Castlemanís disease (CD) is a benign lymphoproliferative disease
characterised by hyperplasia of lymphoid follicles.
It is known as giant or
angiofollicular lymph node hyperplasia, lymphoid hamartoma, or
angiofollicular lymph node hyperplasia.
It is named after Dr Benjamin Castleman who described this disease
in 1954 from Massachusetts General Hospital.
The aetiology of the disease is unknown.
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.
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.
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.
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.
three types are identified - hyaline vascular variety (90%), plasma
cells type (8-9%) and intermediary mixed type (1-2%).
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.
Hyaline variant is generally asymptomatic and may be associated with
iron deficiency anaemia and thrombocytopenia.
Plasma cell verity is associated with infection, lymphoma,
immunodeficiency, Kaposiís sarcoma, non-Hodgkin lymphoma,
glomeruloid haemangioma, plasmacytoma, malignancies of colon, kidney
POEMS- polyneoropathy, organomegaly, endocrinopathy, monoclonal
gammopathy and skin changes are also manifestations of plasma cell
type Caslemanís disease.
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.
multicentric Castleman's disease may appear as bilateral hilar and
mediastinal enlargement or diffuse reticulonodular pulmonary
The prognosis is good
after surgical excision in unicentric Castlemanís disease, and
5-year survival is 100%
Castlemanís disease has a poor prognosis with a median survival of
systemic chemotherapy and steroids can improve the prognosis in
multicentric Castlemanís disease