Donor-type bone marrow aplasia following hematopoietic stem cell transplantation in a child with a novel variant.

Pathogenic variants in the genes ( and cause bone marrow failure with characteristic syndromic features. We report a case of a previously healthy, 3-year-old boy with no dysmorphology, who presented with severe aplastic anemia and a novel variant in the gene. His father, elder brother and sister who harbored the same variant were completely healthy.

Determining age-specific prostate-specific antigen for healthy Indian men: A retrospective study.

Introduction: Prostate cancer usually affects older men and accounts for considerable cancer-associated mortality in men globally. In India, there is a rise in prostate cancer, and the published literature to determine age-specific prostate-specific antigen (PSA) is limited by a small sample size. The objective of our study was to determine age-specific PSA for Indian men based on a very large cohort of apparently healthy men undergoing health screening.

Robot assisted laparoscopic adrenalectomy: Should this be the new standard?

Introduction: Minimal invasive surgeries (MIS) for large size adrenal tumors are still debatable. The objective is to evaluate the contemporary peri- and post-operative outcomes of patients undergoing (open = OA, laparoscopic = LA, and robotic = RA) adrenalectomies in three institutions.

Materials And Methods: Retrospectively gathered peri- and post-operative data of 235 patients, underwent adrenalectomy at three Institutions over a 7-year period (2013-2020) were analyzed.

Multicenter Outcome of Hematopoietic Stem Cell Transplantation for Primary Immune Deficiency Disorders in India.

Background: Hematopoietic stem cell transplantation (HSCT) is the curative option for many primary immune deficiency disorders (PID). In the last 5 years, increased awareness, availability of diagnostics based on flow cytometry, genetic testing, improved supportive care, use of reduced toxicity conditioning, and success of haploidentical donor HSCT have improved access to HSCT for children with PID in India. We present results on children with PID who underwent HSCT across India and the factors that influenced outcome.

Augmented immunosuppression and PTCY-based haploidentical hematopoietic stem cell transplantation for thalassemia major.

Alternate donor HSCT for thalassemia major from a matched unrelated donor or haploidentical family donor is a feasible therapeutic option in children with no matched family donor. Aggressive pretransplant immunosuppression, reduced toxicity conditioning, and PTCY result in excellent thalassemia-free survival. We describe here our experience in this cohort.

Cranial nerve schwannoma - A pictorial essay.

Schwannomas are peripheral nerve sheath tumours arising from cranial, spinal or peripheral nerves. Most of the schwannomas are benign with the rare possibility of malignant transformation. Cranial nerve schwannomas can be seen along the course of any cranial nerve in the intracranial region or head and neck location.

A multi-institutional real world data study from India of 3453 non-metastatic breast cancer patients undergoing upfront surgery.

The present analysis reports the clinical, pathological, treatment profile and overall survival (OS) and disease-free survival (DFS) outcomes of consecutive breast cancer patients from three Indian centres, who underwent curative surgery as their first treatment. Among the 3453 patients, stage I, II, and III cases were 11.75%, 66.

Assessment of Age and Duration of Symptoms on Outcomes of Emergency Scrotal Exploration for Acute Scrotal Pain.

Objective: To study the effects of age and duration of symptoms on the outcomes of scrotal explorations for acute scrotal pain at our institution.

Study Design: Case series.

Place And Duration Of Study: Bradford Teaching Hospital NHS Trust, from January 2006 to December 2017.

Cystic trophoblastic tumor - The effect of chemotherapy in metastatic testicular germ cell tumor to retroperitoneal lymph nodes.

Cystic trophoblastic tumor is an uncommon lesion which is occasionally seen after chemotherapy in metastatic retroperitoneal lymph nodes in patients with testicular germ cell tumor. The tumor cell clusters show cystic change lined by single to multiple layers of cells with abundant dense eosinophilic vacuolated cytoplasm, large pleomorphic vesicular nuclei with smudged chromatin, and prominent nucleoli. It is important to identify this lesion as its prognosis is similar to a teratoma and does not require any additional chemotherapy.

Haploidentical Stem Cell Transplantation in Children with Benign Disorders: Improved Survival and Cost-Effective Care Over 15 Years from a Single Center in India.

We present our experience in haploidentical stem cell transplantation (haplo SCT) in children with benign disorders. We performed a retrospective study where children aged up to 18 years diagnosed to have benign disorders and underwent haplo SCT from 2002 to September 2017 were included. Of the 54 children, the most common indications were Fanconi anaemia 12 (22%), severe aplastic anaemia 8 (14%) and primary immune deficiency disorders (PID) 25 (46%).

Safety and efficacy of fresh whole blood donor lymphocyte infusion in children.

Donor lymphocyte infusion (DLI) is a form of cellular immunotherapy which is known to be effective in preventing relapse in leukemia by inducing graft versus leukemia (GVL) effect. In hematopoietic stem cell transplantation (HSCT) for benign hematological conditions including primary immune deficiency, mixed chimerism is seen with the use of reduced intensity conditioning. DLI can help prevent graft rejection by boosting the existing graft in these situations.

Successful Treatment of Relapsed Isolated Extraocular Retinoblastoma in the Right Fibula With High-dose Chemotherapy and Autologous Stem Cell Transplantation.

Literature on high-dose chemotherapy followed by autologous stem cell rescue in relapsed retinoblastoma is limited to <150 cases reported so far. We present our experience and the challenges faced in the management of a 7-year-old boy with relapsed isolated extraocular retinoblastoma in the right fibula who received salvage chemotherapy followed by high-dose chemotherapy and autologous stem cell rescue. Electrolyte disturbances, renal tubulopathy, and seizures were the most significant transplant-related morbidity.

Well-differentiated liposarcoma of the breast arising in a background of malignant phyllodes tumor in a pregnant woman: A rare case report and review of literature.

Pure mesenchymal sarcomas and phyllodes tumor with sarcomatous differentiation of the breast are rare tumors. Primary breast sarcomas are a heterogenous group of neoplasms which include malignant fibrous histiocytoma, fibrosarcoma, angiosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. Sarcomatous differentiation in phyllodes tumor is seen in the form of angiosarcoma, chondrosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma.

Hematopoietic Stem Cell Transplantation for Primary Immunodeficiency Disorders: Experience from a Referral Center in India.

Objective: To share experience of over 15 years in hematopoietic stem cell transplantation in children with primary immunodeficiency disorders.

Design: Medical record review.

Setting: A referral center for pediatric hemato-oncological disorders.

A review on adverse event profiles of epidermal growth factor receptor-tyrosine kinase inhibitors in nonsmall cell lung cancer patients.

The epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitors (TKIs) have revolutionized the treatment of EGFR-mutant nonsmall cell lung cancer (NSCLC). These EGFR TKIs demonstrate a different adverse event (AE) profile as compared to conventional chemotherapy agents. They are more commonly associated with cutaneous AEs and diarrhea while hematological AEs occurred commonly with chemotherapy agents.

Affordable and Safe Health Care for All Children: Lessons Learned from the Use of Peg-asparaginase in a Developing Country.

Peg-asparaginase has widely replaced the use of conventional asparaginase in treatment of children with acute lymphoblastic leukaemia in developed countries. In developing countries like India, with financial constraints being a part of clinical challenge to the treatment of cancers, uniform use of Peg-asparaginase in all children is not practically possible. However, we found by a retrospective analysis of 211 children treated for acute lymphoblastic leukaemia, uniform use of this drug was feasible with indigenous techniques like storing the drug with strict cold chain maintenance and sharing the drug amongst 2 or 3 patients to reduce the burden on each family.

Cytomegalovirus reactivation posthematopoietic stem cell transplantation (HSCT) and type of graft: A step toward rationalizing CMV testing and positively impacting the economics of HSCT in developing countries.

We aimed to determine a correlation between cytomegalovirus reactivation post hematopoeitic stem cell transplantation (post-HSCT) with the type of graft source, defining children at risk. We analyzed data on children less than 18 years of age undergoing HSCT from 2002 to May 2016 (n = 464). Correlation between reactivation and graft source was analyzed statistically.