Autologous haematopoietic stem cell transplantation followed by thalidomide therapy in poems syndrome
M Cioch, J Manko
Ann Transplant 2009; 14(1): 74-75
Available online: 2009-05-21
Background: POEMS syndrome is a rare plasmatic cell dyscrasia, which is associated with overproduction of VEGF and characterized by polyneuropathy,
organomegaly, endocrinopathy, monoclonal gammapathy, and skin changes. A specific treatment for this disease is not established. In recent reports, high dose chemotherapy followed by autologous haematopoietic stem cell transplantation (AHSCT), performed at an early stage can improve clinical
symptoms, especially for polyneuropathy. We describe the clinical course of a patient with POEMS syndrome in an advanced stage and unresponsive to chemotherapy.
Case Report: A 47-year-old man was admitted to Department of
Haematooncology and Bone Marrow Transplantation of Medical University in Lublin with quadriparesis, hepatosplenomegaly, sclerotic bone lesion, acrocyanosis, IgG lambda gammapathy and thrombocytosis. The result of a myelogram was normal. The first symptoms of the disease appeared three
years ago and diagnosis of POEMS was established 18 months ago. The patient was unresponsive to chemotherapy that consisted of cyclophosphamide and prednisone, so we decided to perform AHSCT. Peripheral blood stem cells were collected after mobilization by
cyclophosphamide (4g/m2) and G-CSF. The number of collected CD34+ cells was 12-106/kg. The conditioning regimen was with high dose melphalan (140 mg/m2). The number of transplanted CD34+ cells was 4-106/kg. The post-transplantation course was complicated by fever with no identifi able infectious aetiology, diarrhoea, cutaneous rash and swelling without hipoalbuminaemia (engraftment syndrome?). Neutrophil (0.5 G/l) and platelet (20 G/l) engraftment occurred on days 11 and 13, respectively. The improvement was manifested by disappearance of gammapathy and organomegaly. The withdrawal of neurological symptoms was unsatisfactory. Further improvement of neurological status was achieved after thalidomide therapy. The second autologous transplantation is considered.
Keywords: Stem Cell Transplantation, case report