CASE OF THE LATE DIAGNOSIS OF POEMS-SYNDROME

Klinicist

S. V. Meres¹  P. N. Barlamov¹  E. R. Vasiliev¹  V. V. Shchekotov¹  M. E. Golubeva¹ 

[1] Perm City Hematological CenterPerm State Medical Academy;

关键词: poems-syndrome;    polyneuropathy;    monoclonal gammopathy;    organomegaly;    hepatosplenomegaly;    paraprotein;    endocrinopathy;    diabetes;    multiple myeloma;    plasmacytoma;    alkeran;    prednisone;   

DOI  :  10.17650/1818-8338-2011-4-48-52

来源: DOAJ

【 摘 要 】

POEMS-syndrome (P — polyneuropathy, O — organomegaly, E — endocrinopathy, M — M-protein, S — skin) in 64-year old patient isdescribed in the article. The clinical picture was marked by such symptoms, as polyneuropathy, multiple myeloma, organomegaly (hepatosplenomegaly), endocrinopathy (diabetes), skin changes (redness and induration of the dermis in the neck), fever, hypoproteinemia, edema, weight loss, thrombocytosis. Bone-destructive syndrome was absent. In myelogram 18 % of the cells with signs of some plasmatic anaplasia were found. In blood, low level of paraprotein secretion Aλ, increased β2-microglobulin was fixed. A course of therapy with prednisolone and alkeranom was accompanied by slight positive effect. However, a second course was interrupted in the third day due to worsening of concomitant cardiac disease (ischemic heart disease in combination with hypertension). Last episode of recurrent of pulmonary edema occurred fatal. Autopsy study was not conducted. This observation illustrates the need for more rigorous examination (myelogram, immunochemical study of blood and urine) in the presence of clinical signs of POEMS-syndrome for the timely diagnosis of the underlying disease and its treatment.

【 授权许可】

Unknown