Archives of Pathology & Laboratory Medicine,  Jul 2005  by Roshong-Denk, Stacie L,  Bohman, Summer L,  Booth, Robert L

The multicentric form of CD is morphologically identical to the plasma cell variant. Although this type of CD is most common in individuals infected with HIV, reports of this disease have also been described in patients who have undergone renal transplantation or received immunosuppression therapy, such as cyclosporin A.8 The multicentric form is associated with HHV-8 infection. Fever, hepatosplenomegaly, peripheral lymphadenopathy, edema, and pulmonary symptoms, as well as secondary tumors related to HHV-8 infection (eg, Kaposi sarcoma, non-Hodgkin lymphoma, or plasmablastic lymphoma) are common, especially with coexisting HIV disease.2 Like the plasma cell variant, the pathogenesis of multicentric disease stems from high levels of IL-6; however, it is virally mediated.4 Human herpesvirus 8 encodes a viral IL-6 (v-IL-6), which induces endogenous human IL-6 production.4 Multicentric disease follows an aggressive course in patients with HIV and may represent a medical emergency.2 Treatment includes chemotherapy, and the HHV-8 viral load is monitored in conjunction with chemotherapy to assess the effectiveness of treatment.

The etiology of CD is unknown. It has been hypothesized that the 2 variants may represent a continuum of the same disease, that is, the plasma cell variant evolving into the hyaline-vascular variant; however, most investigators consider the 2 variants to be distinct entities.4 Interestingly, Menke et al7 found an aberrant immunophenotypical population of mantle zone B lymphocytes in all the variants of CD.

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