Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome with dilated cardiomyopathy: A case report

BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro-gressive sensorimotor polyneuropathy,organ enlargement,endocrine disorders,darkening skin,a monoclonal plasma cell proliferative disorder,and lymph node hyperplasia.The organomegaly consists of hepatosplenomegaly and/or lym-phadenopathy;cases of cardiomyopathy are rare.Diagnoses are often delayed because of the atypical nature of the syndrome,exposing patients to possibly severe disability.Therefore,identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.lenalidomide and dexamethasone.CONCLUSION When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin,the POEMS syndrome is the most possible diagnosis.

Polyneuropathy organomegaly endocrinopathy M-protein and skin changes syndrome with ascites as an early-stage manifestation:A case report

BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome are diverse.Due to the insidious onset and lack of specific early-stage manifestations,POEMS syndrome is easily misdiagnosed or never diagnosed,leading to delayed treatment.Neurological symptoms are usually the first clinical manifestation,while ascites is a rare symptom in patients with POEMS syndrome.CASE SUMMARY A female patient presented with unexplained ascites as an initial symptom,which is a rare early-stage manifestation of the condition.After 1 year,the patient gradually developed progressive renal impairment,anemia,polyserosal effusion,edema,swollen lymph nodes on the neck,armpits,and groin,and decreased muscle strength of the lower extremities.The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion.Treatment comprised bortezomib+dexamethasone,continuous renal replacement therapy,chest and abdominal closed drainage,transfusions of erythrocytes and platelets,and other symptomatic and supportive treatments.The patient’s condition initially improved after treatment.However,then her symptoms worsened,and she succumbed to the illness and died.CONCLUSION Ascites is a potential early manifestation of POEMS syndrome,and this diagnosis should be considered for patients with unexplained ascites.Furthermore,multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.

Effective treatment of polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes syndrome with congestive heart failure:A case report

BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a plasma cell proliferative disorder.The syndrome is characterized by elevated plasma cells,platelets,and vascular endothelial growth factor levels.Although heart disease rarely occurs in POEMS syndrome,the death rate increases sharply after heart failure.We report a patient who initially presented with an endocrine disease and developed congestive heart failure related to POEMS syndrome 9 years later.CASE SUMMARY A 23-year-old woman with no history of menstruation and a 9-year history of type I diabetes reported feeling breathless after activities.She could not lie down and rest at night.Three months prior,she experienced pain and increased tension in her left thigh accompanied by tenderness and edema in both lower extremities.The chief complaint upon hospital admission was that blood sugar has increased for more than 9 years,pain in the left thigh,and edema in both legs for more than 2 mo.After a multisystem evaluation,she was diagnosed with POEMS syndrome.Her echocardiogram showed left ventricular dilation with systolic dysfunction,and the left ventricular ejection fraction was only 38%with severely elevated brain natriuretic peptide.She received a combination of dexamethasone and thalidomide for 1 mo,but her symptoms did not improve.Therefore,we added a two-per-week bortezomib injection.After 2 wk,the patient’s heart function had improved significantly.CONCLUSION This case provides information about the treatment of POEMS syndrome with complications and highlights the challenges of developing a standardized treatment.

Clinical and electrophysiological profiles in early recognition of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome

Background:The detection of polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes (POEMS) syndrome at early stage is challenging for neurologists.Since polyneuropathy could be the first manifestation,it could be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP).The present study aimed to determine the clinical and electrophysiological features of POEMS syndrome to distinguish from CIDP.Methods:The data of a group of patients with POEMS (n =17) and patients with CIDP (n =17) in Zhongshan Hospital Fudan University from January 2015 to September 2017 were analyzed in this retrospective study.The clinical features,neurological symptoms,and electrophysiological findings were compared between the two groups.Results:Clinically,patients with POEMS demonstrated significantly more neuropathic pain in the lower extremities than patients with CIDP (58.8 % vs.11.8 %,P =0.01).Multisystem features like edema,skin change,organomegaly,and thrombocytosis were also pointed towards the diagnosis of POEMS syndrome.Electrophysiologically,terminal latency index (TLI) was significantly higher in patients with POEMS than that in patients with CIDP (median nerve:0.39 [0.17-0.52] vs.0.30 (0.07-0.69),Z =-2.413,P =0.016;ulnar nerve:0.55 [0.23-0.78] vs.0.42 [0.12-0.70],Z =-2.034,P =0.042).Patients with POEMS demonstrated a higher frequency of absent compound muscle action potential of the tibial nerve (52.9% vs.17.6%,P =0.031),less conduction block (ulnar nerve:0 vs.35.3%,P =0.018),and less temporal dispersion (median nerve:17.6% vs.58.8%,P =0.032) than CIDP group.The combination of positive serum monoclonal protein and high TLI (if either one or both were present) discriminated POEMS from CIDP with a sensitivity of 94.1% and 47.1% and specificity of 76.5% and 100.0%,respectively.Conclusions:POEMS syndrome could be distinguished from CIDP through typical clinical and electrophysiological characteristics in practice.The combination of serum monoclonal protein and high TLI might raise the sensitivity of detecting POEMS syndrome.

Gastromegaly infiltrated with plasma cells： a new feature of organomegaly in patients with POEMS syndrome

Organomegaly is a major component of POEMS syndrome （an acronym of polyneuropathy,organomegaly, endocrinopatfiy, M protein, and-skin changes）, which is a rare multisystem disorder of unknown pathogenesis, In patients with POEMS syndrome, the organs involved in organomegaly usually are the liver, spleen, and lymph nodes,a-4 Three studies have reported the frequencies of hepatomegaly as 25%, 68%, and 78%; of splenomegaly as 22%, 52%, and 35%;

Volumetric modulated arc radiotherapy for limited osteosclerotic myeloma

AIM:To assess the feasibility of volumetric intensity-modulated arc radiotherapy (VMAT) in patients with limited polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. METHODS:A 70-year-old male with histologically confirmed osteosclerotic myeloma was treated in our department in July 2010 with VMAT. Fourty-six Gray in 23 fractions were given on three bone lesions. Doses delivered to target volume and critical organs were compared with a tridimensional conformal radiotherapy (3D-RT) plan. Treatment was well tolerated without any side effects.RESULTS:VMAT improved dose homogeneity within the target volume, as compared to 3D-RT (standard deviations:2.9 Gy and 1.6 Gy for 3D and VMAT, respectively). VMAT resulted in a better sparing of critical organs. Dose delivered to 20% of organ volume (D20) was reduced from 22 Gy (3D-RT) to 15 Gy (VMAT) for small bowel, from 24 Gy (3D-RT) to 17 Gy (VMAT) for bladder and from 47 Gy (3D-RT) to 3 Gy (VMAT) for spinal cord. Volumes of critical organs that received at least 20 Gy (V20) were decreased by the use of VMAT, as compared to 3D-RT (V20 bladder:10% vs 99%; V20 small bowel:6% vs 21%). One year after treatment completion, no tumor progression has been reported. CONCLUSION:VMAT improved dose distribution as compared to 3D-RT for limited osteosclerotic myeloma, with better saving of critical organs.