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Castleman’s disease

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Rare process associated with lymph node enlargement at a single site or throughout the body.

 

Involves the proliferation of B cells that produce cytokines.

Associated with hyper secretion of the cytokine IL-6.

Unicentric disease implies enlargement of lymph nodes at a single site.

In 90% of cases removal of a single node is curative.

Multicentric disease involves enlarged lymph nodes at multiple sites ad 50% of cases are caused by Kaposi’s sarcoma-associated Herpesvirus (KSHV).

Human herpesvirus (HHV)-8 and IL6 R. etiologic factors and therapeutic targetsLocalized or unicentric disease affects a single lymph node and the chest and abdomen are affected most often.

In unicentric Castleman’s disease 80% of cases have chest lymphadenopathy.

Unicentric Castleman’s disease rarely present with peripheral adenopathy.

50% of cases of multicentric Castleman’s disease (MCD) of unknown cause.

A nonclonal lymphoproliferative disorder that can affect single lymph node, or generalized involvement.

IL-6 plays role in pathogenesis of the disease.

Human herpesvirus 8, encodes for viral homolog of IL6 and is a driving force in HIV positive patients.

Outcome in HIV negative CD is excellent.

Responds to monoclonal antibody therapy directed at IL-6 receptor or IL-6 itself.

IL6 elaborated by B lymphocytes in germinal centers of CD lymph nodes

Multicentric disease associated with Kaposi’s sarcoma Herpesvirus is the plasmacytic form of Castleman’s disease.

The hyaline vascular form of Castleman’s disease is negative for the KSHV.

Multicentric disease may be associated with fever, anemia, weight loss, anorexia and leukopenia, all of which may be associated with excess interleukin 6.

Because of the nonspecific nature of the presentation diagnosis is difficult.

Prognosis for unicentric disease is excellent.

Treatment of multicentric disease has no standard approach as of this time.

AIDS related multicentric disease treated by management for HIV disease.

Multicentric form acts like lymphoma, and in fact, many patients develop lymphomas.

There is presently no standard treatment for multicentric CD.

Bortezomib therapy may be efficacious.

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