Acquired pure megakaryocytic aplasia report of two cases with long-term responses to antithymocyte globulin and cyclosporine.

Acquired pure megakaryocytic aplasia is a rare disorder defined by severe thrombocytopenia with no other hematologic abnormalities and absent, or severely decreased marrow megakaryocytes. The etiology may be immune suppression of megakaryocyte development. Two patients are described who both responded rapidly to a combination of antithymocyte globulin and cyclosporine and who remain in remission 13-20 months after discontinuation of cyclosporine. This regimen is well described for treatment of aplastic anemia and may also be effective for acquired pure megakaryocytic aplasia.

Treatment of acute lymphoblastic leukemia in adults with intensive induction, consolidation, and maintenance chemotherapy.

The Southwest Oncology Group conducted a study of acute lymphoblastic leukemia (ALL) in adults over a 5-year period, testing the utility of the L-10M regimen initially described by the group from Memorial Sloan-Kettering Cancer Center. One hundred sixty-eight eligible patients were treated with this intensive combination chemotherapy regimen. One hundred fifteen (68%) achieved complete remission. With the current median follow-up time of 34.5 months, the median durations of remission, relapse-free survival, and overall survival were 22.9, 20.9, and 17.7 months, respectively. Only 35% of the patients over 50 years of age achieved a complete remission. Age was a significant prognostic factor for complete response, survival, relapse-free survival, and remission duration. In addition, a low initial WBC count was found to have a statistically significant association with longer remission duration. Responders between the ages of 20 and 49 years with WBC counts of less than 15,000 appear to have an exceptionally good prognosis.

Social and public health implication of water supply in arid zones in the Sudan.

The quantity and physical, chemical and bacteriological quality of water from wells, tap and zeers in Port Sudan and from wells, rectified and unrectified hafirs in South Kordofan Province were investigated. The relationship between water quantity and quality and prevalence of water-related diseases was also assessed. Both areas are semi-arid and tragically suffer from shortage of water. The average per capita consumption in Port Sudan was about 1001, and about 301, in South Kordofan. All water sources in both areas were invariably contaminated with coliforms. In South Kordofan the provision of water was primarily the responsibility of women and children. Depending on the crowds and the distance of the water source people spend between 3-5 hours per day carrying water. 50% of the families have to cover more than 2 km to reach the water source. It was evident that hafirs rectification increased the amount of water stored. Tap and zeer (home pots) water in Port Sudan was also contaminated with coliforms. The content of suspended and dissolved solids and turbidity of hafirs water was exceptionally high, which warrants proper protection and water treatment before distribution to the public. Scarcity of water rather than bacterial contamination was the cause of alarmingly high prevalence of diarrhoeal, skin and eye communicable diseases (water-washed diseases) among children and adults of Port Sudan and South Kordofan Province. However, it is plausible to suggest that even minor improvements on the provided quantity of water will reduce the prevalence of water-washed diseases.

Management of adult acute leukemia. A Southwest Oncology Group study.

Five hundred forty three adult patients with acute leukemia were entered on the study designed to: (1) test efficacy of vincristine and prednisone used as primary drug therapy in patients with good prognosis as judged by a circulating blast cell count less than 30,000 cm2; (2) investigate the use of either simultaneously or sequentially administered Adriamycin plus cytosine arabinoside plus vincristine and prednisone; and (3) to assess the use of bacillus Calmette-Guerin (BCG) in the maintenance phase on both the response and duration of response. Complete remissions were seen in 21% of patients with the vincristine and prednisone arm. Complete remission rates were similar in both the simultaneously and sequentially administered chemotherapy with overall complete remission rates of 55%. Median durations of complete remission and survival were 35 and 62 weeks, respectively, for patients with AML; and 47 and 75, respectively, for patients with ALL. Toxicity was within acceptable limits. BCG administered during the maintenance phase of therapy caused no differences in duration of complete remission and survival. These results demonstrate an improved response and duration of response over previous studies done by this group.

Acute leukemia in idiopathic sideroblastic anemia: response to combination chemotherapy.

Three patients with idiopathic sideroblastic anemia of variable duration developed acute leukemia. In two the leukemia was morphologically and histochemically myeloblastic, in one lymphoblastic. With combination chemotherapy remission was achieved in all three. The remission inductions were complicated by long periods of bone marrow suppression and the duration of remissions was brief (3, 2 and 3 months). Survival after diagnosis was 13, 10 and 9 mo, respectively. The ring sideroblast abnormality persisted during the leukemic and remission phases and transfusion requirements remained unaltered in the two patients with transfusion dependent anemia throughout their courses.

Autoimmune thrombocytopenia and peripheral neuropathy heralding Hodgkin's disease.

A patient with an autoimmune thrombocytopenic syndrome was treated unsuccessfully with splenectomy. Treatment with corticosteroids and 6-mercaptopurine was partially successful, but the patient developed peripheral neuropathy and over signs of Hodgkin's disease. The latter reponded completely to radiation therapy, but the thrombocytopenia was not reversed until combination chemotherapy was given. The association of thrombocytopenia with Hodgkin's disease is reviewed.