Xeroderma pigmentosum (XP) is an autosomal recessive disorder caused by defect in nucleotide excision repair and is associated with increased incidence of skin cancer. Lymphoblastic leukemia in XP is rare. We report a case of a 11-year-old boy with XP, who developed B – Acute lymphoblastic leukaemia (ALL). He was started on Modified BFM (Berlin-Frankfurt-Münster) ALL Protocol. Tolerance to ALL chemotherapy was good in our XP patient except for the transient derangement of liver function tests during induction, Pseudomonas sepsis and oral mucositis during protocol M and hyperbilirubinemia during maintenance chemotherapy. Though rare, children with XP requires to be investigated for acute leukaemia on development of bicytopenia.

Introduction: L‑Asparaginase is an essential chemotherapeutic agent in the therapy of Acute Lymphoblastic Leukemia (ALL), which has led to improvement in survival. In low and middle-income countries like India, the outcomes in ALL are inferior compared to the published literature, one of the cause of which are believed to be due to the inferior quality of bioequivalent Asparaginase. The following survey attempts to understand the practice of using this agent among oncologists treating children with cancer in our country. Methods: The researchers designed a structured online questionnaire comprising 25 aspects of L-Asparaginase usage in the study. The questionnaire was directed to the healthcare providers involved in treating children with cancer in India. Results: Of the total 80 responses recorded, 51 (64%) respondents had more than five years of experience in pediatric oncology and were treating at least 5-10 newly diagnosed ALL patients per month. Forty-one (51%) respondents utilized Native Asparaginase, and 21 (26.3%) oncologists used PEGylated-Asparaginase exclusively. The most common route of administration was the intramuscular route (66.3%). Seventy percent of respondents utilized Native form at a dose of 10,000IU/m 2 and 20% at 6000IU/m 2. The amounts used for PEGylated L-Asparaginase were 1000IU/m 2, 2500IU/m 2, and variable doses in 48%, 40%, and 10% of responses, respectively. Though Serum Asparaginase assay (SAA) was not measured routinely in most of the centres, 39 (48.8%) healthcare providers perceived performing SAA helps to make the clinical decision. Conclusion: This survey shows a wide variation in L-Asparaginase usage among healthcare providers caring for children with cancer in our country. As L-Asparaginase is the pivotal component of ALL therapy, uniformity in its usage and dosing with the possibility of monitoring SAA due to the quality of bioequivalent may be one of the critical steps towards improving outcomes in ALL in our country.

Corticosteroids and L-asparaginase used in the treatment of pediatric acute lymphoblastic leukemia (ALL) results in Drug induced Diabetes Mellitus (DIDM). Literature on the management of DIDM among children with ALL is sparse and the diagnostic criteria for pediatric diabetes should be carefully applied considering the acute and transient nature of DIDM during ALL therapy. Insulin remains the standard of care for DIDM management and the choice of Insulin regimen (standalone Neutral Protamine Hagedorn (NPH) or basal bolus) should be based on the type and dose of steroids used for ALL and the pattern of hyperglycemia. A modest glycemic control (140-180 mg/dl) to achieve euglycemia and prevent hypoglycemia would be the general approach. This review is intended to suggest a evidence based practical guidance in the diagnosis and management of DIDM during pediatric ALL therapy.

Background: Pediatric B-Lymphoblastic lymphoma(pB-LBL) is a rare entity, and appropriate treatment for pB-LBL is not well defined. While intensive Acute Lymphoblastic leukemia(ALL) type regimens achieve long term event free survival of 90% across western co-operative group trials, published data from Asian studies on long term outcomes in pB-LBL are scarce. We evaluated the outcomes and prognostic factors of pediatric B-LBL patients treated at our center. Methods: We retrospectively analyzed the data of pediatric B-LBL patients treated between January 2010 and December 2017 on a uniform protocol(modified BFM 90). Patients were evaluated for early response post-induction and monitored for toxicity and long term outcomes. Kaplan-Meier method was used to estimate the event free survival(EFS) and overall survival(OS). Cox regression models were performed to identify prognostic factors. Results: Of 21 patients who received treatment on the modified BFM 90 protocol, 17(81%) were alive in remission, 3(14%) had relapse, and 1(4%) had treatment-related mortality(TRM) while in remission. Two of 3 relapsed patients subsequently expired. With a median follow-up of 66 months(range 6–114), 5-year Event free survival(EFS) and overall survival(OS) were 80%(95% CI:71–89%) and 91% (95% CI:85–97%), respectively. While delayed presentation (≥3 months) had inferior EFS(p-0.030), patients with elevated baseline Lactate Dehydrogenase(LDH) had a worse OS(p-0.037). Age, gender, site of origin, stage, and post-induction response had no bearing on outcome. Conclusions: Outcomes of pB-LBL patients treated on modified BFM 90 protocol are excellent. Higher disease burden manifested by elevated baseline LDH and delayed presentation(≥3 months) portend poorer survival.