Acta haematologica
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Review Case Reports
Myelofibrosis and systemic lupus erythematosus: reversal of fibrosis with high-dose corticosteroid therapy.
A case of myelofibrosis in association with systemic lupus erythematosus (SLE) is reported. Acute thrombocytopenia and a bleeding tendency developed in a 24-year-old woman with SLE. Bone marrow aspiration was unsuccessful due to myelofibrosis. ⋯ A review of the literature revealed that the coexistence of SLE and myelofibrosis is a rare occurrence. Only 7 cases, to our knowledge, have ever been reported in detail. The present case is the 3rd in which myelofibrosis was reversed by corticosteroids.
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Congenital severe dysfibrinogenemia was discovered in a small Saudi family. Their single child exhibited abnormal severe bleeding tendency since birth and his coagulation profile revealed evidence of severe dysfibrinogenemia. ⋯ The child presented with large cephalohematoma and evidence of intracranial hemorrhage and left hemiparesis. He was treated with cryoprecipitate and his hematoma resolved, but his neurological deficit remained.
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The association between thrombotic thrombocytopenic purpura (TTP) and autoimmune hematological conditions is reported in 2 patients. In a 35-year-old man, acute autoimmune hemolytic anemia (AIHA) was diagnosed in 1960; until 1965 he was free of disease, when he abruptly developed TTP and failed to respond to blood transfusions and corticosteroids. ⋯ Four years later the patient was admitted with acute catastrophic signs and symptoms of TTP and failed to respond to plasmapheresis and plasma transfusions. The present case reports of associations between AIHA and AITP with TTP support the connection of the latter with abnormalities of the immune system.
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A case of dapsone-induced severe haemolytic anaemia and agranulocytosis is described. A possible common pathogenic mechanism for the simultaneous occurrence of these side effects of dapsone therapy in a patient with normal glucose-6-phosphate-dehydrogenase activity is proposed.
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Randomized Controlled Trial Clinical Trial
Transcutaneous electrical nerve stimulation treatment of sickle cell pain crises.
Transcutaneous electrical nerve stimulation (TENS) has been used in a variety of acute and chronic painful conditions, but has not been studied in sickle cell pain crises. We compared TENS versus placebo in a randomized, double-blind, cross-over study involving 60 trials in 4 crisis severity categories. ⋯ Patients assessments of overall treatment efficacy indicated that TENS was more frequently helpful, but there was a substantial placebo effect. Although the value of TENS in this condition was not established in this study, further trials, using varied stimulation parameters, are warranted.