Multicentric plasmocytic Castleman's disease with polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes syndrome and coexistent human herpes virus-6 infection--a possible relationship.

Authors report a case of Castleman's disease (CD) with polyneuropathy, organomegaly, endocrinopathy, M protein, skin change (POEMS) syndrome. According to the present knowledge, these two rare conditions are often induced by Human Herpes Vírus- 8 (HHV-8) or by Human Immunodefeciency Virus, separately or in combination. In this case, however, HHV-6 viral DNA had been detected in the blood and lymph node samples by PCR. The authors conclude that the modulation of immune functions by HHV-6 might be responsible for the development of CD and POEMS syndrome in the referred case.

Human herpesvirus-8-positive microvenular hemangioma in POEMS syndrome.

We report a case of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome in a 55-year-old African American woman in which human herpesvirus-8 (HHV-8) was demonstrated within rare lymphocytes in a Castleman disease lymph node biopsy and numerous endothelial cells and lymphocytes in a microvenular hemangioma skin biopsy. Initial symptoms and findings of night sweats, weight loss, anorexia, generalized lymphadenopathy, and hemangiomas improved after chemotherapy with cyclophosphamide and prednisone. However, in the year following the initial diagnosis, the patient suffered from recurrent bouts of night sweats, gastroparesis, and lymphadenopathy, which required further treatment with plasmapheresis, cyclophosphamide, prednisone, and rituximab. One year later, the patient is asymptomatic but has persistent gammopathy. Although HHV-8 has previously been detected in POEMS-associated Castleman disease tissue, to our knowledge, this is the first case report in which HHV-8 has been directly demonstrated within the endothelial cells of a POEMS-associated hemangioma.

Kaposi's sarcoma herpesvirus-associated Castleman's disease with POEMS syndrome.

The pathogenic link between POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes, and various other clinical signs) syndrome and Castleman's disease is unclear. Roles for M protein in POEMS syndrome and cytokines in systemic manifestations of multicentric Castleman's disease have been suggested. Recently, pathogenic roles of cytokines in POEMS syndrome and Kaposi's sarcoma-associated herpesvirus (KSHV) in Castleman's disease have been reported. We report on a patient with KSHV-associated Castleman's disease with POEMS syndrome, and suggest a possible role of KSHV in initiating and linking these two diseases.

Human herpesvirus 8 infection in patients with POEMS syndrome-associated multicentric Castleman's disease.

The polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS) syndrome is a rare multisystemic disorder associated with osteosclerotic myeloma and multicentric Castleman's disease (MCD). Human herpesvirus type 8 (HHV-8) DNA sequences have been detected in lymph nodes of about 40% of human immunodeficiency virus (HIV)-negative patients with MCD, and in bone marrow stromal cells of patients with multiple myeloma. Considering these data, we investigated the presence of HHV-8 in 18 patients with POEMS syndrome (9 with MCD), by nested polymerase chain reaction (N-PCR) to detect DNA sequenses in various cells and tissues obtained by biopsy or at autopsy (13 patients, of whom 7 had MCD), and by an immunofluorescence assay to detect anti-HHV-8 IgG antibodies in blood (18 patients, of whom 9 had MCD). Detection of HHV-8 DNA was performed using three different N-PCR, targeting nonoverlapping regions in open reading frame (ORF) 25 and ORF26. Seven of 13 (54%) POEMS patients had HHV-8 DNA sequences in their tissues, as assessed by all three N-PCR, and 9 of 18 (50%) had circulating anti-HHV-8 antibodies. HHV-8 was mainly detected in the subset of POEMS patients with MCD (6 of 7 [85%] for DNA sequences; 7 of 9 [78%] for antibodies). The percentage of positive N-PCR was higher in lymph nodes than in bone marrow samples (P <.02). Sequencing of amplicons showed a homogeneous restricted variability in the ORF26 region, characteristic of the minority subgroup B defined by Zong, and responsible for isoleucine and glycine substitutions at amino acid positions 134 and 167. These findings strongly suggest an association of HHV-8 infection with POEMS syndrome-associated MCD.