitely responsive to corticosteroids. Some cases of Behcet's syndrome and MDS have been reported in association with trisomy 8 and HLA B51, with the pathology varying from vasculitis to acute neutrophilic inflammation. Our patient with orogenital ulcers had neither trisomy 8 nor HLA B51. Also lacking were other features typical of Behcet's syndrome, such as uveitis, vasculitis, and central nervous system abnormalities. However, his response to corticosteroids on the initial presentation and subsequent episodes was dramatic. Esophageal ulcerations have not been described in Behcet's syndrome. In this respect, our patient was unique.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
5/14. trisomy 8 involved in myelodysplastic syndromes as a risk factor for intestinal ulcers and thrombosis--Behcet's syndrome.Only 12 myelodysplastic syndrome (MDS) cases with Behcet's syndrome have been previously reported and trisomy 8 was found to have accumulated in all these patients. Five of the cases had complications in the form of multiple intestinal ulcers, which is one of the symptoms of Behcet's syndrome. To investigate the relationship between trisomy 8 and multiple intestinal ulcers in MDS patients, we analyzed 46 MDS cases treated in our hospital over the last decade, and trisomy 8 was observed in eight of them. Three of these cases had complications of both multiple intestinal ulcers and thrombosis, and two cases showed episodes of thrombosis without intestinal ulcers. All these five cases featured trisomy 8, while the other 38 MDS patients without trisomy 8 had no episode of either intestinal ulcer or thrombosis. Two of the three cases suffering from multiple intestinal ulcers were treated with granulocyte-colony stimulating factor (G-CSF), which resulted in aggravation of the symptoms. Although the influence of G-CSF on such symptoms in MDS patients with trisomy 8 remains unclear, it seems advisable to exercise caution in the use of G-CSF when an MDS patient with trisomy 8 has intestinal ulcers or thrombosis.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
6/14. Massive hemorrhage in a patient with intestinal Behcet's disease: report of a case.Major gastrointestinal bleeding is a rare manifestation of intestinal Behcet's disease. We report herein the case of a 64-year-old man with intestinal Beh,et's disease complicated by myelodysplastic syndrome who suffered massive hemorrhage. colonoscopy demonstrated ulceration of the entire colon from the cecum to the rectum, characterized by punched-out ulcers. angiography demonstrated apparent extravasation of contrast material in the terminal ileum, and embolization was not successful. Continued and massive bleeding necessitated surgical resection of the involved segment of ileum; however, massive bleeding recurred. Re-endoscopy showed oozing hemorrhage from the multiple colon ulcerations. Intra-arterial prednisolone injection therapy was given, following which the melena gradually subsided and completely stopped within a few days.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
7/14. Successful cord blood stem cell transplantation for myelodysplastic syndrome with Behcet disease.A 10-year-old girl who had been treated as an outpatient for neurofibromatosis type 1 was admitted with fever, 10% weight loss, and abdominal pain that had persisted for 1 month. The clinical manifestations and the results of endoscopy led to the diagnosis of intestinal Behcet disease. Blood cell dysplasia appeared during the hospitalization. The patient subsequently developed blast cells, and the diagnosis of myelodysplastic syndrome (MDS) was made. Behcet disease was first controlled with steroids and diet followed by chemotherapy and cord blood stem cell transplantation (SCT). Both the MDS and Behcet disease went into remission after transplantation. The use of hematopoietic SCT has the potential not only to cure but also to help explicate the mechanism of Behcet disease.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
8/14. Resolution of Behcet's disease after HLA-mismatched unrelated cord blood transplantation for myelodysplastic syndrome.In 1991, a 27-year-old woman who presented with recurrent oral and genital ulcers, fever, and erythema nodosum was diagnosed with Behcet's disease (BD). Her symptoms were refractory to conventional therapy. In 1999, pancytopenia was noticed in this patient for the first time, and in 2000, her white blood cell count decreased to 0.94x10(9)/l with 1% myeloblasts and 24% neutrophils. bone marrow examination showed mild hypocellularity with 8% myeloblasts and 6% mature neutrophils with dysplastic features. A diagnosis of myelodysplastic syndrome (MDS)-refractory anemia with excess blasts was made. Despite marked neutropenia, the BD symptoms continued. Since her neutropenia worsened to 0.24x10(9)/l with 21% neutrophils, the patient underwent cord blood transplantation (CBT) from an unrelated donor in July 2001. Myeloid engraftment was documented on day 26. Grade I acute graft-versus-host disease occurred, but resolved spontaneously. Cyclosporin treatment was reduced gradually and discontinued 6 months after CBT. Twenty-three months after CBT, the patient is doing well and has no signs or symptoms of BD or MDS. These observations suggest that allogeneic hematopoietic stem cell transplantation, which encompasses CBT, may be an effective therapy in patients with high-risk aggressive BD.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
9/14. Constitutional trisomy 8 mosaicism with myelodysplastic syndrome complicated by intestinal Behcet disease and antithrombin iii deficiency.trisomy 8 is the most common acquired chromosomal abnormality associated with myeloid malignancy. As a constitutional trisomy 8 mosaicism (T8M), it exhibits an extremely variable phenotype. In addition, Behcet disease (BD) has been reported as an unusual complication of myelodysplastic syndrome (MDS). To our knowledge, 12 case reports of various hematologic malignancies in patients with T8M and 18 case reports of MDS with acquired trisomy 8 complicated by BD have been published to date. We report a case of constitutional T8M with MDS complicated by intestinal BD and antithrombin iii deficiency.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
10/14. Behcet's disease associated with malignancies. Report of two cases and review of the literature.OBJECTIVE: To investigate the incidence of malignancies in a cohort of Behcet's disease patients and review the world literature. methods: Our database of 128 patients was searched and the age standardized rate (ASR) for cancer was calculated. Furthermore, we performed a medline search from 1970 through 2003, as well as, a search in the proceedings of international conferences for cases of malignancies associated with Behcet's disease. RESULTS: Two of our 128 patients with Behcet's disease were found to have solid tumors. One male had lung cancer and the other female had kidney cancer. The ASR for cancer cases in our population was investigated and it was found to be 1,600 per 100,000 in 10 years. The ASR for cancer cases in greece according to WHO is 272.51 per 100,000 per year and therefore 2,725 per 100,000 in 10 years. In the world literature 112 cases of malignancies associated with Behcet's disease were found: Sixty five cases were of male patients and 46 of female with 1 case of unknown gender. The solid malignancies associated with Behcet's disease included cases of bladder, breast, uterus, thyroid and stomach cancer, whereas haematological malignancies included leukemia, myelodysplastic syndrome, lymphoma, multiple myeloma, Hodgkin's disease and lymphosarcoma. The treatment administered in these patients with their disease is also reported. CONCLUSION: The age standardized rate of cancer in our population was lower than that of the general population in greece, although the difference was not statistically significant. However, there is discrepancy in the world literature and the possibility of development of malignancies in Behcet's disease patients should not be ignored.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
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