Castleman ' s Disease-Unicentric Plasma Cell Variant-A Case Report with Review of Literature

disorder, characterized by lymphadenopathy. It may involve a single lymph node or a lymph node chain or present with disseminated disease. The localized variant is often pauci symptomatic and treatment is surgical, in certain situations radiotherapy. Disseminated form presents with constitutional symptoms, organomegaly, cytopenias or transaminitis and treatment options vary from biological agents to combination chemotherapy.

eventually found to have large mediastinal lymphadeopathy 1 (LAP).It was followed by a dozen additional patients who 2 were largely asymptomatic, but had mediastinal masses.This disorder is also known as angiofollicular lymph node hyperplasia, giant lymph node hyperplasia, lymphoid hamartoma and benign lymphoma or follicular 3 lymphoreticuloma.HPE revealed increased number of lymphoid follicles.Germinal canters varied in their cellularity, ranging from acellular fibrinous hyalinization of capillaries to proliferation of pale eosinophilic cells with copious cytoplasm.In subsequent years, additional cases of patients with diffuse LAP and the histologically characteristic

Discussion
Fifty years ago, Dr. Benjamin Castleman described this rare lymphproliferative disorder and was named as Castleman's disease after him.He initially described a patient who presented with many years of fever and weakness,  were surrounded by concentric sheets of plasma cells in the 5 inter follicular space.This novel histologic variant was termed 'plasma cell variant (PCV-CD).The recognition of both patients with localized LAP and disseminated disease led to an additional clinical categorization of CD: 'Unicenteric(UCD) versus "multicentric" (MCD).More, recently a third 'sub variant' known as 'plasmablastic MCD' has been described in association with particularly aggressive cases of MCD.In such first series CD patients with POEMS syndrome (Polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, skin changes) were found to have lymph nodes which resembled PCV, but also had large plasma cells in mantle zone with copious cytoplasm and prominent single or multiple nucleoli, second series found it associated with HHV-8 infection and progression to plasma 6,7,8 blastic lymphoma.The initial step in the development CD appears to be production of IL-6 by B-cells in the lymph node mantle zone, stimulated in the majority of cases by HHV-8 infection and in a minority of cases by a heretofore unidentified exogenous or endogenous factor.Local elaboration of IL-6, and in turn VEGF, produces the characteristic B-cell proliferation and vascularization of CD.In patients with multicentric disease, systemic symptoms may result from circulating IL-6, IL-6 producing B-cells, the generation of excess antibodies or Multicentric-Plasma cell Variant (M-PCV), least common, affects the people in 5th and 6th decade.Patients present with fever, weight loss and night sweats, 13 organomegaly, anemia, thrombocytopenia or transaminitis. .One of the most devastating disease associated with M-PCV is POEMS syndrome, seen in upto 15% of cases, more likely and radiotherapy may be a viable option for patients who are poor surgical candidates.A myriad of treatment options exist for patients with MCD.Gancyclovir, interferon-alpha and rituximab may be the best treatment for patients with HHV-8 infection and CD-20 positivity, while CHOP and VAD may be more appropriate for patients with 1 severe systemic manifestations of MCD.
In conclusion, five decades of steady research into the etiology and management of the unusual and fascinating lymphoproliferative disorder has resulted in many answers, but generate a myriad of questions.Innovative treatment strategies have been developed.
In the coming years, however, fundamental questions remain to be answered.Why is CD seen only in small proportion of patients infected with HHV-8?Why do some patients present with localized disease only?What is the role of IL-6 and angiogenesis?What are the best treatment options?

4 CD
lymph node architecture were identified.These series also revealed a new verdict of CD, the germinal centres in the involved nodes showed no evidence of hyalinization, but were normal.Right thoracotomy revealed multiple lymph nodes around right hilum.Excision biopsy of the lymph node was done.Histopathology revealed plasma cell variant of Castleman's disease (Figures 3, 4 & 5) and immunohistochemistry highlighting CD20, CD23, MIB-1 reactive Tcells CD3, plasma cells highlighted by CD-138, Kappa and Lambda -2 and negative for BCL-2 and cyclin -D.Patient is planned for radiotherapy as complete excision was not done.

14 infected with HHV- 8 .
Case reports have been documented of association of Hodgkin's lymphoma, development of 15 follicular dendritic cell sarcoma, paraneoplastic pemphigus.The best treatment of localized form is complete surgical 2,3,4,11 resection