Results of hematopoietic cell transplantation in pediatric leukemia

Hematopoietic cell transplantation [HCT] is an accepted treatment for acute myeloid leukemia [AML] in first remission, the treatment of choice for chronic myeloid leukemia [CML] and high risk groups of ALL who relapse with conventional chemotherapy. We assessed results of HCT for pediatric leukemia in our center. A total of 92 children, 63 with diagnose of AML, 23 with ALL and 6 with CML received allogeneic transplantation from HLA full matched siblings [57.6%] and autologous transplantation [42.4%]. Source of hematopoietic cells were peripheral blood 83.7%, bone marrow 15.2% and cord blood 1.6%. The median transplanted nucleated cells were 6.4 +/- 4.7 X 10[8] /Kg [body weight of patients] and mononuclear cells were 5.5 +/- 2.9 X 10[8]/Kg. The most common conditioning regimens were cyclophosphamide + busulfan. Prophylaxis regimen for GVHD was cyclosporin +/- methotrexate. GVHD occurred in 50 [54.3%] patients. Eighty five of children had engraftment, 26 [28.6%] relapsed and 57 [62%] are alive. The most common cause of death was relapse [68.6%]. Five years overall survival of patients with AML and ALL were 49% and 44% respectively and disease free survival of them were 52% and 49%. One year overall survival and disease free survival of CML was 57%. Overall survival increased with increasing age of patients at transplantation time [P = 0.06]. Longer survival significantly related to earlier WBC and platelet recovery [P < 0.0001 and P = 0.006 respectively]. Considering acceptable overall and disease free survival of patients after HCT, we concluded that is a good modality in treatment of leukemia of children

[A retrospective evaluation of epidemiologic and clinical parameters in Myeloma patients]

Myeloma is a hematologic malignancy in which there is monoclonal proliferation of bone marrow plasma cells that produce abnormal proteins. Reviewing the medical records of patients admitted to Dr.Shariati and Imam Khomeini Hospitals of Tehran University of Medical Sciences from 199 1-2000, we assessed distribution of epidemiologic and clinical parameters of patients with Myeloma and for each parameter we evaluated the missed data percentage as an index of active data collection. Among the epidemiologic parameters only age and sex had no missing data. For smoking, missing data were 22.9% and in other parameters there were more than 90% missing data. For clinical parameters, missing data percentages were between 46.2% and 93.1%. Establishing a documented system for object oriented data collection based on specific epidemiologic and clinical parameters is essential for optimum prevention and management of Myeloma

Frequency of BCR-ABL fusion transcript in Iranian patients with chronic myeloid leukemia

Reverse transcriptase-polymerase chain reaction [RT-PCR] assay is a useful tool for the detection of fusion transcript resulting from specific chromosomal translocation of the leukemia cells. A specific chromosomal abnormality, the Philadelphia chromosome [Ph], is present in 90% to 95% of CML patients. The aberration results from a reciprocal translocation between chromosome 9 and 22, creating a BCR-ABL fusion gene. There are two major forms of the BCR/ABL fusion gene, involving ABL exon 2, but including different exons of BCR gene. The transcripts b2a2 or b3a2 code for a p210 protein. Another fusion gene leads to the expression of an e1a2 transcript, which codes for a p190 pro-tein. Another, less common fusion genes are b3a3 or b2a3 [p203] and e19a2 [p230]. The incidence of one or other rearrangement in chronic myeloid leukemia [CML] patients varies in different reported series. In general, fusion transcripts are determined individually, a process which is labor intensive in or-der to detect all major fusion transcripts. This study was designed to determine the frequency of different fusion genes in 75 iranian patients with CML. peripheral blood samples were analyzed by multiplex reverse transcriptase poly-merase chain reaction [RT-PCR] from adult patients to detect all types of BCR-ABL transcripts of the t [9:22] and found that all cases were positive for some type of BCR/ABL rearrangement. Most of our patients showed b3a2 fusion gene [62%], while the remaining showed one of the transcripts of b2a2, b3a3, b2a3, e1a2 or coexpression of b3a2 and b2a2. The rate of coexpression of the b3a2 and b2a2 was 5%. In contrast to the other reports, we did not see any coexpression of p210/p190. This may reflect either the sensitivity of the detection techniques used or the possibility of genetic differences be-tween the populations studied. Coexpression may be due to alternative splicing or to phenotypic varia-tion, with clinical course different from classical CML

Primary gastric lymphoma: clinicopathologic study of gastric lyphoma cases and the treatment option of choice

Lymphoma may involve the gastrointestinal tract either primarily or as a manifestation of extensively disseminated systemic disease. Stomach being the most frequent site of primary gastroin-testinal lyphoma, followed by the small bowel and colon respectively [1 and 2 and 3]. For diagnosis of pi-mary small intestinal lymphoma [PSIL], one most satisfies the criteria specified by Dawson and co-workers.[5] Gastric lymphoma is a common presentation of non-Hodgkin's lymphoma. Controversy reigns about many aspects of its classification and management, especially regarding roles for surgical resection. The aim of this study is evaluation of 5 years survival and methods of treatment of primary gastric lymphoma in a group of Iranian patients. The authors review the clinical features, staging, pathology, prognosis, and management of 30 patients with an emphasis on the role of chemotherapy, surgical resection and radiotherapy of 71 gastrointestinal lymphoma cases. A total of 30 patients [19 male and 11 female] with a mean age of 51 years and a range of 34 - 68 years were included in the study. The frequency of primary gastric lymphoma in our series was 42% of the total of primary gastrointestinal lymphoma. The overall survival rate was 47.8% at 5 years. Stag-ing usually was completed using noninvasive techniques. Patients with stage I or II disease were treated with Surgery [gastric resection] and chemotherapy showed improved Free Disease Survival [FDS] of 67% at 5 years. The five-year survival for stage I, II, III and IV patients were 87%, 61%, 25%, and 11% respectively, and the five-year survival for low grade and high grade were 91% and 56%, respec-tively. Stage III or IV and inoperable primary gastric lymphoma were treated with chemotherapy and radiotherapy showed improved Free Disease Survival [FDS] of 67% at five years. The five-year sur-vival for stage I, II, III, IV were 87%, 61%, 25% and 11% respectively, and the five year survival for low grade and high grade were 91% and 56% respectively. Early stage disease and high-grade Lymphoma have a better prognosis and patients who have complete surgical removal of primary tumor and chemotherapy