POEMS syndrome treated with melphalan high-dose therapy and autologous blood stem cell transplantation: a single-institution experience
| Autor: | Ruediger Liersch, Wolfgang E. Berdel, Andrea Kerkhoff, Carsten Müller-Tidow, Nils H. Thoennissen, Rolf M. Mesters, Martin Kropff, Gabriela B. Thoennissen, Fleur Fritz, Steffen Koschmieder, Andreas Hilbig, Utz Krug |
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| Rok vydání: | 2011 |
| Předmět: |
Melphalan
Adult Male Vascular Endothelial Growth Factor A medicine.medical_specialty Engraftment Syndrome Transplantation Autologous Organomegaly Dexamethasone Autologous stem-cell transplantation medicine Humans Anthracyclines POEMS syndrome Neurologic Examination Peripheral Blood Stem Cell Transplantation business.industry Hematology General Medicine Middle Aged Myeloablative Agonists medicine.disease Combined Modality Therapy Surgery Transplantation Treatment Outcome POEMS Syndrome Female medicine.symptom business Complete Hematologic Response Progressive disease medicine.drug |
| Zdroj: | Annals of hematology. 91(9) |
| ISSN: | 1432-0584 |
| Popis: | The acronym POEMS syndrome stands for a rare multi-system disorder, comprised of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Here, we present a single-center report of a series of five POEMS patients treated with melphalan high-dose therapy (HDT) with subsequent autologous blood stem cell transplantation (ABSCT). After a median follow-up of 52 months from time of diagnosis (range, 15–192) and a median follow-up of 18 months after ABSCT (range, 11–120), all patients were alive. Overall, no severe transplantation-associated complications such as engraftment syndrome or peri- or post-transplant death were noted. In two cases, HDT followed by ABSCT resulted in a complete hematologic response; in the additional three cases, partial responses (PR) were achieved including one very good hematologic PR. Only one patient with initial PR developed progressive disease nearly 2.5 years after transplantation. Consequently, a second HDT with ABSCT was successfully applied resulting in clinical improvement and hematologic PR. In line with previous single-center reports, melphalan HDT followed by ABSCT proved to be a first-line treatment option with tolerable side effects in severely affected POEMS patients with progressing symptoms. |
| Databáze: | OpenAIRE |
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