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.
Hudnall
S David
SD
Department of Pathology, University of Texas Medical Branch, Galveston, Tex 77555-0741, USA. shudnall@utmb.edu
Chen
Tiansheng
T
Brown
Kelty
K
Angel
Tiffany
T
Schwartz
Mary R
MR
Tyring
Steven K
SK
D005871:Castleman Disease / Q000473:pathology / Q000821:virology; D005260:Female; D018324:Hemangioma, Capillary / Q000175:diagnosis* / Q000473:pathology / Q000821:virology*; D006566:Herpesviridae Infections / Q000175:diagnosis* / Q000473:pathology; D019288:Herpesvirus 8, Human / Q000302:isolation & purification*; D006801:Humans; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000473:pathology / Q000821:virology; D012878:Skin Neoplasms / Q000098:blood supply / Q000473:pathology / Q000821:virology*