Clinicopathological Spectrum of Nodal Peripheral T-cell Lymphomas: Observations and Inferences of Half a Decade EC12-EC17
Dr. Bidish Kumar Patel,
Office, Department of Cytogenetics, 5th Floor, OT Block, Christian Medical College (CMC),
Ida Scudder Road, Vellore-632004, Tamil Nadu, India.
Introduction: Nodal Peripheral T-Cell Lymphomas (NPTCL) are a rare and heterogeneous group of lymphomas seen to occur more often in Asian, including South Indian population. They have an overall poor prognosis.
Aim: To document the varied spectrum of clinico-radiological, histopathological and follow-up parameters of NPTCL.
Materials and Methods: This descriptive cohort study involved clinical, histopathological and haematological workup of NPTCL cases diagnosed from January 2008 to June 2013 in JIPMER, Puducherry, India. The cases were diagnosed as per World Health Organisation (WHO) 2008 classification.
Results: NPTCL accounted for 25% of all Non-Hodgkin Lymphomas (80 cases). Peripheral T-cell Lymphoma-not otherwise specified (PTCL-NOS), Anaplastic Large Cell Lymphoma, ALK-positive (ALCL, ALK+), Anaplastic large cell lymphoma, ALK-negative (ALCL, ALK-) and Angioimmunoblastic Lymphoma (AITL) contributed to 51%, 29%, 10% and 10% of the cases respectively. A majority of patients (68%) had generalised lymphadenopathy, 26% had hepatosplenomegaly and 38% had marrow infiltration. Histomorphology showed wide variation within and in between the subgroups. Overall, necrosis, fibrosis and high mitosis were more common in ALCL while increased vascularity was seen prominently in AITL. At least 70% of the cases in each entity had advanced stage of disease.
Conclusion: There is a three-fold increase in incidence of NPTCL lymphomas in JIPMER compared to WHO (2008). Most patients presented with advanced stage and in sixth decade, except ALCL. There was wide histomorphological variability among these cases. Awareness of the entity and its histopathological heterogeneity would be worthwhile in arriving at a conclusive diagnosis.