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The present study describes a 7-year-old male child who had attended the Pediatric Oncology Clinic of the Regional Cancer Centre, Thiruvananthapuram, Kerala, India, and was pathologically confirmed to have B-Acute Lymphoblastic Leukemia (B-ALL). Conventional cytogenetics analysis at diagnosis showed the presence of a double Philadelphia chromosome and the karyotype of the case was 47, XY, t(9;22)(q34;q11.2), + der(22)t(9;22). FISH, done as a molecular confirmation of the translocation, t(9;22)(q34;q11.2), and this case showed an additional fusion signal that confirms the presence of double Ph. As far as we are aware, this represents the initial and only occurrence of an abnormality report regarding the double Philadelphia chromosome in pediatric B-ALL within India. The double Philadelphia chromosome in B-ALL has a very poor prognosis despite aggressive treatment with chemotherapy. This study reveals the importance of conventional and molecular cytogenetic analysis in risk stratification and prognosis prediction of pediatric B-ALL. The risk stratification based on the conventional and molecular cytogenetic analysis may be taken into consideration for deciding the treatment strategy for each patient. [ABSTRACT FROM AUTHOR]

We report here a 7-year girl with B-Acute Lymphoblastic Leukemia (ALL) on Berlin Frankfurt Munster (BFM) based induction chemotherapy who presented with fever, cough, and painful necrotic skin lesions simulating pseudomonas sepsis. The patient was eventually diagnosed with disseminated fusariosis. While on combination antifungal therapy, fever reappeared with pancytopenia and hepatosplenomegaly, and she was subsequently diagnosed with secondary Hemophagocytic lymphohistiocytosis (HLH) and was treated using the HLH 2004 protocol. The child responded to treatment well. This report highlights the high index of clinical suspicion, appropriate investigations needed to diagnose fusariosis and secondary HLH in pediatric oncology practice promptly, and the successful treatment outcome despite having them both. [ABSTRACT FROM AUTHOR]

Methotrexate containing chemotherapy is less commonly used for treatment of osteosarcoma in resource limited settings. We present our experience with the administration of high dose methotrexate (HDMTX) containing chemotherapy over a period of three years. Children between 1 and 14 years of age with newly diagnosed nonmetastatic extremity osteosarcoma, registered in Pediatric Oncology Department of a tertiary care cancer center in South India between 1st January 2016 and 31st December 2018 and receiving MAP chemotherapy were included. Patients in this study received HDMTX at 12 g/m2. Twenty two patients were included. After neoadjuvant chemotherapy, 20 patients underwent surgery (limb salvage surgery in 16, amputation in 4). The median time from initiation of chemotherapy to surgery was 97.5 days. Eighteen of 22 patients (81.8%) completed planned chemotherapy in our cohort, one patient was lost to follow up after progression and three patients required change of chemotherapy due to toxicities. Of a total of 227 cycles of HDMTX infusions in 22 patients, delayed clearance occurred in 22 cycles (9.7%). Major toxicities were myelosuppression (30 episodes in 17 patients), blood stream infections (24 episodes in 15 patients) and mucositis (15 episodes in 10 patients). Hearing loss was documented in 7 patients. There was no treatment related mortality. Chemotherapy was completed in a median duration of 38.5 weeks. Administration of high dose methotrexate containing chemotherapy is feasible in pediatric patients with osteosarcoma, even in resource limited settings, if there are facilities for hydration, determination of methotrexate levels and good supportive care. [ABSTRACT FROM AUTHOR]

Rasburicase is a novel drug used during the management of tumor lysis syndrome. In countries with limited resources, it is frequently given at a lower dose and only for the treatment of established tumor lysis syndrome and not as prophylaxis. A retrospective study was conducted in the department of pediatric oncology at a tertiary referral oncology center in south India to analyze the use of rasburicase over the past 3 years. Data of all the 18 children (< 14 years of age) who were given rasburicase for the management of hyperuricemia were collected and analyzed. With a mean rasburicase dose of 0.085 mg/kg, hyperuricemia was managed efficiently without the need for a hemodialysis in 16 children (88.8 %). The fall in mean serum uric acid levels after the administration of a single dose of rasburicase at 4, 24, and 48 hours was 31.18 %, 64.8 %, and 74.5 %, respectively. Rasburicase efficiently decreases the uric acid levels to a normal level within a short period. In resource-limited settings, rasburicase at a lower dose is a promising option for managing hyperuricemia in the event of a tumor lysis syndrome. [ABSTRACT FROM AUTHOR]

Precursor B-cell lymphoblastic lymphoma (LBL) occurring as a primary bone tumor is a rare clinical presentation in children, and data regarding this condition are limited to small case series or a few individual case reports. We report two pediatric cases of precursor B-cell LBL of the bone. The tumor cells from bone biopsies of both patients were seen to be positive for leukocyte common antigen (LCA), terminal deoxynucleotidyl transferase, CD10, CD20 and weakly positive for CD99. Bone marrow studies were normal. They were treated according to the modified Berlin-Frankfurt-Münster acute lymphoblastic leukemia high-risk protocol. The first patient has completed treatment including local radiotherapy and has been disease free for the past 10 years. The second patient has recently completed treatment and shows good response. [ABSTRACT FROM AUTHOR]