Multicentric Castleman's disease in India – Does EBV rather than HHV8 play a role?
Viral Bhanvadia1, Tanuja Shet2, Vidya Rao3, Sridhar Epari3, Sumeet Gujral4, Hasmukh Jain5, Bhausaheb Bagal5, Manju Sengar6
1 Department of Pathology, M & J Western Regional Postgraduate Institute of Ophthalmology, affiliated to B. J. Medical College, Ahmedabad, Gujarat, Ex. Praful B. Desai/UICC Fellow, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India
2 Department of Pathology and Head of Department of Biochemistry, Tata Memorial Hospital and Homi Bhabha National Institute, Eight Floor Annex Building, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India
3 Department of Pathology, Eight Floor Annex Building, Parel, Mumbai, Maharashtra, India
4 Department of Pathology, Convener Adult Hematolymphoid Disease Management Group, Parel, Mumbai, Maharashtra, India
5 Department of Medical Oncology, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India
6 Professor and Secretary Adult Hematolymphoid Disease Management Group, Department of Medical Oncology, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India
Department of Pathology, Eight Floor Annex Building Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra
Source of Support: None, Conflict of Interest: None
Background and Aim: Multicentric Castleman's disease (MCD) is a rare lymphoproliferative disorder manifesting as multiple lymphadenopathy, multiorgan involvement, and inflammatory symptoms. This study aims at highlighting some unique features of MCD in Indian patients. Materials and Methods: These 17 patients from review of 78 cases of Castleman's disease (CD) diagnosed. Besides routine tissue sections were stained for Human Herpes Virus 8 latency associated nuclear antigen (HHV8-LANA) by immunohistochemistry (IHC) and Epstein Barr virus latent membrane protein (EBV-LMP) or Epstein Barr Virus by in situ hybridization (EBER-ISH). Result: The cases included Plasma cell variant (11 cases), mixed MCD (4 cases) and two concurrent MCD with large B cell lymphoma in HIV positive patients. Median age of disease onset was 47 years and female predominance was seen. Out of 15 MCD uncomplicated by lymphoma, 5 had POEMS (Polyneuropathy, organomegaly, endocrinopathy, myeloma protein, skin changes) and one also had TAFRO (Thrombocytopenia, anasarca, fever, marrow reticulin fibrosis, organomegaly, normal or slightly elevated immunoglobulin) syndrome. Out of 10 MCD without lymphoma, 2 cases showed few EBV positive large cells, both have features of POEMS. All 17 MCD cases were negative for HHV8-LANA IHC. Two HIV patients with MCD had large cell lymphoma, intrasinusoidal pattern, of which one was EBV positive. Though four relapses were seen, none died from disease. One of the two patients complicated by lymphoma died from disease. Conclusion: Indian patients with MCD show female preponderance and are negative for HHV8 but show EBV positive cells. This makes a case for role of EBV in etiopathogenesis of MCD in India.