POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome in China.

BACKGROUND/AIM: The clinical characteristics of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome in China are largely unknown. This work thus studied the clinical manifestations of POEMS syndrome in China. METHODS: We retrospectively reviewed the medical records of 82 patients with POEMS syndrome in our hospital and made a comparison with those reported outside China. RESULTS: There were 82 patients. Forty (49%) were 45 years old or younger. Sensorimotor deficits were the common initial symptoms. The clinical manifestations are as follows: (i) peripheral neuropathy and abnormal electromyogram were seen in all patients (100%); (ii) organomegaly was present in 72 patients (88%); 61 of them (74%) had splenomegaly; (iii) endocrinopathy was present in 74 cases (90%); hypothyroidism was seen in 51 of 70 patients (73%); (iv) 60 patients (73%) had monoclonal plasmaproliferative disorder; only 22 of 40 (55%) had M-protein; (v) skin changes were seen in 71 patients (87%); (vi) 68 patients (83%) had oedema and effusions; of these, hydropericardium was seen in 23 patients (28%); (vii) 35 of 55 patients (64%) had abnormal electrocardiogram and only 21 of 46 (46%) had bone lesions in X-ray. CONCLUSIONS: POEMS syndrome in China has its own distinctive features, parts of which are commoner in the young people, the higher frequency of splenomegaly, hypothyroidism, hydropericardium and abnormal electrocardiogram, as well as the lower M-protein and bone lesions in X-ray.

POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS Syndrome.

OBJECTIVE: To determine whether there are peculiarities of the POEMS syndrome (a multisystemic disorder associated with polyneuropathy, organomegaly, endocrinopathy of various forms, production of a monoclonal [M] component, and skin changes) in Caucasian patients, especially signs and symptoms absent in other series; and to attempt a reappraisal of the neuropathy and endocrinopathy to find a unifying mechanism. DESIGN: A retrospective, cooperative study compared 25 cases, observed over a 15-year period, with two published series of patients, one of Japanese patients and one of American patients, and with a review of the literature on non-Asian cases. Details were obtained of patients' medical history, physical examination, immunochemical and hormonal testing, roentgenographic examination, computed tomography imaging, and electromyography. RESULTS: The main features of the syndrome found in these patients were those first described in Japan: polyneuropathy, enlargement of the lymph nodes, liver, and spleen, endocrine disturbances, low concentration of the monoclonal component, hyperpigmentation, and hypertrichosis. Three other symptoms were found more frequently than previously reported: skin angiomas, scleroderma changes of the hands, and thrombocytosis. Electromyography and nerve biopsy showed a variety of abnormalities ranging from demyelination to axonal degeneration. Nerve deposits of immunoglobulin were absent. Organomegaly seemed to be heterogeneous. Pathologic findings in the enlarged lymph nodes and spleen were compatible with Castleman's disease. Liver biopsies were usually normal. The endocrine changes were surprisingly diverse, with some observations combining unrelated primary and secondary insufficiencies. No single hypothesis emerged regarding their mechanism. All M components had a lambda-light chain. An IgG M component was found more frequently in solitary lesions. An IgA M component was found more frequently in patients without bone lesions. Sedimentation rate was usually normal. Radiotherapy of solitary plasma-cytomas was followed by dramatic improvement of extramedullary signs and symptoms in all cases. CONCLUSIONS: The symptoms, clinical course, and management of the patients reported here were similar to those observed in the literature. This study confirms the existence of a close link between symptoms and lambda-light-chain production. There are indications that a plasma cell growth factor that does not cross-react with interleukin-6 (IL-6) may be involved.

Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia.

The POEMS syndrome is an infrequently reported multisystem disorder which presents usually as an obscure polyneuropathy, with almost all cases reported in Japan. A 64 yr old caucasian man presented with a 12 mth history of a severe sensorimotor neuropathy in association with dermato-endocrine features. Detection of a monoclonal IgG kappa paraprotein and mixed osteosclerotic/lytic bone lesions consistent with a plasma cell dyscrasia led to diagnosis of the POEMS syndrome. Unique ultrastructural features were present on sural nerve biopsy in addition to the unusual association with monoclonal kappa-light chain. This case illustrates that the POEMS syndrome may also occur in caucasian subjects.

POEMS syndrome in Chinese.

POEMS (polyneuropathy, organomegaly, endocrine disturbance, M-protein and skin hyperpigmentation) syndrome occurring in Chinese has been inadequately described. Here we report our experience of 12 Chinese patients with the syndrome. This series is similar to those reported from Japan and from the Western world by the predominance of middle-aged males. Diabetic mellitus, a common feature in POEMS syndrome, was not present in our patients. M-protein of the lambda type was demonstrated in 7 patients by immunoelectrophoresis or by immunofixation electrophoresis. Osteosclerosis in Chinese patients is not as common as in patients reported from Japan or from the Western world; a common radiographic finding in our patients was a compression fracture of the thoraco-lumbar spine with or without sclerotic and lytic lesions. The condition has a grave prognosis; 9 patients die within 4 years after onset of the disease despite various kinds of treatment.