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

Three patients with acute febrile neutrophilic dermatosis (AFND, Sweet's syndrome) in association with myelodysplastic syndromes (MDS) are described and, in addition, another 10 published cases are reviewed. In the reviewed patients the male/female ratio is 2:1. The great majority of the patients had decreased or normal leucocyte counts at the time the syndrome developed. Chromosome analysis was available in eight cases: in three the karyotype was normal while five patients had a clonal abnormality. No clinical or laboratory parameters seemed to predict the response to steroids: all but one of the treated patients responded promptly. Moreover, three patients recovered spontaneously. Shortly after the appearance of Sweet's syndrome nine patients developed acute leukaemia and one patient died with hypoplasia. Development of Sweet's syndrome in association with MDS may not be an uncommon clinical finding. The occasional MDS patient developing AFND will often progress into acute leukaemia and thus the occurrence of AFND warrants a bone marrow examination and close follow-up of the patient.
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PMID:Acute febrile neutrophilic dermatosis (Sweet's syndrome) in association with myelodysplastic syndromes: a report of three cases and a review of the literature. 267 62

A 49-year-old man was hospitalized because of cutaneous plaques and pancytopenia. Hematological findings, and the skin eruption suggested Sweet's syndrome associated with myelodysplastic syndrome (refractory anemia with excess of blasts; RAEB). Treatment for pancytopenia was attempted without effect. Also we tried treatment with antibiotics. The skin lesions healed and the body temperature returned to normal. This case was unusual in the association of myelodysplastic syndrome with Sweet's syndrome.
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PMID:[Sweet's syndrome associated with myelodysplastic syndrome]. 279 88

Sweet's syndrome is known often to associate with non-lymphocytic leukemia (ANLL); however, there have been very few reports of Sweet's syndrome associated with myelodysplastic syndrome (MDS). It was reported that improvement and exacerbation of these two syndromes occurred simultaneously. We present here a 49-year-old male with Sweet's syndrome developed in RAEB in T. He complained of fever and infiltrative eruptions on the trunk and legs. At the time of admission to Tsukuba University Hospital, the peripheral blood showed leukocytopenia (WBC 2,000/microliter: Blast 9%, PMN 51%) and anemia (Hb 6.5 g/dl). Pseudo-Pelger anomaly of neutrophils was found on the blood smear. From the hematological findings and the result of skin biopsy, the patient was diagnosed as having MDS (RAEB in T) complicated by Sweet's syndrome. Prednisolone was effective to improve his fever and eruptions. However, when treated with low-dose Ara-C and when transformed into acute myelogenous leukemia, there was no correlation between the condition of Sweet's syndrome and the percentages of blasts in the marrow. We suggest that eruptions of Sweet's syndrome associated with MDS are not always a good index of exacerbation of MDS.
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PMID:[Appearance of Sweet's syndrome in a patient with myelodysplastic syndrome (MDS) without relation to the hematological findings of MDS]. 279 96

An unusual case of disseminated pustular eruption associated with polycythemia vera is described. This eruption can be included in the group of neutrophilic dermatoses of myeloproliferative disorders (pyoderma gangrenosum, Sweet's syndrome). A defect in superoxide anion (O2-) generation is detected. The fact that both the clinical condition and the neutrophil function improved with the use of dapsone suggests that the O2- generation defect may play a role in the pathogenesis of the eruption. From an evolutionary standpoint, the occurrence of a neutrophilic dermatosis in polycythemia vera could be an unfavorable sign of preleukemia.
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PMID:Disseminated pustular dermatosis in polycythemia vera. Relationship with neutrophilic dermatosis of myeloproliferative disorders: study of neutrophil function. 283 82

Sweet's syndrome is an acute febrile neutrophilic dermatosis in which approximately 20% of the reported patients have an associated cancer. We review the 79 patients with malignancy-associated Sweet's syndrome documented in the world literature. The most common underlying neoplasm was acute myelogenous leukemia (AML). Lymphomas, chronic leukemias, myelomas, myelodysplastic syndromes, and a variety of solid tumors have also been observed. The onset of Sweet's syndrome either preceded or coincided with the discovery of a previously undiagnosed cancer in greater than 60% of malignancy-associated Sweet's syndrome patients. In contrast to patients with the idiopathic form of the disease, those with a malignancy often presented with more severe cutaneous lesions, cytopenias, and/or immature cells in the peripheral blood. Extracutaneous sites of involvement included the eyes, muscles and joints, kidneys, lungs, and liver. All the manifestations of Sweet's syndrome improved dramatically with corticosteroid therapy, regardless of the response of the associated neoplasm to tumor-directed therapy.
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PMID:Malignancy-associated Sweet's syndrome: review of the world literature. 305 78

A new case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with a malignant hemopathy is presented. The blood disease was a chronic myelomonocytic dysmyelopoiesis which was discovered during the eruption and resulted in the patient's death within a few months, probably through acutization. The skin lesions were atypical, bullous and ulcerated. On this occasion, the international literature concerning all cases of Sweet's syndrome associated with malignant or premalignant hemopathies is reviewed. Several concepts emerge from this study: the association is frequent (about 20 p. 100 of all published cases of Sweet's syndrome); there is a strong predominance of granulocytic hemopathies over lymphoplasmocytic and monocytic hemopathies; the blood disease is revealed by the skin eruption in some 50 p. 100 of the patients; there are frequent chronological relations between Sweet's syndrome and the events that occur in the course of the hemopathy; finally, the association is usually of poor prognosis. A comparison with Sweet's syndrome unassociated with a blood disease showed only three significant points: the frequency of bullous lesions, of the initial anaemia (the most important element) and of extreme figures in leucocyte counts (leucopenia or major hyperleukocytosis). The atypical character of the skin lesions in the patient presented here incites to discuss the nosological relationship between Sweet's syndrome and bullous pyoderma, an entity closely associated with hemopathies. It has recently been suggested by several authors that this anatomico-clinical kinship should be turned into a wide spectrum of acute neutrophilic dermatoses, with typical Sweet's syndrome at one end and Pyoderma gangrenosum at the other end. The interface between this spectrum and haemopathies seems to be maximum at its intermediate stage: the bullous and superficially ulcerated lesions. The aetiology and pathogenesis of this new nosological entity are uncertain. The presence of chemoattractants or of polymorphonuclear cell abnormalities is still open to discussion. The relationship between the entity and leukocytoclastic vasculitis has recently been questioned.
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PMID:[Acute febrile neutrophilic dermatosis and malignant hematologic diseases: report of a new bullous case and review of the literature]. 305 44

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is characterized by pyrexia, neutrophilia, and the abrupt appearance of erythematous, painful, cutaneous plaques, primarily on the upper extremities, head, and neck. Histologically, the salient feature is a dense dermal infiltrate of neutrophils. Approximately 10 to 15 percent of published cases of Sweet's syndrome occurred in patients with cancer. This report reviews the 39 patients with malignancy-associated Sweet's syndrome described in the world literature and compares Sweet's syndrome in cancer patients with the idiopathic form of the disease. The most common associated malignancy was acute myelogenous leukemia. However, other myeloproliferative disorders, lymphoproliferative disorders, myelodysplastic syndrome, and carcinomas have been observed. Importantly, the diagnosis of Sweet's syndrome was often the presenting sign of a new or recurrent tumor. The presence of anemia, abnormal platelet counts, immature cells in the differential, and/or severe vesiculobullous or ulcerative cutaneous lesions is infrequent in idiopathic Sweet's syndrome and should alert physicians to the possibility of a more serious underlying disease. Extracutaneous manifestations may occur and most often involve the musculoskeletal system. Response to systemic steroids is dramatic in virtually all patients, regardless of the presence of malignancy.
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PMID:Sweet's syndrome and malignancy. 330 Mar 6

Acute febrile neutrophilic dermatosis (AFND) is sometimes associated with leukemia. We present a patient with myelodysplastic syndrome who subsequently developed AFND, and we briefly review the association of AFND and myeloproliferative disorders.
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PMID:Neutrophilic dermatosis and myelodysplastic syndrome. 343 97

Acute febrile neutrophilic dermatosis (ND) consists of the abrupt onset of red, tender, cutaneous plaques on the face, extremities, and upper trunk, accompanied by fever, malaise, and neutrophilic leukocytosis. Histologically, there are distinctive, dense, dermal infiltrates of neutrophils. Response to systemic steroids is dramatic. This report describes four patients with leukemia or preleukemia and ND (LND), reviews reports of 12 similar patients, and compares LND with ND in otherwise healthy individuals (idiopathic ND, IND). Although lesions of LND more frequently had vesiculobullous appearances or location on mucous membranes, this study showed no consistent difference between LND and IND with regard to cutaneous signs, symptoms, histologic findings, and response to therapy. The first episode of LND either preceded or followed documentation of the myeloproliferative disorder, and the most common associated hematologic conditions were acute myeloid or myelomonocytic leukemia. Moderate to severe anemia was present in nine of ten patients whose first episode of LND preceded the discovery of the hematologic condition by eight months or less. The presence of anemia is the most obvious and readily detectable difference between LND and IND. The possibility of an underlying myeloproliferative disorder should be considered in all patients with ND, and LND should not be confused with infectious complications in patients known to have myeloproliferative disorders.
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PMID:Acute febrile neutrophilic dermatosis (Sweet's syndrome) and myeloproliferative disorders. 657 88

A patient with aplastic anaemia developed Sweet's syndrome (a febrile neutrophilic dermatosis) during granulocyte colony-stimulating factor (G-CSF) therapy. Three repeated episodes of appearance and disappearance of erythematous nodules after administration and withdrawal of G-CSF confirmed that G-CSF induced Sweet's syndrome in the patient. Sweet's syndrome has been reported in patients with myelodysplastic syndrome and acute leukemia, but not in patients with aplastic anaemia. This is the first report of a patient with aplastic anaemia who developed G-CSF-induced Sweet's syndrome.
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PMID:Sweet's syndrome during therapy with granulocyte colony-stimulating factor in a patient with aplastic anaemia. 751 39


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