Sobrevida e evolução leucêmica de portadores de síndromes mielodisplásicas / Survival and leukemic evolution of patients with myelodysplastic syndromes

As síndromes mielodisplásicas constituem um grupo de doenças de ordem clonal hematopoética evidenciadas por estudos em todo o mundo. A estimativa de sobrevida dos pacientes e de casos que apresentam evolução leucêmica requer investigação na população brasileira, pois não se conta com nenhum dado dessa natureza. Assim, este estudo objetivou caracterizar e estimar a sobrevida e evolução leucêmica de portadores de síndromes mielodisplásicas acompanhados em um serviço de referência. Trata-se de um estudo de coorte retrospectiva realizada de janeiro de 2000 a dezembro de 2010. Para análise descritiva foi utilizado o programa Epi Info 2002, versão 3.5.2, e para os cálculos das probabilidades de sobrevida foi empregado o método de Kaplan-Meier pelo uso do programa Statistic Package for Social Sciences (SPSS), versão 10.0. Os cálculos da probabilidade de associação entre as características analisadas e o gênero foram realizados pelos testes do qui-quadrado de tendência, de Fisher, Mann Whitney, e de Log Rank. O nível de significância considerado foi de 0,05. O trabalho foi aprovado em seus aspectos ético e metodológico pelo Comitê de Ética em Pesquisa do Hospital Universitário Onofre Lopes (HUOL), sob o Protocolo n. 432/10. Dos 29 pacientes selecionados, houve predomínio de idosos, do sexo masculino, com baixa escolaridade. Apresentaram baixa probabilidade acumulada de sobrevida e índices de evolução leucêmica em torno de 27%, sem nenhum resultado satisfatório para o tratamento quimioterápico, bem como nenhuma indicação de transplante de medula óssea como possibilidade de cura. Fazem-se necessárias pesquisas com populações maiores para caracterização em todo território nacional.

Myelodysplastic syndromes constitute a group of clonal hematopoietic diseases shown by studies all around the world. The survival estimation of the patients and the cases presenting leukemic evolution demand investigation in the Brazilian population, as there's no data with regard to this theme. Thus, this study aimed to characterize and estimate the survival and leukemic evolution of patients with myelodysplastic syndromes followed up in a reference service. This is a retrospective cohort study carried out from January 2000 to December 2010. The software Epi Info 2002, version 3.5.2, was used for descriptive analysis, and for the calculations of survival probabilities the Kaplan-Meier method was employed through the software Statistic Package for Social Sciences (SPSS), version 10.0. The calculations of the association probability between the characteristics analyzed and gender were performed using the chi-square for trend, Fisher, Mann Whitney, and Log Rank tests. The significance level was 0.05. The ethical and methodological aspects of the study were approved by the Research Ethics Committee of HUOL, under the Protocol 432/10. Out of the 29 selected patients, there was a predominance of elderly people, males, with low education. They showed low cumulative probability of survival and leukemic evolution rates around 27%, with no satisfactory outcome from chemotherapy, as well as no indication of bone marrow transplantation as a possible cure. There is a need for researches with larger populations for the characterization all over the national territory.

A Case of der(19)t(1;19) in Refractory Anemia with Ring Sideroblasts Associated with Marked Thrombocytosis

Translocation between chromosomes 1 and 19 is well documented in ALL. Here, we report a case of refractory anemia with ring sideroblasts associated with marked thrombocytosis with der(19)t(1;19). A 67-yr-old man was admitted to our hospital with anemia and thrombocytosis. The aspirated bone marrow showed erythroid and megakaryocytic hyperplasia and dyspoiesis. Iron staining showed that the ring sideroblasts increased in number. Bone-marrow cell karyotyping showed 46,XY,der(19)t(1;19)(q23;p13)[9]/46,XY,del(5)(q21)[2]/46,XY[9]. PCR analysis showed the absence of the TCF3-PBX1 rearrangement. The patient was treated with hydroxyurea and intermittent blood transfusion. It is known that t(1;19)(q23;p13) leads to a TCF3-PBX1 fusion gene, whose product is a powerful transcriptional activator that plays a key role in the development of ALL. However, t(1;19) has rarely been reported in myeloid neoplasms and the TCF3-PBX1 fusion gene has not been detected. This implies that other genes might be involved in the TCF3-PBX1 rearrangement, or an alternative TCF3-PBX1 fusion transcript with a different breakpoint has not been detected to date. Further research and case studies, including the use of molecular analysis techniques, are required to evaluate the clinical and prognostic significance of t(1;19) in the development of myeloid neoplasms.

A Strategy for Iron Refractory Anemia by Rikkunshi-to / 日本東洋医学雑誌

During the administration of Rikkunshi-to, hematological findings and complications of underlying disease were examined in patients with refractory anemia treated with oral iron preparations. In all 11 evaluative cases, hemoglobin levels before the administration of Rikkunshi-to were 10.7±0.8g/dL, and 11.5±0.8g/dL post-administration (mean administration period 17.3±11.6 months), indicating a significant hemoglobin level increase. In 9 out of 11 cases (82%), an improvement in anemia was observed. Eight out of 11 cases (73%) had atrophic gastritis, reflux esophagitis, or other gastrointestinal disease complications. Rikkunshi-to is considered to be effective such factors as: an improvement in hematopoiesis through a promotion of DNA synthesis in bone marrow cells with prostisol; and an improvement in iron absorption and utilization with improving appetite, although, the exact mechanisms have yet to be elucidated.<br>In conclusion, Rikkunshi-to can be considered an appropriate medication for the improvement of refractory anemia treated with the administration of oral iron preparations. However, its mechanism of action, as well as its recommended administration for specific disease states remain important issues for the future.

A case of myelodysplastic syndrome after oral methotrexate overdose / 대한임상병리학회지

Methotrexate is a very potent inhibitor of dihydrofolate reductase and causes bone marrow suppression and megaloblastic anemia. It is widely used in combination with other chemotherapeutic agents in lymphoproliferative disorders. A 63 year old man with ischioneuralgia developed exertional dyspnea and dizziness after he had intentionally taken methotrexate in doses of 5mg per day for 2months. Five months after discontinuation of methotrexate, his bone marrow showed the hypercellular marrow with 90% cellularity, 15% blasts and marked dysgranulopoiesis, suggestive of refractory anemia with excess blasts(RAEB). The hematopoietic cells were not enough aspirated for proper diagnosis in follow up bone marrow after three months. The bone marrow aspirates showed 13% blasts, and marked dysgranulopoiesis. The bone marrow biopsy showed hypercellular marrow with 100% cellularity, but marked fibrosis was developed. The cytogenetic study revealed normal karyotype.

The Clinical Effects of Ren-Shen-Yang-Rong Tang (Ninjin-youei-to) on Refractory Anemia / 日本東洋医学雑誌

In order to study the clinical efficacy of Ninjin-youei-to (Ren-shen-yang-rong-tang) the authors administered it to five cases of aplastic anema (AA) and two cases of primary acquired refractory anemia (PARA). It was found to be clinically effective in three of the AA cases and one case of PARA. The percentage of improvement of anemia and platelet counts were 43% and 57%, respectively, but no effects were seen on the white blood cell count. This suggested that the mechanism of effect of this Chinese herb was to not only stimulate the stem cell directy, but also to activate the stromal cells of the microenvironmental system indirectly.