Demographics and Outcome of Philadelphia-positive ALL in a Pediatric Population in North India: a Single-center Experience.

Philadelphia-positive (Ph+) acute lymphoblastic leukemia (ALL) in children had a worse outcome before the use of tyrosine kinase inhibitors. We have evaluated the demographics and outcome of Ph+ ALL patients treated with imatinib without blood marrow transplantation. Of the 206 children with ALL registered for treatment, the demographic data of 15 Ph+ ALL patients were compared with the remaining Ph- patients. Imatinib (340 mg/m) was started on day 5 (D5) of induction in Ph+ patients, and their overall survival was compared with Ph- high-risk patients treated on similar protocols. Statistical analysis was carried out by the Fisher exact test and the t test. The Kaplan-Meier test was used for survival analysis. Philadelphia positivity noted in 15/206 (7.28%) ALL patients was higher than reported earlier. Median initial total leukocyte count and central nervous system positivity were significantly higher in Ph+ patients. Myeloid markers, CD13 and CD33, were also positive in 33.3% Ph+ patients. D15 and D35 marrow showed remissions in a larger proportion of Ph+ ALL, as compared with Ph- patients, but chemotherapy interruptions and neutropenic deaths were significantly higher after starting imatinib, as compared with Philadelphia high-risk patients. Overall survival was similar in Ph+ and Ph- high-risk ALL patients. Ph+ ALL, noted in 7.28%, presented with high initial white blood cell counts, high central nervous system positivity, poor steroid response, and higher induction deaths, as compared with high-risk Ph- ALL, and raised the question about the appropriate dose and time of introduction of imatinib to prevent toxicity.

Gallbladder empyema complicating acute myeloid leukaemia in an adolescent boy.

Gallbladder (GB) empyema is an uncommon complication in acute myeloid leukaemia (AML). Non-specific signs and symptoms and rarity of disease in AML make it difficult to diagnose early. We report a case of 13-year-old boy who had AML and developed GB empyema in the neutropaenic period. The patient was managed with antibiotics, ultrasound-guided tapping and interval cholecystectomy and recovered well. GB empyema is a life-threatening complication which should be kept as a possibility while evaluating patients with neutropaenia with pain abdomen localised to right hypochondrium as early diagnosis and treatment can lead to better outcomes.

Outcome of extraocular retinoblastoma in a resource limited center from low middle income country.

Retinoblastoma (RB) is the most common ocular malignancy in children, and is managed by multimodal treatment. There is a paucity of data regarding the clinical profile and outcome of children with extraocular retinoblastoma from Low Middle Income Countries (LMIC) including India. Case records of children with newly diagnosed extraocular RB from January 2013 to August 2016 treated at our unit were analysed for clinical profile, treatment, and outcome. Over the 44 month study period, 91 children were diagnosed with RB, out of which 41 had extraocular disease. While 26 children had extraocular spread limited to orbit (IRSS stage III), 15 had a distant spread to brain (IRSS stage IV). Median lag period for diagnosis was eight months. Treatment abandonment rates were 38.5% and 46.6% in International Retinoblastoma Staging System (IRSS) stage III and IV respectively. With a median follow up of 31.5 months, the projected overall survival for IRSS III at one, two, and three years was 87.5%, 55.6%, and 39.7%. All patients with stage IV disease died after a median follow up duration of three months. High treatment abandonment rates and limited availability of resources lead to suboptimal survival in children with extraocular RB from LMIC. Initiatives aimed at improving early diagnosis, so that the disease is detected in the intraocular stage, are critical to improve the survival in children with RB.