ABSTRACT
Background: 2008 World Health Organization (WHO) classification of hematolymphoid neoplasms (HLN) has classified them based on morphology, results of various ancillary techniques, and clinical features.[1] There are no studies looking at the applicability of WHO classification. Aims: The aim of the study was to calculate proportions of all HLN subtypes seen during 1-year period based on 2008 WHO classification of HLN and study applicability and also shortcomings of practices in a tertiary care center in India. Materials and Methods: This was a 1-year retrospective study (January 1st, to December 31st, 2010) where cases were identified using hospital/laboratory electronic records. Old follow-up and referral cases were excluded from the study. Only newly diagnosed cases classified into categories laid down by 2008 WHO classification of HLN included. Results: Out of 2118 newly diagnosed classifiable cases, 1602 (75.6%) cases were of lymphoid neoplasms, 489 (23.1%) cases of myeloid neoplasms, 16 (0.8%) cases of histiocytic and dendritic cell neoplasms, and 11 (0.5%) cases of acute leukemias of ambiguous lineage. Overall, most common HLN subtype was diffuse large B-cell lymphoma (n = 361, 17.0%). Precursor B-lymphoblastic leukaemia/lymphoma (n = 177, 48.2%) was the most common subtype within pediatric age group. Conclusions: All major subtypes of HLN were seen at our center and showed trends almost similar to those seen in other Indian studies. Molecular/cytogenetic studies could not be performed on a significant number of cases owing to logistic reasons (unavailability of complete panels and also cost-related issues) and such cases could not be classified as per the WHO classification system.
ABSTRACT
CONTEXT: Langerhans cell histiocytosis (LCH) is a rare atypical cellular disorder characterized by clonal proliferation of Langerhans cells leading to myriad clinical presentations and highly variable outcomes. There is a paucity of Indian studies on this subject. AIM: To present the experience of management of LCH at a single institution. SETTINGS AND DESIGN: This is a retrospective observational study of patients with LCH who presented at the Tata Memorial Hospital between January 1987 and December 2002. MATERIALS AND METHODS: Fifty-two patients with LCH were treated in the study period. Due to the long observation period and variability in diagnostic and therapeutic protocols, the patients were risk-stratified based on present criteria. The disease pattern, management approaches and treatment outcomes of patients were recorded. STATISTICAL ANALYSIS USED: Statistical analyses were done using Student's 't' test, test for proportion and survival estimates based on the Kaplan-Meier method. RESULTS: The median age at presentation was 3 years and more than 48% of the patients had Group I disease. Skeleton, skin and lymphoreticular system were the commonly involved organs. Majority (80%) required some form of therapy. The projected overall survival is 63% at 10 years and mean survival is 118 months. Seventeen percent of surviving patients developed long-term sequelae. CONCLUSIONS: The clinico-biologic profile of LCH patients in India is largely similar to international patterns except a higher incidence of lymphoreticular involvement. Majority of the patients respond favorably to therapy and have a good outcome, except a subset of Group I patients who warrant enrollment in clinical trials with innovative therapeutic strategies to improve outcome.