Cytogenetic abnormalities in chromosomal number and structure are common in pediatric ALL and some have prognostic significance. One interesting association between cytogenetic status and treatment response involves the metabolism of methotrexate. Hyperdiploid lymphoblasts accumulate increased amounts of MTX and MTX polyglutamates, and they have higher basal apoptotic rates compared with leukemic cells with lower ploidy and normal cells. These characteristics may contribute to the better outcomes observed for patients with hyperdiploid lymphoblasts. A number of recurrent chromosomal abnormalities have been shown to have prognostic significance, especially in B-precursor ALL. Some chromosomal abnormalities are associated with more favorable outcomes, such as high hyperdiploidy (51-65 chromosomes) and the ETV6-RUNX1 fusion. Others are associated with a poorer prognosis, including the Philadelphia chromosome [t(9;22)], rearrangements of the MLL gene (chromosome 11q23), and intrachromosomal amplification of the AML1 gene (iAMP21).
Aims:The purpose of this analysis was to address the outcome of GTN from a tertiary care centre of India.Materials and Methods:We undertook a retrospective and prospective review of GTN cases treated at our centre from 2006 to 2014. Patients of GTN were assigned to low-risk or high-risk categories as per the FIGO scoring system. The low-risk group was treated with combination of actinomycin-D and methotrexate (MTX) and the high-risk group received the EMA/CO regimen. Salvage therapy was EP/TP. Treatment was continued for 3 cycles after normalization of β-hCG level, after which the patients were kept on follow-up.Results:In total, 52 GTN patients were treated at our institution during this period; 21 were low-risk and 31 were in the high-risk category. The lung was the most common site of metastasis. All low risk patients achieved complete remission. Among high risk patients one patient died while receiving first cycle chemotherapy, one patient relapsed and 29 patients achieved complete remission. The single relapsed patient also achieved remission with 2nd line chemotherapy.Conclusion:1. Two drug combination of Actinomycin-D and Methotrexate is a better alternative to single drug chemotherapy especially in developing countries were proper risk stratification is not always possible. 2. Patients with high disease burden should initially be treated with low dose chemotherapy to avoid life threatening visceral haemorrhage.
Introduction: Acute myeloid leukemia (AML) is a clonal accumulation of myeloid precursors in body tissues, which ultimately leads to bone marrow failure. This is an 8-year prospective, observational study in which 254 patients were enrolled. Aim of the Study: To document the clinical profile of AML and differential outcome in M3 versus non-M3 phenotype and to see impact of different variables on its survival. Methods: Patients enrolled in the study were examined, evaluated, and given standard 3:7 induction protocol, and acute promyelocytic leukemia (APML) patients were given the ICAPL 2006 protocol. Results: In our study, males outnumbered females and most of our patients were in 20-60 years of age group. The better prognosis was in patients who were in the second decade of life. Total leukocyte count and platelet count had a significant impact on the survival of the a patient. Bone marrow morphology of M3 type has extremely good prognosis and was the most common FAB type seen in our study. Flow cytometric markers such as CD15, CD33, CD117, and myeloperoxidase had positivity among 90% of patients. Overall survival is around 40% in whole-study group, 87% in APML group, and 16.5% in non-M3 group. There are still unmet needs in managing the non-M3 patients in resource-constraint countries where allogenic transplant and newer drugs have the least access. For improving the outcome in M3 AML, further newer molecules such as Flt3 and PIK3 inhibitors are being used in trials. Conclusion: There are still unmet needs in managing the non-M3 patients in resource-constraint countries where allogenic transplant and newer drugs have the least access. For improving the outcome in M3 AML, further newer molecules such as Flt3 and PIK3 inhibitors are being used in trials.
Pure red cell aplasia (PRCA) is a known entity in clinical medicine. Patients are often transfusion dependent for their whole life. Ascertaining its etiology is always a herculean task. We received a similar transfusion-dependent patient, who on evaluation was found to have thymoma as an etiological factor. Thymoma presenting as PRCA is seen in 2%–5% patients and evaluating PRCA for thymoma is seen in 5%–13% patient. As per the WHO histopathological classification, thymoma has six types and Type A is associated with PRCA and Type B is associated with myasthenia gravis. This correlation was not seen in our patient, who had Type B thymoma. Surgical resection of thymus improves 30% of PRCA and rest needs immunosuppression. Our patient was not the surgical candidate, and hence he was put on chemotherapy.
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