The survival of patients with acute leukemia who do not achieve a remission with primary therapy is very poor. High-dose chemoradiotherapy followed by allogeneic bone marrow transplantation (BMT) has been shown to be effective therapy for patients with acute and chronic leukemia. Therefore, we determined the long-term disease-free survival of patients who did not achieve a remission and were then treated with high-dose therapy and bone marrow allografting from matched sibling donors. Twenty-one patients (median age, 28 years) who did not achieve a remission with induction chemotherapy were subsequently treated with allogeneic BMT. After BMT, 90% achieved a complete remission. Six died of complications of the therapy, and six patients relapsed between 27 and 448 days after BMT. Nine patients (43%; median age, 25 years) are alive between 556 and 4,174 days after BMT. The cumulative probability of disease-free survival at 10 years is 43%. This study suggests that allogeneic BMT can be an effective therapy to achieve long-term control of acute leukemia, even in those patients who do not achieve a remission with primary therapy.

20 enzymatic activities and the glutathione content of newborn erythrocytes are compared (a) to normal and (b) to comparably reticulocyte-rich nonneonatal red cells. Six were very high in comparison to either control group (GSH, PGK, Enol., G-3-PD, GPI, G-6-PD). Five were very low (ACHE, RPK, GSH-Px, AK and PFK). The mean of the remainder differed from the mean of comparably reticulocyte-rich blood by less than 1 SD of the latter mean. Cord erythrocytes exhibit a characteristic metabolic pattern not explained by a young mean cell age alone.

FUNDAMENTO: O melanoma é o tumor cutâneo com maior taxa de mortalidade, apesar de representar apenas 5% do total. Sua incidência vem aumentando em todo o mundo, principalmente, entre indivíduos de pele clara; a cidade de Criciúma, onde predomina a etnia italiana, apresenta maior susceptibilidade a esse tipo de tumor. OBJETIVO: Conhecer o perfil epidemiológico e histopatológico do melanoma cutâneo primário, na cidade de Criciúma, e compará-lo com a literatura. MÉTODOS: Realizou-se um estudo retrospectivo, descritivo e transversal dos laudos anatomopatológicos de melanoma cutâneo primário, na cidade de Criciúma, entre janeiro de 2005 e dezembro de 2007. Analisou-se a população acometida segundo o sexo e a idade, a topografia, o tipo histológico, o nível de Clark e o índice de Breslow. RESULTADOS: Encontraram-se 72 laudos, com média de idade de 51 anos. A localização mais frequente foi o tronco, nos homens (60%), e os membros inferiores nas mulheres (30,5%). O tipo histológico mais comum foi o extensivo superficial (50%). Quanto ao nível de Clark, o mais frequente foi o nível III (32,3%). A maior parte dos melanomas apresentava Breslow in situ (29,6%). CONCLUSÃO: O perfil do paciente com melanoma cutâneo, em Criciúma, apresenta características semelhantes às descritas na literatura nacional e mundial. Quanto à topografia da lesão, o estudo mostra a importância de um exame clínico mais rigoroso , especialmente, do tronco em homens e dos membros inferiores em mulheres. A incidência de casos de melanoma foi maior que a média nacional e estadual. Por outro lado, os melanomas apresentaram um predomínio de Breslow, considerados de prognóstico mais favorável

A 29-yr-old black woman was found to have a long-standing, nonspherocytic hemolytic disorder associated with a marked reduction in the activity of erythrocyte ribosephosphate pyrophosphokinase (RPK, PRPP synthetase, E.C. 2.7.6.1). Although the patient’s erythrocytes had about 50% of the average RPK activity of normal mature human erythrocytes, this level represented only about 20-30% of the activity in comparable reticulocyte-rich blood samples from patients with other types of hemolytic anemias. The concentrations of adenosine triphosphate adenosine diphosphate, adenosine monophosphate and, therefore, of total adenine nucleotides in her erythrocytes were markedly increased, even well above the levels in extracts of comparable reticulocyte-rich blood samples. ATPase activity was increased three- to fourfold, consistent with the reticulocytosis. Adenylate kinase and adenine phosphoribosyltransferase activities were normal. The activities of all enzymes of the Embden-Meyerhof and hexose monophosphate shunt pathways and enzymes related to glutathione metabolism were normal or increased, consistent with the reticulocytosis. The concentrations of glycolytic intermediates, other than adenine nucleotides, were normal. The conversion of glucose, adenosine, and inosine to lactate was normal or increased. Autohemolysis was of the Dacie Type II. The concentrations of erythrocyte-reduced glutathione were high normal or elevated. The stained blood film showed a striking degree of basophilic stippling of the erythrocytes. Studies of the erythrocytes of the patient’s only known relative, a son, have failed to reveal any hematologic or enzymatic abnormalities. A direct causal relationship between RPK deficiency, high ATP concentrations, and nonspherocytic hemolytic anemia could not be derived from data now available. The final decision as to whether the deficiency is primary and causative or is an epiphenomenon requires investigation of additional cases.