Poems Syndrome: A Rare and Relatively Unknown Disease

Case Report

Ann Hematol Oncol. 2015;2(5): 1042.

Poems Syndrome: A Rare and Relatively Unknown Disease

Paola Finsinger, Marta Chisini, Robin Foa and Maria Teresa Petrucci*

Department of Cellular Biotechnologies and Hematology, “Sapienza” University, Italy

*Corresponding author: Maria Teresa Petrucci,Department of Cellular Biotechnologies and Hematology, “Sapienza” University, Via Benevento 6, 00161 Rome,Italy

Received: May 15, 2015; Accepted: June 02, 2015; Published: June 10, 2015


POEMS syndrome is a rare paraneoplastic syndrome associated with plasma cell dyscrasia. We report a case of solitary plasmacytoma with osteosclerotic lesion in a 53 year-old man treated with local radiotherapy. After four years, the patient experienced peripheral polineuropathy, cutaneous hyperpigmentation, evidence of multiple bone lesions on CT scan, deep venous thrombosis of the lower extremities, and the presence of a serum M protein. The patient was diagnosed with POEMS syndrome. He received a double highdose melphalan with autologous stem-cell support which was followed by a systemic and hematologic improvement. Plasmacytoma is very rare as an initial manifestation of POEMS syndrome. Patients presenting with plasmacytoma and an osteosclerotic lesion should be carefully observed and evaluated for the possible development of POEMS syndrome, as most bone plasmacytomas in POEMS syndrome patients are reported to be osteosclerotic

Keywords: Plasmacytoma; Neuropathy; Osteosclerotic lesion


POEMS syndrome is a rare multiorgan disorder resulting from an underlying plasma cell dyscrasia, which is characterized by (P) Polyneuropathy, (O) Organomegaly (hepatosplenomegaly or lymphadenopathy), (E) Endocrinopathy (diabetes mellitus, hypothyroidism, or hypogonadism), (M) M-proteinemia, (S) Skin changes (most frequently hyperpigmentation) and associated manifestations, including Castleman’s disease, edema (peripheral edema, as cites, pleural effusions), thrombocytosis/erythrocytosis, a predisposition towards thrombosis and abnormal pulmonary function tests [1]. In addition, 88–100% of patients with POEMS syndrome have plasmacytomas with osteosclerotic lesions [2], while the majority of plasmacytomas in patients with classical myeloma is osteolytic. The pathogenesis of POEMS syndrome is not fully understood, but the overproduction of Vascular Endothelial Growth Factor (VEGF) secreted by the plasmacytoma is thought to be responsible for the characteristic symptoms [3]. Usually the first symptom is peripheral polyneuropathy, which is more or less frequently associated with osteosclerotic bone lesions. According to the guidelines and recommendations published by the Mayo Clinic group and updated in 2014 [4,5], the POEMS syndrome diagnostic policy includes: two mandatory criteria (peripheral polyneuropathy and monoclonal paraproteinemia); one major criterion (Castleman’s disease, osteosclerotic lesions, increased levels of VEGF); one minor criterion (organomegaly, extra vascular edema, endocrinopathy, skin changes, papilledema, thrombocytosis/erythrocytosis, hyperhydrosis, pulmonary hypertension, thrombotic events) (Table 1). A case of POEMS syndrome is reported below, which developed after an initial manifestation of a solitary plasmacytoma with an osteosclerotic lesion, more than 3 years before the onset of the first systemic syndrome.