Acta haematologica
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Thirty-four cases of inherited bleeding disorders are reported. All are Saudi patients from the Eastern Province of Saudi Arabia. ⋯ Consanguinity was common among the families of these patients. Different age groups were affected and the severity of bleeding varied in the different conditions reported.
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Classification and differential diagnosis of erythroid neoplasias still are a matter of discussion. Eleven cases of primary acute erythremia were diagnosed between 1981 and 1984 at the Institute of Pathology, University of Kiel. Erythremia represented 0.5% of all hematological diagnoses and 1.0% of the myeloproliferative disorders. ⋯ Acute erythremia must be strictly distinguished from mixed erythroid/myeloid erythroleukemia and from secondary erythroid neoplasias, especially the erythremic 'blast crisis' of chronic myeloid leukemia or polycythemia vera rubra. Distinguishing the myelodysplastic variant of sideroblastic anemia from anerythremic acute erythremia can be extremely difficult. We discuss the differential diagnosis and classification of erythroid neoplasias based upon reproducible hematological criteria to facilitate the gathering and comparison of epidemiological and clinical data on these rare malignancies.
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Case Reports
Lymphadenopathy complicating hairy cell leukaemia. A case of disseminated Mycobacterium kansasii infection.
A patient with hairy cell leukaemia is described who developed progressive lymphadenopathy, which proved to be due to Mycobacterium kansasii infection. Clinicians should be alert to this rare but well-described complication of hairy cell leukaemia. Early lymph node biopsy is recommended in patients developing otherwise unexplained fever and lymph node enlargement.
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A significant reduction (30%) in the amount of postoperative blood loss from 1.12 to 0.70 ml/kg/h was noted in pediatric patients subjected to cardiopulmonary bypass for congenital heart disease surgery when the activated whole blood clotting time was utilized to monitor anticoagulant therapy. However, no difference in the incidence (11%) of postoperative hemorrhage was noted. Extensive preoperative laboratory hemostasis testing and routine immediate postoperative use of platelet transfusion and fresh frozen plasma (in cyanotic heart defects) failed to predict or correct those patients with significant postoperative hemorrhage.
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Case Reports
Thrombotic thrombocytopenic purpura in early pregnancy. Remission after plasma exchange.
A 24-year-old Chinese female developed fever, fluctuating neurological and renal manifestations, microangopathic hemolytic anemia, and thrombocytopenia during the 11th week of her first pregnancy. Therapy for thrombotic thrombocytopenic purpura (TTP), initiated during the 15th week of her pregnancy, including corticosteroids, platelet aggregation inhibitors, hysterotomy, and splenectomy was ineffective. However, dramatic improvement and remission occurred after plasma exchange of 3,560 ml was performed with the Haemonetics Model 30 Blood Cell Separator.