Incidence and outcome of Kaposi sarcoma after hematopoietic stem cell transplantation: a retrospective analysis and a review of the literature, on behalf of infectious diseases working party of EBMT

Kaposi sarcoma in a child after hematopoietic stem cell transplantation: Should pre‐transplant HHV‐8 screening be considered in recipients from high prevalence areas?

“…There are no standard guidelines to address this complication in children after HSCT, the reduction/withdrawal of immunosuppression is the most frequently used approach. [7][8][9][10][11]14 A limited number of children received different therapies including doxorubicin (2), surgery (2) and local radiotherapy (1). In our case, all the lesions resolved discontinuing immunosuppression, including pulmonary nodules.…”

“…In the HSCT setting, the pre-transplant donors/recipients testing of HHV-8 and the prospective monitoring of HHV-8 load has not been recommended, because of the low seroprevalence for HHV-8 and the very rare occurrence of KS after HSCT in Europe. 4,11,12 Nevertheless, as some authors have previously suggested in solid organ transplants, 5,6,13 we propose to consider the pre-HSCT antibody screening in donors/recipients coming from high HHV-8 prevalence areas.…”

“…HSCT is associated with severe and prolonged immunosuppression, but few cases of KS after HSCT have been reported. According to Cesaro et al, 11 incidence after allogeneic HSCT is 0,17%. Part of the explanation for the low incidence of KS after HSCT in our media may lie in the worldwide variation in the HHV-8 prevalence: >50% in Africa and parts of Amazon basin, 5-20% in Mediterranean countries, Middle-Eastern countries and Caribbean and < 5% in North America, North Europe and Asia.…”

“…In the retrospective analysis and review of the literature published by Cesaro et al, 11 the majority of patients had suffered from acute or chronic GvHD at the time of KS diagnosis, and half of them had involvement of more than one site, skin, mucosae and lymph nodes most frequently. 11 The diagnosis of KS may be challenging and PTLD must be included in the differential diagnosis, especially in those patients with lymph node involvement. The presence of blue round subcutaneous lesions is indicative of an angioproliferative neoplasia, and the biopsy is mandatory to confirm the diagnosis.…”

“…In our case, all the lesions resolved discontinuing immunosuppression, including pulmonary nodules. However, only one of five cases of post-HSCT KS with lung involvement described in the literature resolved with reducing immunosuppressive treatment, 10,11 the rest received treatment with doxorubicin (2), doxorubicin plus interferon (1) and radiotheraphy (1).…”