To Study The Clinical Behavior And Treatment Outcome In Patients Of Extranodal Non-Hodgkin'S Lymphoma.

Aims and Objectives of the Study: Aim of this study is to study the clinic pathological feature, distribution and treatment outcomes of different types of extranodal non Hodgkin's lymphoma patients with special consideration to their diverse clinical presentations. Primary objective is to study the diverse clinical presentation and distribution of different types of extranodal non Hodgkin's lymphoma and secondary objectives are to determine response rates, pattern of relapses, progression free survival and overall survival. Material & Methods: Study Area: Department of Medical Oncology, Rajiv Gandhi Cancer Institute, New Delhi. Study population: A total of 61 biopsy and IHC proven patients with primary extranodal non Hodgkin's lymphoma were enrolled while patients with concomitant nodal involvement and those with plasmacytomas or any other malignancy were excluded. Data collection technique and tools: All enrolled patients were studied with respect to their clinical presentations, Ann Arbor staging, serum LDH levels, IPI at diagnosis and treatment outcomes. Clinical features, pathological features, response to treatment and their treatment outcome and follow up data were recorded. Data analysis: Results are expressed as mean standard deviation for continuous variables and in numbers and corresponding percentage for categorical variables. Categorical data is compared using Chi-square or Fisher's exact test for a two-sided p-value, whereas for ordinal data, the nonparametric tests are applied. The actuarial survival analysis is performed according to the method described by Kaplan and Meier. Cox regression analysis is used to correct for the confounding effect of differences in prognostic factors. The multivariate analysis of the variables predicting response is performed by using a logistic regression. The multivariate analysis for survival is performed by using the stepwise proportional hazards model after testing the proportionality assumptions for the co-variates. P value less than 0.05 is considered as statistical significance. All analyses performed with the statistical software SPSS version 22 (SPSS, Inc., Chicago, Ill., USA). Results: The median age of the patients is 54 years with 61 % patients being males. There were 8 deaths while 8 patients were lost to follow up at the time of evaluation. 21% patients had multifocal disease at presentation while systemic B symptoms were present only in 30 % cases. Most common site of involvement is GI tract (36%) followed by Bone (28%), Liver (13%), CNS (10%) and nose/nasopharynx (10%). Other uncommon sites of involvement included breast, kidney, testes, pancreas, skin, eyelids, uterus and submandibular gland. In GI Lymphoma, gastric lymphoma (26%) being most common followed by intestinal involvement (10%). Most common clinical presentation in case of GI lymphomas is abdominal pain and vomiting, in case of bony lymphomas with localized pain and swelling while focal neurological deficit is most common presentation in case of primary CNS lymphomas. DLBCL is the most common histological pattern (82%) observed in our study. 62% patients received R CHOP based chemotherapy while remaining patients received different regimens depending upon the site of involvement and histological type (DeAngelis protocol for primary CNS lymphoma (10%), R DA EPOCH (10%), Hyper CVAD (5%), CODOXM/IVAC for Burkitt's lymphoma). 57% patients achieved CR with first line induction chemotherapy, 33% patients achieved PR, and 3 % achieved stable disease while 2% progressed. Median follow up period of our study is 24.2 months (range, 1.03- 59.9). As estimated by Kaplan Meier curves, an overall 5 year disease free survival as estimated is 69.98% while 5 Year overall survival calculated is 80.05%. There were 14 relapses which were more common with multifocal disease, higher IPI, failure to achieve CR with first line chemotherapy and those with primary CNS lymphoma. 43% patients had early relapse while 57% patients had late relapse. Conclusions: 1) Primary extranodal lymphoma more commonly presents with site specific symptoms and with lesser B symptoms. 2) Gastrointestinal tract, mainly stomach and bone are the two most common extranodal sites. 3) DLBCL is the most common histology encountered in most of the studies in both nodal as well as extranodal location. 4) Multifocal disease at presentation imparts higher IPI and poor prognosis with higher chances of relapse despite achieving CR with first line treatment. 5) Response to chemotherapy is excellent with majority of patients achieving CR with first line chemotherapy however relapses are more frequent in those with higher IPI at presentation, CNS disease at presentation and in those who fail to achieve CR with first line chemotherapy. [ABSTRACT FROM AUTHOR]