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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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, necrotising panniculitis, Hashimoto's thyroiditis, autoimmune thrombocytopenia, and Sweet's syndrome. Survival times, sex ratio and distribution of MDS subtypes were similar in the two groups. The patients in group A were younger than those in group B (median 66 vs. 76 years, p < 0.01). Four patients (40%) in group A had a history of previous genotoxic therapy for malignant disorders. The bone marrow karyotype was evaluated in 62 patients. Clonal chromosomal abnormalities were found more frequently in Group A than in group B (8/9 vs. 26/53, p = 0.03), and complex karyotypes, i.e., three or more aberrations, were also observed to be more common in group A (3/9 vs. 8/53). The results indicate that IMC preferentially develop in patients with secondary MDS, in younger MDS cases, and in patients with cytogenetic abnormalities.
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PMID:Immune-mediated complications in patients with myelodysplastic syndromes--clinical and cytogenetic features. 761 49

The clinical course of a 56-year-old female patient with Sweet's syndrome (SS) preceded by a myelodysplastic syndrome (MDS) is described. During the acute phase of the disease with high remittent fever, painful skin lesions and maximal leucocytosis IL-6 and G-CSF serum levels were extremely high, while TNF-alpha was only slightly elevated and gamma-interferon and IL1-beta were not increased. On clinical improvement IL-6 serum levels rapidly fell, whereas G-CSF values already slightly elevated before the manifestation of the disease slowly declined. High G-CSF levels triggered by a yet unknown factor could explain the leucocytosis, neutrophilic dermatosis and skin lesions in SS, while IL-6 probably induced the associated clinical symptoms of fever and pain.
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PMID:Sweet's syndrome associated with myelodysplasia: possible role of cytokines in the pathogenesis of the disease. 752 52

We describe three patients with primary myelodysplastic syndrome and immunological manifestations. One had painful cutaneous plaques, arthritis and peripheral neuropathy, another showed cutaneous vasculitis, and a third had Sweet's syndrome. All patients responded dramatically to steroids. Before treatment the patient with Sweet's syndrome had high serum levels of interleukin 6 (IL-6), which were reduced to zero after treatment with steroids. Myelodysplastic syndrome should be considered when evaluating patients with immunological or rheumatic manifestations associated with one or more cytopenias.
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PMID:[Immunologic manifestations in myelodysplastic syndrome]. 807 62

We analyzed the relationship between autoantibody and dermatosis in 22 patients with myelodysplastic syndrome (MDS). These MDS patients consisted of five cases with refractory anemia (RA), three RA with ringed sideroblasts (RARS), eight RA with excess of blasts (RAEB), four RAEB in transformation (RAEB-t), and two chronic myelomonocytic leukemia (CMMoL) according to the FAB classification of MDS. The autoantibody was detected in seven patients, of whom four had rheumatoid factor (RF) and three had antinuclear antibody (ANA). Neither RF-positive nor ANA-positive MDS patients had other autoantibodies. Dermatosis was observed in nine cases of these 22 MDS patients. Five of 7 MDS patients (71%) with autoantibody developed dermatosis in their clinical course, as did four of 15 MDS patients (27%) without autoantibody. All four MDS patients with RF had dermatosis such as anaphylactoid purpura, xerotic dermatitis, thrombophlebitis, ephelides, and genital herpes. One of three MDS patients with ANA had pruritus senilis. The four MDS patients without autoantibody had dermatosis such as erythema nodosum, ichthyosis vulgaris, Sweet syndrome, and thrombophlebitis. Three of four MDS patients with RF had normal liver function tests, while three MDS patients with ANA showed liver dysfunction. Our studies presented here suggested that the dermatosis could develop frequently in MDS patients with autoantibody and that RF was closely related to development of dermatosis in MDS patients, although the dermatosis is not specially fixed.
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PMID:Relationship between autoantibody and dermatosis in myelodysplastic syndrome. 815 7

The clinical and hematological characteristics of ten children with myelodysplastic syndromes diagnosed and followed up over a 3 year period are presented. All of them had anemia and a low platelet count whilst the white blood cell count was variable. Presentation with bilateral proptosis and acute febrile neutrophilic dermatosis (Sweet's syndrome) were unique features observed in one case each. None of these cases could afford specific therapy and thus serve to illustrate the natural history of the disease in pediatric practice.
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PMID:Clinico-hematological profile and natural history of childhood myelodysplastic syndromes. 826 94

This is the first report of the rare association of acute febrile neutrophilic dermatosis (Sweet's syndrome) and myelodysplastic syndrome (MDS) in a child. The skin lesions showed a dramatic response to colchicine.
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PMID:Acute febrile neutrophilic dermatosis (Sweet's syndrome) in myelodysplastic syndrome. 829 19

A cutaneous or systemic vasculitis occurs in myelodysplasia as well as in myeloproliferative and lymphoproliferative disorders. The most common lesion is a leucocytoclastic vasculitis, with neurological or joint involvement occurring less often. The vasculitis may appear contemporaneously with or precede the clinical onset of the blood dyscrasia. Occasionally the lesions respond dramatically to the use of steroids but in general, patients with vasculitis have a worse prognosis than those with uncomplicated myelodysplasia. Vasculitis and myelodysplasia appear together too often for the association to be coincidental and the vasculitis in most cases cannot be attributed to intercurrent infections, therapeutic agents or a pre-existing rheumatological disorder. While autoantibodies are frequently present in myelodysplasia, and ANA and anti-neutrophil cytoplasm antibodies (ANCA) are found in other vasculitides, neither of these antibodies is associated with the vasculitis of myelodysplasia. There has however been one report of ANCA in Sweet's syndrome a non-vasculitic skin condition that also occurs in the myelodysplastic syndromes.
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PMID:Myelodysplasia, vasculitis and anti-neutrophil cytoplasm antibodies. 847 1

Several adverse effects have been reported to occur after clinical application of all-trans retinoic acid (RA) in acute promyelocytic leukemia (APL). Except for severe side effects including retinoic acid syndrome, the mechanism of action of RA on adverse effects remains unclear. Here we describe some rare adverse effects and their management. We reviewed the English literature, and we added our cases of endocrine and metabolic adverse effects, such as hypercalcemia, male infertility, bone marrow necrosis, fibrosis and acute pancreatitis. We also described our cases of thromboembolic events, RA-dependent growth of pathologic cells including Sweet's syndrome, erythema nodosum, hyperhistaminemia, granulomatous proliferation, and mild cases of pulmonary complications. In addition, we reviewed the efficacy of RA administration for other types of leukemia or myelodysplastic syndrome. RA and chemotherapeutic agents might induce complete remission, but we obtained a response in only one case of M2 in the third relapse. During RA administration the patient should be monitored for these adverse effects, and early diagnosis and appropriate treatment are important.
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PMID:Rare but important adverse effects of all-trans retinoic acid in acute promyelocytic leukemia and their management. 922 Jun 56

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is reported to be a marker for underlying malignancy. Much of the evidence for this is based on case reports, small series of cases and reviews of the literature. In order to clarify the association with malignancy and determine the common clinical features of Sweet's syndrome, we reviewed the case notes of patients presenting to six dermatology units in the U.K. Eighty-seven cases of histologically proven Sweet's syndrome were reviewed. Fourteen patients (16%) developed associated malignancy, predominantly haematological, two patients (2%) had a history of previous malignancy and four patients (5%) had premalignant conditions (monoclonal gammopathy, two: myelodysplasia, two). Malignancy developed up to a year after presentation with Sweet's syndrome. Patients with associated malignancy were more likely to be anaemic (P < 0.01) at presentation, had a lower mean platelet count (207 x 10(9)/L vs. 332 x 10(9)/L; P < 0.003) and were, on average, older (59 years vs. 49 years; P = 0.002). Contrary to previous reports, a greater percentage of females developed malignancy than males.
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PMID:Sweet's syndrome and malignancy in the U.K. 939 Mar 41

A 64-year-old woman presented with cardiomegaly, Sweet's syndrome, and refractory anemia (RA), and died of sudden cardiac arrest. The autopsy revealed a perivascular and myocardial infiltration by neutrophils, which could be responsible for the cardiomegaly and probably had caused disturbances in the conduction system leading to sudden cardiac arrest. Myocardial infiltration by functionally defective neutrophils can develop in a patient with myelodysplastic syndrome (MDS) without peripheral neutrophilia or leukemic blood picture and needs a special diagnostic and therapeutic consideration.
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PMID:Neutrophilic infiltration of the myocardium in a patient with myelodysplastic syndrome. 969 1


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