POEMS综合征相关性肾病

POEMS Syndrome Related Nephropathy

病史及临床表现 :患者 ,男 ,4 7岁 ,因消瘦、色素沉着 3年 ,四肢麻木、性功能减退 2年 ,双下肢水肿半年入院。查体 :全身皮肤色素沉着 ,浅表淋巴结、颌下腺、甲状腺及肝脏肿大 ,四肢末端痛觉减退 ,双手、足杵状指 (趾 )。实验室及病理检查 :尿蛋白 (2 +) ;血清免疫球蛋白电泳检查示IgA -λ型M蛋白血症。骨髓穿刺、活检示浆细胞占 5 5 % ,可见异型的浆细胞。肾脏活检 :光镜下见肾小球系膜细胞及基质增生 ,少数毛细血管基膜增厚呈节段性双轨 ;电镜检查示基质增生 ,毛细血管内皮空泡样变性 ,内皮下间隙增宽并见致密复合物沉积。治疗及随访 :确诊POEMS综合征后 ,即给予强的松治疗。随访 10月后患者症状、体征及实验室检查结果明显好转。结论 :POEMS综合征相关性肾病临床症状的轻重与病理变化的严重程度无相关关系 ,临床上易被忽视 ;组织学上以膜增殖性肾小球肾炎样病变为主 ;其发病机理推测与毛细血管内皮细胞的慢性损伤有关 ;

A 47 year-old male was admitted to the hospital because of weight loss and pigmentation for 3 years,paresthesia of extremities and hypogonadism for 2 years,and bilateral pretibial edema for 1/2 years.Physical examination revealed generalized pigmentation,diffuse peripheral lymphadenopathy,slight finger clubbing,submaxillary gland and thyroid enlargement,ascites,moderate hepatomegaly,bilateral pretibial edema and polyneuropathy.The blood pressure was 90/60mmHg.Examination Erythrocyte sedimentation rate(ESR)was 4mm/h,mild proteinuria(2+),a few red blood cell(3～6 per high-power field)was found.Relevant laboratory data after admission were as follows:Blood urea nitrogen(BUN)8 0mmol/L,and serum creatine 110 9μmol/L,urinic acid 463 8μmol/L.T 3 0 96(range:1 30～3 10)nmol/L,T 4 130 0(range:66 0～174)nmol/L,TSH 0 389(range:0 27～4 20)mU/L,rT 30 86(range:0 34～0 85)nmol/L,M-protein IgA-λ type.The ultrasound examination showed enlarged liver,spleen,ascites.The biopsy of an inguinal lymph node disclosed reactive lymphadenosis.Examination of an aspirate sample of the patient's bone marrow yielded 5 5% of dysmorphic plasmocytes.The bone marrow biopsy revealed neither plasmocytoma nor a malignant systemic disease.Renal biopsy demonstrated prominent glomerular changes which were similar to membranoproliferative glomerulonephritis(MPGN).Mesangial proliferation and thickienng of the capillary wall with double contour evoke by light microscopy,mild to moderate interstitial fibrosis were presented.Immunofluorescent microscopy of the renal biopsy revealed the segmental localization of immunoglobulin(IgM)in some of the glomerular capillaries.Immunostaining for the kidney tissue showed a distribution of typeⅣ collagen and λ light chain in some of the glomerular capillaries and mesangial area as well as tubular cells.The moderately to severely expanded mesangium and widened subendothelial space with widespread deposition of amorphous material as well as swelling and vascuolization of endothlial cells in the glomerular capillaries were noted by electron microscopy.Treatment and follow-up:As POEMS syndrome was diagnosed,predinisone(30mg,Bid)was given.Ten months later,an improvement in the clinical condition of the patient was observed,together with disappearance of the M-protein component and the normalization of the initially abnormal laboratory parameters.Conclusion:According to the literature,in patient with POEMS syndrome,pathological activity represented by mesangiolysis does not correspond to the extent of renal insufficiency,urinalysis is always mild and does not reflect the severity of renal lesion,mild proteinuria often passes unnoticed.Most times a membranoproliferative-like histologicall glomerulopathy;it is proposed that the pathological findings might be caused by chronic injury of endothelial cells,the clinical symptoms and renal involvement respond to corticosteroid therapy.