Résumé
POEMs syndrome is a rare paraneoplastic disorder related to plasma cell dyscrasia. The present study deals with anunusual case of POEMs syndrome in a young male who came with ascending demyelinating polyneuropathy, resultingin complete quadriparesis. The diagnosis was based on clinical findings of gynecomastia, hyperpigmentation andhypertrichosis of skin and lymphadenitis. Detailed laboratory investigations and imaging confirmed POEMS syndrome.In view of the rapidly progressing polyneuropathy, the patient was treated with chemotherapy followed by stem celltransplantation. The patient recovered completely after the treatment.
Résumé
POEMS syndrome is a rare disorder, also known as Crow-Fukase, PEP or Takatsuki syndrome. The acronym, POEMS, represents polyneuropathy, organomegaly, endocrinopathy, M protein and skin change. However, there are associated features not included in the acronym such as sclerotic bone lesions, Castleman disease, papilledema, thromobocytosis, peripheral edema, ascites, effusion, polycythemia, fatigue and clubbing. In most cases, osseous lesions in POEMS syndrome present as an isolated sclerotic deposit and that reveal as osteosclerotic myeloma. Several cases of 18F-FDG PET in multiple myeloma involvements were reported, but there was no previous literature that reported FDG PET findings in POEMS syndrome. We describe here a 66-year-old patient with POEMS syndrome who underwent 18F-FDG PET/CT image.
Sujets)
Sujet âgé , Humains , Ascites , Oedème , Fatigue , Fluorodésoxyglucose F18 , Hyperplasie lymphoïde angiofolliculaire , Myélome multiple , Oedème papillaire , Syndrome POEMS , Polyglobulie , Polyneuropathies , Tomographie par émission de positons couplée à la tomodensitométrie , PeauRésumé
Se presentó el caso de una paciente de 42 años de edad, raza blanca, que presentó hiperpigmentación dérmica en cara, cuello y parte superior del tórax, decaimiento, cefalea occipital y pérdida de peso, posteriormente le aparecen manchas blanquecinas en las zonas hiperpigmentadas que le producen prurito y ardentía intensa. En los estudios que se le realizaron la eritrosedimentación siempre tuvo cifras elevadas, se informó una marcada agrupación eritrocitaria y hepatomegalia ligera, en las dos biopsias de piel que se le hicieron no se concluyó diagnóstico, el informe de la biopsia de médula ósea fue: infiltrado intersticial por células plasmáticas maduras e inmaduras, marcada osteosclerosis, compatible con un mieloma osteosclerótico. En los días que se esperaba el resultado de este estudio inició un síndrome febril agudo acompañado de sudoración, falta de aire intensa y fallece.
A 42 year old Caucasian female patient presented dermic hyperpigmentation in face, neck and upper thoracic part. Depression, occipital headache and weight loss were other presenting symptoms; some whitish spots appeared in those hyperpigmented zones producing itch and burning pain. Erytrosedimentation tests always had augmented level a marked erythrocyte aggregation and mild hepatomegaly were observed. Diagnosis was not concluded in two skin biopsies performed. The report of bone marrow biopsy was: interstitial infiltration by mature and immature plasmatic cells a marked osteosclerosis associated with osteosclerotic myeloma. While expecting for the results of this study an acute fevered syndrome and sweating and severe dyspnea appeared and the patient died.
Résumé
POEMS syndrome is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein (M-protein), and skin changes. The authors describe a patient with POEMS syndrome who had osteosclerotic myeloma confirmed by open bone biopsy. Magnetic resonance imaging (MRI) showed discrete lesions of low signal intensity in both T1 and T2-weighted images. This patient is now being successfully treated with melphalan and prednisone with much improvement in skin thickening and sensory change in the lower extremities.