Prophylactic Use Of Levofloxacin During Induction Of Acute Lymphoblastic Leukaemia In Children-Experience From Pakistan.

To assess whether prophylactic use of Levofloxacin would reduce the number of febrile neutropenia episodes during the induction phase, a single-centre, case-control study was carried out. Data was collected prospectively of patients who received Levofloxacin prophylaxis during the induction chemotherapy from September 2019 till October 2020. The cases were compared with historical controls who did not receive antibiotics prophylaxis. A total of 121 patients were enrolled, among which 61 patients were cases, whereas 60 patients were controls. The patients who received Levofloxacin prophylaxis had lower rate of febrile neutropenia episodes than patients who did not receive any prophylaxis (p≤0.01) (odds ratio [OR]:0.23, CI 95%). No significant difference in induction mortality was seen between the two groups (p≤0.14). Levofloxacin prophylaxis reduced the rate of febrile neutropenia episodes among patients, but it did not affect the infection related mortality.

10-Year-Old Pakistani Boy With Multiple Malignancies: Loss Of Pms2-Constitutional Mismatch Repair Deficiency.

Constitutional Mismatch repair deficiency (CMMRD) is a cancer predisposition syndrome. Four main common malignancies seen are, haematological, brain tumours, colorectal cancers, and intestinal polyps. We are reporting a 10-year-old boy with CMMRD; diagnosed with hematological malignancies, followed by low grade glioma in thalamus. There was loss of PMS2, whereas immunostaining was retained for MLH1, MSH2 and MSH6. Early diagnosis of CMMRD is crucial especially in countries like Pakistan, where consanguineous marriages are common. Increasing awareness among the physicians will help in early diagnosis, surveillance and in providing appropriate genetic counselling to the family.

Translocation t(1;19) in acute precursor B-cell lymphoblastic Leukaemia in paediatric patients- Pakistani population.

OBJECTIVE: To determine the impact of translocation t(1;19) in paediatric patients diagnosed with precursor B-cell acute lymphoblastic leukaemia. METHODS: The retrospective study was conducted at the Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan, and comprised data from January 2012 to January 2018 of paediatric patients diagnosed with precursor B-cell acute lymphoblastic leukaemia. Data of patients having t(1;19) translocation with or without complex karyotype formed group A, while data of patients without any cytogenetic abnormality formed the control group B. Relapse and event-free survival were calculated and the outcomes were compared between the groups. Data was analysed using SPSS 20. RESULTS: Of the 450 subjects whose data was analysed, 84(18.7%) were included; 25(30%) in group A and 59(70%) were in group B. There were 21(84%) males and 4(16%) females in group A with mean age on presentation 3.68±0.6 years compared to 41(69.4%) males and 18(30.5%) females with mean age on presentation 4.0±0.92 (p>0.05). There were no differences between the groups in terms of baseline markers (p>0.05). The relapse and event-free survival rates were also not significantly different between the groups (p>0.05). CONCLUSIONS: There was no significant difference related to outcomes of precursor B-cell acute lymphoblastic leukaemia patients having t(1;19) translocation with or without complex karyotype and those without any cytogenetic abnormality, indicating that translocation t(1;19)-positive patients do not need treatment intensification.

Survival outcomes of unilateral retinoblastoma based on pathological risk stratification-experience at a tertiary care centre in Pakistan.

Retinoblastoma (RB) is the most common childhood intraocular malignancy. In high-income countries over the past decade, upfront enucleation for unilateral RB is least favoured due to other alternatives that can help in globe preservation, but in low-middle income countries it is still the preferred option due to lack of resources and expertise. The treatment of RB after enucleation is tailored based on the histopathological risk features, as adjuvant chemotherapy with high-risk features reduces the risk of metastasis. The aim of our study was to analyse the survival outcomes of adjuvant therapy based on histopathological risk stratification in patients who underwent upfront enucleation for unilateral RB with advanced disease. A retrospective study was carried out at Shaukat Khanum Memorial Cancer Hospital and Research Centre, Pakistan. A total 113 patients (aged 3 months till 16 years) diagnosed with unilateral RB who had upfront enucleation from July 2009 till January 2019 were included in this study. The mean age of diagnosis was 37.4 months (±24.5) and male-to-female ratio of 1.3:1. The most common clinical presentation was leukocoria (74.3%). Patients who underwent enucleation had advanced disease; group D present in 62.8% followed by group E (32.7%). Histopathology revealed high-risk features in 29 patients (25.7%) and intermediate risk in 54 patients (47.8%). Disease progression and relapse was seen in patients with high-risk histopathological features. The 4-year over-all survival and EFS observed for this cohort was 74% and 71%. Awareness about the early symptoms among the general population and health care personnel at a nationwide level is needed to facilitate early detection and lessen disease related morbidity and mortality.

A 17-year-old girl with massive Intra-abdominal Germ Cell Tumour.

Teratomas comprise of three germ layers. Mature cystic teratomas are the most common among them. Due to cystic and solid components the radiological presentation varies. Even today surgical resection is the treatment of choice for mature teratoma. We share the case of a 17-year old girl with massive abdominal distension; subsequently diagnosed with mature teratoma.

Clinical course, long-term outcomes, and chemotherapy toxicity profile in B-cell Non-Hodgkin Lymphoma in children: Single institution experience of Pakistan.

OBJECTIVE: To analyse the disease presentation, clinical course and long-term outcomes of children diagnosed with B-cell non-Hodgkin lymphoma. Methods: The retrospective descriptive study was conducted at the Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan and comprised data of patients diagnosed with B cell non-Hodgkin lymphoma between January 2012 and December 2016, with follow-up time of 4 years post-treatment. Data was collected from the institutional database. Data was analysed using SPSS 20. RESULTS: Of the 286 patients, 217(75.6%) were males and 69(24.1%) were females. The overall mean age at presentation was 7.6±4.1 years (range: 1-16 years). Burkitt lymphoma was diagnosed in 194(67.8%) patients, and diffuse large B cell in 83(29.0%). Majority of patients had presented with stage III disease 159(55.6%). Complete remission was achieved in 173(60.5%) patients, while 90(31.5%) died during treatment. The 4-year overall survival observed was 67.1%, whereas event-free survival was 60.5%. Conclusion: Majority of the paediatric patients presented with extensive disease. Measures are needed for early recognition, identification, and prompt referral to paediatric cancer institute.

Outcomes of Advanced Retinoblastoma Treated with Local Salvage Treatment; a Retrospective Case Series.

Introduction: Retinoblastoma (RB) is the most common intraocular malignancy arising from the developing retina and occurs in approximately one of every 15,000-20,000 births. With the introduction of the intra-arterial chemotherapy (IAC), the 5-year overall survival of children with RB is 99%, though in low- and middle-income countries, it rarely exceeds 35% due to limited resources and lack of expertise. The aim of this study was to determine the outcome of local salvage in advanced RB. Materials and Methods: A retrospective analysis was conducted on children diagnosed with advanced RB that had local salvage therapy along with systemic chemotherapy from January 2015 to January 2018 at Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan. Results: Fifteen patients were included in the study, among these 10 were male. The median age of presentation was 20 months (range 2-40 months). Among participants, 11 patients had bilateral RB. Fourteen patients received local control along with systemic chemotherapy. Relapse disease was seen in 12 patients and 2-year disease-free survival (DFS) was 20%. Conclusion: The results of the present study suggest that centres lacking the resources for IAC should treat advance cases of RB with an upfront or early enucleation.

Malnutrition, Sepsis, and Tumor Lysis Syndrome Are Associated with Increased Rate of Acute Mortality in Mature B Cell Non-Hodgkin Lymphoma in a Pediatric Population-Study from Tertiary Care Hospital in Pakistan.

BACKGROUND: Outcomes of pediatric mature B cell non-Hodgkin's lymphoma in resource-challenged countries are negatively affected by an increased rate of early and toxic deaths. Aim of this study is to assess the rate of acute mortality and define significant risk factors present in children with mature B cell non-Hodgkin's lymphoma. METHODS: A retrospective analysis was done of patients with B cell non-Hodgkin's lymphoma from January 2012 till December 2016. Risk factors studied for acute mortality were malnutrition, stage, prior surgery with open laparotomy, lactate dehydrogenase levels, tumor lysis syndrome, sepsis, and fungal infection. RESULTS: A total of 233 patients were enrolled in the study. Eighty-five (36.4%) were below 15th percentile weight for age. Treatment was started in 226 patients. Eighty-eight percent of children showed a 20% response after COP pre-phase. Tumor lysis syndrome was developed in 20.6% (n = 48) children and 42.9% (n = 100) patients had sepsis, 71/100 patients had culture-proven sepsis. 19.7% (n = 46) patients developed fungal infection. There was 19.7% (n = 46) acute mortality. The most common cause of death was sepsis (n = 22, 47.8%) followed by acute renal failure secondary to tumor lysis syndrome. On multivariate analysis, three independent variables found significant for early death are malnutrition, sepsis, and tumor lysis syndrome. CONCLUSION: Rate of acute mortality in mature B cell NHL is high in our set up and significant risk factors are tumor lysis syndrome, sepsis, and malnourishment at the time of presentation.

Six-year-old boy with mediastinal mass.

Lymphoblastic lymphomas account for 20-30% of all non-Hodgkin lymphomas (NHL) in children, and most cases of childhood lymphoblastic lymphoma are T-cell type (T-LL). T-LL occurs most frequently in late childhood and adolescence; with male predominance of 2:1.We present a paediatric case with a right sided mediastinal mass causing mediastinal shift diagnosed as T-LL.