A 71-year-old woman presented with a high-grade fever, neck pain, anemia and thrombocytopenia. After performing further examinations, we concluded that she had simultaneously developed large vessel vasculitis and myelodysplastic syndrome (MDS). Although glucocorticoid administration improved her clinical symptoms, the MDS transformed into acute myeloid leukemia and she died one year after receiving the diagnosis. The occurrence of immune-mediated disorders in patients with MDS is a well-known phenomenon; however, large vessel vasculitis is a rare complication of MDS. Our case suggests that the association between systemic vasculitis and MDS may result in poor outcomes.
Last. David Saadoun(UPMC: Pierre-and-Marie-Curie University)H-Index: 73
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Abstract Takayasu arteritis (TA) is a rare large vessels vasculitis. Conventional therapy consists of glucocorticoids which may be associated with other immunosuppressive drugs. However, some patients fail to achieve remission with conventional treatment. The use of anti-tumor necrosis factor-α (TNF-α) in patients with difficult to treat TA could be useful. We report here the main characteristics, treatment and outcome of 84 patients (5 personal cases and 79 patients from the literature) with re...
#2Go Haraguchi(Tokyo Medical and Dental University)H-Index: 19
Last. Mitsuaki Isobe(Tokyo Medical and Dental University)H-Index: 69
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Background: We aimed to describe the recent clinical characteristics of Takayasu arteritis (TA). Methods and Results: We enrolled 106 consecutive TA patients and compared the clinical characteristics of patients with TA onset before 1999 and after 2000, patients with onset at age less than 39 years and more than 40 years, patients with monophasic and relapsing-remitting clinical course, and patients with and without human lymphocyte antigen (HLA)-B52 allele. Among the patients with TA onset afte...
Last. Cristina Belizna(French Institute of Health and Medical Research)H-Index: 17
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Objective The presence of systemic and/or immune manifestations in myelodysplasia has been currently reported. The influence of these manifestations on the natural outcome of myelodysplastic syndrome has to be considered. We present a multicenter retrospective study (2002–2009) of patients with myelodysplastic syndrome disclosing systemic and/or immune manifestations. Methods Forty-six patients with myelodysplasia presenting with systemic and/or immune manifestations were compared in terms of su...
#1Matan J. Cohen(HUJI: Hebrew University of Jerusalem)H-Index: 15
#2Anat Shyman(HUJI: Hebrew University of Jerusalem)H-Index: 1
Last. Neta Goldschmidt(HUJI: Hebrew University of Jerusalem)H-Index: 24
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Abstract Autoimmune phenomena may complicate the course of myelodysplastic syndromes (MDS) but large vessel arteritis is a rare event. We report a case of large vessel arteritis in a patient with MDS. A 62-year-old male presented with thrombocytopenia and was diagnosed with low-risk MDS, (
Abstract: It has been recognized in recent years that some patients with myelodysplastic syndromes (MDS) develop immune-mediated complications (IMC), but little is known about the correlations to MDS-specific disease features. In a retrospective study of 82 MDS patients, we identified 10 (12%) with IMC (group A) and compared them to the remaining 72 cases (group B). Group A consisted of 5 patients with biopsy-verified skin vasculitis and 1 case each with temporal arteritis/polymyalgia rheumatica...
Accumulating evidence has shown that marrow failure in some patients with myelodysplastic syndrome is associated with autoimmunity, T-cell mediated myelosuppression and cytokine-induced cytopenias. In this perspective article, Drs. Barrett and Sloand expound on auto-immunity in myelodysplastic syndromes, in particular focusing on what has been learned from study of patients with trisomy 8. See related article on page 496.
BACKGROUND. The authors evaluated the long-term efficacy and side effects in patients with nonmetastatic, unilateral, inflammatory breast cancer (IBC) who received homogeneous treatment with intensive induction chemotherapy followed by a maintenance regimen. METHODS. One hundred twenty patients were randomized to receive high-dose fluorouracil, epirubicin, and cyclophosphamide (FEC-HD) (fluorouracil 750 mg/m2 on Days 1 to 4, epirubicin 35 mg/m2 on Days 2 to 4, and cyclophosphamide 400 mg/m2 on D...
Objective. To investigate serum profiles of inflammatory cytokines in patients with Takayasu’s arteritis (TA) and to determine their correlations with disease activity of TA. Methods. Forty-nine patients with TA and 12 age- and sex-matched controls were studied. Blood samples were obtained and were divided into active and stable disease groups. Paired blood samples were available in 19 patients at the active stage before treatment and at the remitted stage after treatment. Serum tumour necrosis ...
Background: To determine whether a dose-dense regimen improves outcome in early breast cancer patients, we compared outcomes with the same fluorouracil, epirubicin, and cyclophosphamide (FEC) chemotherapeutic regimen administered every 3 weeks (FEC 21 ) or administered every 2 weeks (FEC 14 including support with filgrastim, a granulocyte colony-stimulating factor) in a multicenter phase III randomized trial. Methods: A total of 1214 patients with early-stage breast cancer were randomly assigned...
Last. J. Gruber(University of Innsbruck)H-Index: 1
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: Myelodysplastic syndrome (MDS) is frequently associated with autoimmune diseases such as polymyalgia, arthritis, and rarely, with systemic vasculitis. The pathogenesis of these autoimmune complications remains unknown, but there is increasing evidence of profound immune dysregulation in MDS. In the few cases reported so far, vasculitides associated with MDS affected mainly cutaneous vessels. Here we describe two cases of acute large-vessel vasculitis in association with MDS. The first patient ...
Abstract Introduction Myelodysplastic syndromes (MDS) and MDS/myeloproliferative neoplasms (MDS/MPN) can be associated with giant cell arteritis (GCA). In this nationwide study by the “French Network of dysimmune disorders associated with hemopathies” (MINHEMON) the objective was to evaluate characteristics, treatment and outcome of GCA MDS-MDS/MPN. Patients and methods Retrospective analysis of patients that presented a MDS or MDS/MPN associated with GCA. Treatment efficiency, relapse-free and ...
Large-vessel vasculitis (LVV) affects the aorta and its major branches, and has two major variants: Takayasu arteritis (TAK) and giant cell arteritis (GCA). TAK is a chronic disease treated with gl...
Resume Introduction Des manifestations auto-immunes peuvent etre secondaire a des cancers, notamment au cours des hemopathies. Les vascularites des gros vaisseaux, associees aux hemopathies sont cependant rares, particulierement dans les syndromes myelodysplasiques. Observation Nous rapportons le cas d’un patient de 55 ans hospitalise pour bilan de fievre, amaigrissement, polyarthrites et syndrome inflammatoire. Les explorations hematologiques retrouvaient une anemie aregenerative (Hb : 7,8 g/dL...
Background Large vessel vasculitis is a rare disorder usually occurring in the context of the autoimmune conditions of giant cell arteritis and Takayasu’s arteritis. Case reports have described large vessel vasculitis occurring in individuals with myelodysplastic syndrome, preceding transformation to acute myeloid leukemia.
A number of autoimmune disorders, such as large vessel vasculitis, have been recognized in patients who have myelodysplastic syndrome (MDS) and myeloproliferative syndrome (MPS). The influence of an autoimmune disorder on the prognosis of patients who have MDS and MPS remains to be determined. We report three cases of myelodysplastic syndrome and large vessel vasculitis seen at our institution and provide a brief literature review of the two disease processes. More studies are needed to determin...