Evaluation of Baseline Cardiac Function by Echocardiography and its Association With Nutritional Status in Pediatric Cancer Patients at The Indus Hospital in Karachi, Pakistan.

INTRODUCTION: Evidence on conducting baseline echocardiogram before starting chemotherapy in pediatric cancer patients is limited from developing countries where malnutrition and infections are common and which may result in cardiac dysfunction. MATERIALS AND METHODS: A prospective, observational study was conducted from October 2016 to May 2017 at The Indus Hospital, Karachi, Pakistan, among children 1 to 16 years of age suffering from cancer. Echocardiography was performed before starting chemotherapy. Associations between body mass index and cardiac abnormalities were studied. RESULTS: A total of 384 children met the inclusion criteria. The median (interquartile range) age was 8.0 (5.0 to 12.0) years and 62.0% (n=238) were male individuals. Twenty-two of 384 (5.7%) children had systolic dysfunction. Four of 22 had moderate-systolic and one of 22 had mild systolic dysfunction, for whom the therapy was altered, and they were treated without anthracyclines. Four of these 5 patients died, and only 1 of 5 survived through high-risk protocol. Seventeen of 22 children had low-normal systolic dysfunction. We found no evidence of an association between body mass index for age and abnormal left ventricular ejection fraction and abnormal fractional shortening (P-trend=0.587; 0.487, respectively). No associations were found of weight-for-age and height-for-age with these outcomes. CONCLUSIONS: In developing countries, echocardiograms should be expeditiously performed and technology made more accessible to rule out cardiac dysfunction and avoid delay in chemotherapy. Malnutrition was not associated with cardiac dysfunction.

Clinical Characteristics and Cytogenetics of Childhood Acute Lymphoblastic Leukemia in a Single Center in Pakistan.

Objectives. This study aimed to find the association between clinical characteristics, cytogenetics, and post-induction outcomes of childhood acute lymphoblastic leukemia. Methods. The study was conducted at the Indus Hospital in Karachi. Initial total leukocyte count (TLC), cytogenetics, CNS status, and post-induction remission status were recorded. Results. Out of 108 children diagnosed with ALL, 66 (61.1%) were male and 42 (38.9%) were female. The majority 90 (83.3%) had B-ALL. CNS1 status was observed in 76 (84.4%) B-ALL and 18 (88.9%) T-ALL. All T-ALL and 89 (98.8%) B-ALL achieved remission post-induction. In B-ALL, 50 (55.5%) had a normal diploid karyotype, and 22 (24.4%) had numerical abnormalities. No typical gene rearrangement was observed in 66 (73.3%), 11 (12.2%) had BCR::ABL1, 10 (11.1%) had ETV6::RUNX1 and 3 (3.3%) KMT2A on FISH. No significant difference was observed between cytogenetics and clinical characteristics (P > .05). Conclusion. The study provides valuable data on childhood acute lymphoblastic leukemia in the Pakistani population.

Infantile T-cell Acute Lymphoblastic Leukaemia: A Case Report.

Introduction: Acute lymphoblastic leukaemia (ALL) is the most common malignancy in children, with a male predominance. Paediatric ALL is usually of B-cell lineage; T-cell leukaemia is uncommon and extremely rare under 1 year of age. Mixed-lineage leukaemia gene rearrangement is the best-known hallmark of infantile leukaemia and is a poor prognostic indicator. While multiagent high-dose chemotherapy remains the first line of treatment for paediatric T-cell lineage ALL (T-ALL), there are numerous side effects of these regimens, and most patients undergo relapse. Due to the rarity of the disease, treatment protocols for infantile T-ALL have not been established to date. Clinical Description: We present a case of a 7-month-old Pakistani male that presented with fever and cough and was subsequently diagnosed with T-cell ALL. T-ALL was diagnosed on flow cytometry. Due to poor prognosis, the patient was assigned palliative care. Practical Implications: Management of infantile leukaemia has yet to be studied in-depth. With a lack of clear treatment guidelines, the approach toward these patients remains challenging. Further research and clinical trials in this area of study are paramount to improving clinical outcomes for these young patients.

Pilomatrix Carcinoma In A 4-Year-Old Child With An Unusual Presentation.

Pilomatrix carcinoma is a rare, locally aggressive variant of pilomatrixoma with a high rate of recurrence and risk of distant metastasis. We report an unusual presentation of a pilomatrix carcinoma in a 4-year-old male child who presented with recurrent lesions on his left cheek. At the age of 1 month of life, he presented with a soft tissue swelling on his left cheek. The lesion showed a circumscribed proliferation of basaloid cells with central areas of eosinophilic ghost shadow cells and intermediate cells. Basaloid nests showed round to oval, hyperchromatic nuclei with open nuclear chromatin, prominent nucleoli and frequent mitoses but no marked nuclear pleomorphism or infiltration was identified. The lesion recurred twice at the same site. Both recurrences showed similar morphology as the primary tumour however there were extensive areas of stromal necrosis, infiltrating edges, frequent mitoses with atypical forms, and lymphovascular invasion. There was no marked nuclear pleomorphism. Morphological features favoured a diagnosis of pilomatrix carcinoma. The child is still on follow-up and no recurrence has been identified to date. Pilomatric carcinoma is rarely reported in infants. Due to its rarity, aggressive histological features may be missed.