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14 Facts About Pathology Of Castleman's Disease


Dr Sampurna Roy MD   






(1) Castlemanís disease (CD) is a benign lymphoproliferative disease characterised by hyperplasia of lymphoid follicles.

(2) It is known as giant or angiofollicular lymph node hyperplasia, lymphoid hamartoma, or angiofollicular lymph node hyperplasia.

(3) It is named after Dr Benjamin Castleman who described this disease in 1954 from Massachusetts General Hospital.

(4) The aetiology of the disease is unknown.

Several factors have been proposed to be associated with the development of Castlemanís disease, which include chronic low-grade inflammation, an immunodeficient state, and autoimmunity.

There is evidence to support the importance of infection with human herpesvirus 8 or Kaposi sarcoma-associated herpesvirus in the etiology and management of multicentric CD.

(5) Castlemanís disease may affect anyone from adolescent to seventh decade with equal sex distribution. It has a peak age of incidence in the third and fourth decades.

(6) It can develop in a single lymph node or series of lymph nodes. Unicentric Castlemanís disease presents with a slow growing mass, while multicentric variant manifests as fever, malaise, weight loss and generalised lymphadenopathy.

(7) It is reported to involve any lymph nodes in the body such as cervical, mediastinal, intraabdominal  and retroperitoneal. Only 5% can involve extranodal lymph node.

(8) Histologically three types are identified - hyaline vascular variety (90%), plasma cells type (8-9%) and intermediary mixed type (1-2%).

(9) The hyaline vascular type is identified by dense capillary proliferation and lymphocyte Ėpredominant infiltrate surrounding a small germinal centre. The presence of sheets of mature plasma cells surrounding the normal-large germinal centre is the diagnostic feature of plasma cell variant.

(10) Hyaline variant is generally asymptomatic and may be associated with iron deficiency anaemia and thrombocytopenia.

(11) Plasma cell verity is associated with infection, lymphoma, immunodeficiency, Kaposiís sarcoma, non-Hodgkin lymphoma, glomeruloid haemangioma, plasmacytoma, malignancies of colon, kidney and thyroid.

(12) POEMS- polyneoropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes are also manifestations of plasma cell type Caslemanís disease.

(13) Diagnosis of localised Castlemanís disease may be difficult in the presence of very few symptoms. On chest radiograph, it may appear as an incidental rounded solitary mediastinal or hilar mass with a differential diagnosis that includes thymoma, lymphoma, neurogenic tumor or bronchial adenoma.

In contrast, multicentric Castleman's disease may appear as bilateral hilar and mediastinal enlargement or diffuse reticulonodular pulmonary infiltrations.

(14) The prognosis is good after surgical excision in unicentric Castlemanís disease, and 5-year survival is 100%

Multicentric Castlemanís disease has a poor prognosis with a median survival of thirty months.

Splenectomy with systemic chemotherapy and steroids can improve the prognosis in multicentric Castlemanís disease



Microscopic examination showing Castleman's disease representing hyaline-vascular type.

Image : Lee J, Paek J, Lee YH, Kong TW, Chang S-J, Ryu H-S. Pelvic Castlemanís disease presenting as an adnexal tumor in a young woman. Obstetrics & Gynecology Science. 2015;58(4):323-326.




Lee J, Paek J, Lee YH, Kong TW, Chang S-J, Ryu H-S. Pelvic Castlemanís disease presenting as an adnexal tumor in a young woman. Obstetrics & Gynecology Science. 2015;58(4):323-326.


Barua A, Vachlas K, Milton R, Thorpe JAC. Castlemanís disease- a diagnostic dilemma. Journal of Cardiothoracic Surgery. 2014;9:170.








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