Aim: To analyze clinical manifestations, course and outcome of Langerhans Cell Histiocytosis in children in resource limited settings lacking salvage therapy. Study design: Observational retrospective study Place and duration of study: Department of Haematology/Oncology, The Children’s Hospital, Lahore Pakistan from 1st January 2011 to 31st December 2018. Methodology: Sixty-five patients with age range from<1 to 8 years included analysing their age, gender, clinical classification, course of therapy and outcome. The major treatment was composed of either prednisolone and vinblastine or cytarabine pulses. Results: There were 59% males and 41% females. Forty-seven (72%) patients presented with multi system-LCH with 49% Risk Organ involvement. Most of them 42 (65%) had bone lesions while 15patients (23%) presented with central nervous system involvement. Forty patients (61%) have completed treatment, 11(17%) left against medical advice and 12(18%)patients expired due to progressive disease and worsening infection. Only 2 patients were put on palliation with progressive brain parenchyma disease. 22 patients (34%) had reactivations of disease requiring therapy for more than one-year (p-value=0.06), while 15 (23%) patients received two cycles of initiation therapy before continuation therapy started. The treatment initiated >6 months after the onset of symptoms in 48 (74%) patients. Conclusion: Early diagnosis and timely initiation of therapy are of utmost importance to reduce mortality and morbidity. There is a dire need of social support to reduce treatment abandonment in low-middle-income countries LMIC. Keywords: Paediatric Langerhans cell histiocytosis, Resource-limited settings, Delayed diagnosis, Abandonment
Introduction: Wilms tumor is the most common pediatric renal neoplasm. Following preoperative therapy, various histological subtypes, risks and clinical stages are determined as per following SIOP 2001 protocol. To determine the clinicopathological features of different histopathological subtypes and stages of Wilms tumor after completing course of preoperative as well as postoperative chemotherapy following SIOP 2001 protocol. Methodology: This is a retrospective cross-sectional study and sample size calculated by non-probability technique. Ninety-three patients with abdominal masses in the Hematology/Oncology Department were included. After radiological and histopathological diagnosis, four weeks of preoperative chemotherapy, given to patients, followed by partial and radical nephrectomies by surgical team. Specimens received in Histopathology Department grossed and microscopically examined for different post chemotherapy histological subtypes. Further risk categorization and clinicopathological staging in accordance with SIOP 2001, done after completion of treatment. Patients called for yearly follow up for the next five years. Categorical variables are presented as frequencies and percentages. Results: Mean age of ninety-three children was 44.4 months + 30.92with predominance of males (55.9%) and more common in right sided kidney (55.9%). Majority patients completed the entire course of treatment (77.4%). Majority were intermediate risk tumors (76.3%) and most common histological subtype was Mixed Tumor subtype (23.4%). In our study majority, tumors were stage III (48.3%) and patients died due to febrile neutropenia (9.6%) Conclusion: In our study the majority of patients completed the entire course of treatment and relapse was fairly less. Patients lost to follow up after nephrectomy and deaths at home caused by febrile neutropenia were our major challenges.
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