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)

Erythromelalgia is caused by platelet-mediated acral inflammation and arteriolar thrombosis in thrombocythemia in its primary form or associated with polycythemia vera. The prompt and lasting relief of burning pain by low-dose aspirin is a prerequisite for the diagnosis of thrombocythemic erythromelalgia. Here we extend observations on the occurrence of erythromelalgia in thrombocythemia associated with primary myelofibrosis, Philadelphia-chromosome positive micromegakaryocytic myelofibrosis, and myelodysplastic syndrome type II. It is concluded that erythromelalgia may occur in thrombocythemia of all variants of chronic myeloproliferative disease as well as myelodysplastic syndrome if platelet counts are sufficiently high.
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PMID:Erythromelalgia in thrombocythemia of various myeloproliferative disorders. 834 46

Erythromelalgia, which is specific for primary thrombocythaemia or polycythaemia with thrombocythaemia, is reported in a case of primary myelofibrosis at platelet counts of between 350 and 450 X 10(9)/l. In addition, the unexpected occurrence of thrombocythaemic erythromelalgia associated with Ph1 chromosome positive micromegakaryocytic myelofibrosis and with myelodysplastic syndrome type II is described. Therefore it is concluded that erythromelalgia may occur in all variants of myeloproliferative disease as well as myelodysplastic syndrome as long as they present with thrombocythaemia.
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PMID:Thrombocythaemic erythromelalgia in chronic myeloproliferative and myelodysplastic disorders. 277 94

Polycythaemia vera (PV) and essential thrombocytosis (ET) are clinically characterised by non-specific neurologic symptoms, peripheral circulatory disturbances (acrocyanosis, wounds, erythromelalgia) or abdominal symptoms. The treatment of PV includes phlebotomy, antiaggregation and cytoreduction. In ET, the primary treatment is also low-dose aspirin except for patients presenting with a haemorrhagic diathesis. Hydroxyurea may be associated with an increased risk of acute leukaemia or myelodysplasia. Therefore alpha-interferon and anagrelide should be considered in younger patients. Early cytoreductive therapy is advocated in patients with idiopathic myelofibrosis (IMF) to inhibit further progression of bone marrow fibrosis and further expansion of myeloid metaplasia in the spleen and liver. Treatment with androgens (danazol) and glucocorticoids may improve severe anaemia and thrombocytopenia. In younger patients, allogeneic bone marrow transplantation should be considered.
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PMID:[The chronic Philadelphia chromosome-negative myeloproliferative syndrome II. Clinical findings, diagnostics and treatment]. 1137 60

Erythromelalgia is a rare syndrome that is characterized by episodic attacks of burning pain in the distal extremities, which last from minutes to days and are precipitated by exercise, warmth, or limb dependency. There is a primary or idiopathic form and a secondary form that occur with myeloproliferative or other diseases. All previous reports about erythromelalgia that is the result of a myeloproliferative process have documented associated thrombocytosis. We describe a 40-year-old woman with myelodysplastic syndrome who experienced erythromelalgia in the setting of acral erythema and thrombocytopenia, first induced by chemotherapy and recurring after a radiation- and chemotherapy-based myeloablative regimen that was administered before a T-cell-depleted allogeneic bone marrow transplantation.
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PMID:Erythromelalgia precipitated by acral erythema in the setting of thrombocytopenia. 1278 98

Essential thrombocythemia (ET) is an acquired myeloproliferative disorder (MPD) characterized by a sustained elevation of platelet number with a tendency for thrombosis and hemorrhage. The prevalence in the general population is approximately 30/100,000. The median age at diagnosis is 65 to 70 years, but the disease may occur at any age. The female to male ratio is about 2:1. The clinical picture is dominated by a predisposition to vascular occlusive events (involving the cerebrovascular, coronary and peripheral circulation) and hemorrhages. Some patients with ET are asymptomatic, others may experience vasomotor (headaches, visual disturbances, lightheadedness, atypical chest pain, distal paresthesias, erythromelalgia), thrombotic, or hemorrhagic disturbances. Arterial and venous thromboses, as well as platelet-mediated transient occlusions of the microcirculation and bleeding, represent the main risks for ET patients. Thromboses of large arteries represent a major cause of mortality associated with ET or can induce severe neurological, cardiac or peripheral artery manifestations. Acute leukemia or myelodysplasia represent only rare and frequently later-onset events. The molecular pathogenesis of ET, which leads to the overproduction of mature blood cells, is similar to that found in other clonal MPDs such as chronic myeloid leukemia, polycythemia vera and myelofibrosis with myeloid metaplasia of the spleen. Polycythemia vera, myelofibrosis with myeloid metaplasia of the spleen and ET are generally associated under the common denomination of Philadelphia (Ph)-negative MPDs. Despite the recent identification of the JAK2 V617F mutation in a subset of patients with Ph-negative MPDs, the detailed pathogenetic mechanism is still a matter of discussion. Therapeutic interventions in ET are limited to decisions concerning the introduction of anti-aggregation therapy and/or starting platelet cytoreduction. The therapeutic value of hydroxycarbamide and aspirin in high risk patients has been supported by controlled studies. Avoiding thromboreduction or opting for anagrelide to postpone the long-term side effects of hydrocarbamide in young or low risk patients represent alternative options. Life expectancy is almost normal and similar to that of a healthy population matched by age and sex.
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PMID:Essential thrombocythemia. 1721 76