ABSTRACT
POEMS syndrome is a rare paraneoplastic condition associated to an underlying plasmacellular dyscrasia. The pathogenesis of POEMS is poorly understood, but overproduction of VEGF, probably secreted by clonal plasma cells, is thought to be responsible for the signs and symptoms of the syndrome, and it seems to be useful for the monitoring of the response to therapy. At present, an effective therapeutic option for the patients is represented by autologous peripheral blood stem-cell transplantation (aPBSCT), although relapses have been described, and there is an important morbidity associated with this procedure. Before the implementation of aPBSCT, the clinical course of POEMS syndrome was characterized by progressive polyneuropathy potentially leading to death for respiratory failure. Given the high serum and plasma levels of VEGF observed in POEMS patients, the use of anti-angiogenetic drugs such as thalidomide and lenalidomide and other drugs with anti-VEGF and anti-TNF effect such as bortezomib have been considered to treat this syndrome. There are evidences of lenalidomide benefit in both front-line and previously treated patients, but scanty data are available about its use for relapse after aPBSCT. Here, we report the successful use of lenalidomide in a patient who relapsed after aPBSCT.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Multiple Myeloma/drug therapy , POEMS Syndrome/drug therapy , Peripheral Blood Stem Cell Transplantation , Salvage Therapy , Angiogenesis Inhibitors/administration & dosage , Biomarkers, Tumor/blood , Combined Modality Therapy , Cyclophosphamide/administration & dosage , Dexamethasone/administration & dosage , Humans , Humerus/pathology , Humerus/radiation effects , Immunologic Factors/administration & dosage , Lenalidomide , Male , Middle Aged , Multiple Myeloma/complications , Multiple Myeloma/radiotherapy , Multiple Myeloma/surgery , Osteosclerosis/etiology , Osteosclerosis/radiotherapy , POEMS Syndrome/etiology , POEMS Syndrome/surgery , Prednisolone/administration & dosage , Recurrence , Thalidomide/administration & dosage , Thalidomide/analogs & derivatives , Transplantation, AutologousABSTRACT
Osteosclerotic myeloma is a rare disorder characterized by paraproteinaemia and osteosclerosis, and may be associated with a progressive peripheral neuropathy. Patients with widespread osteosclerotic lesions can succumb from neurological complications despite systemic chemotherapy. We present a case of disseminated osteosclerotic myeloma associated with POEMS (peripheral neuropathy, organomegaly, endocrinopathy, M band, skin changes) syndrome, which was complicated by a rapidly progressive, life-threatening neuropathy. The patient's symptoms remained unchanged in the face of combination chemotherapy. However, a substantial improvement was seen following outpatient treatment with the commonly available radioisotope strontium 89 in combination with steroids.
Subject(s)
Multiple Myeloma/radiotherapy , Osteosclerosis/radiotherapy , POEMS Syndrome/radiotherapy , Radiopharmaceuticals/therapeutic use , Strontium Radioisotopes/therapeutic use , Anti-Inflammatory Agents/therapeutic use , Combined Modality Therapy , Disease Progression , Humans , Male , Middle Aged , Prednisolone/therapeutic useABSTRACT
We describe a patient with a 3 year history of progressive polyneuropathy that rendered him severely quadriparetic and bedridden. Work up revealed an IgG lambda monoclonal spike and multifocal osteosclerotic myeloma. Remarkable improvement followed combined treatment with surgical excision, radiation therapy, and chemotherapy using chlorambucil, danazol, and hydrocortisone. Hence, we believe that aggressive local therapy associated with systemic chemotherapy should be considered in severely affected patients with multifocal osteosclerotic myeloma and peripheral neuropathy.
Subject(s)
Multiple Myeloma/complications , Osteosclerosis/complications , POEMS Syndrome/complications , Polyneuropathies/complications , Adult , Humans , Humerus/diagnostic imaging , Male , Multiple Myeloma/drug therapy , Multiple Myeloma/radiotherapy , Osteosclerosis/drug therapy , Osteosclerosis/radiotherapy , POEMS Syndrome/drug therapy , POEMS Syndrome/radiotherapy , Physical Examination , Plasmapheresis , Polyneuropathies/surgery , RadiographyABSTRACT
A retrospective analysis of spleen irradiation in 25 patients with progressive idiopathic myelosclerosis (agnogenic myeloid metaplasia), treated between 1948 and 1983, shows that there is a place for the mechanical effect of irradiation being an indication for its use as a palliative measure. A decrease in spleen size of varying extent was achieved in 84% of patients, while the symptoms of abdominal compression improved in 88%. Remission period averaged 10 months. On the other hand, in all seven patients in whom a decrease in the size of the spleen might have been expected to signal an improved blood picture, the frequency of transfusion remained unchanged.
