Portal hypertension and neutrocytic ascites in POEMS syndrome

We report a case of portal hypertension and neutrocytic ascites in a 52 year old man with POEMS syndrome. POEMS syndrome is an association of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy and skin changes. Portal hypertension is rare in POEMS syndrome and this is the first time that culture negative neutrocytic ascites has been described with this syndrome.     

Pulmonary hypertension in respiratory distress syndrome

Pulmonary hypertension was associated with nonresponse to surfactant in six premature infants with respiratory distress syndrome. The diagnosis was suspected on the basis of a discrepancy between the X-ray findings and the severity of the clinical status as reflected by hypoxia despite maximal ventilatory support. The diagnosis of pulmonary hypertension was made by pre- and postductal oxygen saturation differences or by echodoppler cardiography, showing suprasystemic right ventricular pressures or right to left shunts through a patent foramen ovale or the ductus arteriosus. The response to surfactant was quantified by the arterial/alveolar (a/A) ratio difference before and 1 hr after therapy (“delta a/A ratio”); the delta a/A ratio was 0 ± 0.01, which indicates a nonresponse. A single dose of 1 mg/kg tolazoline was administrated and the response assessed by a/A difference. A delta a/A ratio of 0.11 ± 0.11 (range 0.02-0.32) represented a dramatic response and enabled oxygenation in these severely ill infants. No significant side effects were observed. We conclude that pulmonary hypertension may be an important and reversible condition in certain cases of respiratory distress syndrome and has to be considered in infants who do not respond to surfactant. © 1995 Wiley-Liss, Inc.     

POEMS syndrome

POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload (peripheral edema, pleural effusions, ascites), sclerotic bone lesions, thrombocytosis, Castleman disease is the first step in effectively managing the disease. A rise in the blood levels of vascular endothelial growth factor is usually confirmatory. More than 95% of patients will have monoclonal lambda sclerotic plasmacytoma(s) or bone marrow infiltration. In patients with a dominant sclerotic plasmacytoma, first line therapy should include radiation to the lesion. Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion and for those who have not demonstrated stabilization of their disease 3 to 6 months after completing radiation therapy. For those patients with diffuse disease, systemic therapy is indicated. Useful approaches include therapy with corticosteroids, low dose alkylator therapy, and high dose chemotherapy with peripheral blood stem cell transplant. Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome. The role of anti-VEGF therapies, immune modulatory drugs, and proteasome inhibitors has not yet been defined, but drugs with known high rates of treatment related neuropathy should not be considered as first line therapy.     

POEMS综合征相关性肺动脉高压一例

患者男性,59岁,2007年6月开始出现双下肢乏力,活动后明显,伴纳差,体重下降.2008年6月开始出现双下肢浮肿,晨轻暮重,伴腹泻;乏力、纳差加重、体重减轻明显.到某医院就诊检查心脏超声提示:重度肺动脉高压伴重度三尖瓣反流,右心房、右心室增大,少量心包积液.     

Pulmonary manifestations of POEMS syndrome: case report and literature review.

Phrenic nerve paresis is an unusual complication of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein spike and skin changes) syndrome. In this report, we describe a case of POEMS syndrome in which a 56-year-old woman presented with dyspnea and ventilatory failure due to bilateral phrenic nerve paralysis. To our knowledge, only one other case of phrenic neuropathy in POEMS syndrome has been reported.     

Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients

BACKGROUND: POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Rare reports of pulmonary manifestations of POEMS syndrome such as pulmonary hypertension exist; however, a comprehensive review of the pulmonary manifestations of POEMS syndrome is lacking. METHODS: Retrospective review of patients with a diagnosis of POEMS syndrome at our institution between 1975 and 2003. Demographics, signs and symptoms, test findings (ie, radiographs, pulmonary function tests, and echocardiography), and survival data were extracted. Kaplan-Meier survival analysis was performed. In addition, categoric variables were compared using the Pearson chi(2) test or Fisher exact test, where appropriate. RESULTS: The study comprised 137 patients (66% male) with a mean age of 51.6 years. Respiratory symptoms were common within 2 years of diagnosis (28%). The median overall survival time was 147 months. Pulmonary manifestations of POEMS syndrome included pulmonary hypertension, restrictive lung disease, respiratory muscle weakness, and an isolated diminished diffusing capacity. Significant radiographic findings such as pleural effusions, diaphragm elevation, and increased cardiac silhouette were seen in 23% of patients. When separated by the presence or absence of respiratory muscle weakness, the median survival time was 87 vs 139 months, respectively (p < 0.05). The presence of cough was associated with reduced survival time. CONCLUSIONS: Pulmonary manifestations of POEMS are common, and both symptomatic and asymptomatic respiratory involvements are frequent on presentation in patients with POEMS syndrome. Respiratory muscle weakness and cough portend a poorer prognosis. These results suggest the need for increased awareness of the association between POEMS syndrome and pulmonary disease to guide appropriate screening and supportive therapy.     

老年重叠综合征患者肺动脉高压临床分析

目的探讨阻塞性睡眠呼吸暂停低通气综合征（OSAHS）合并慢性阻塞性肺病（COPD）,即重叠综合征（OS）与肺动脉高压的关系。方法回顾性分析2000年1月至2009年1月北京世纪坛医院老年医学科门诊及住院,经过完整的肺功能、动脉血气分析、超声心动图及夜间多导睡眠监测的60岁以上患者89例。结果 OSAHS组与OS组之间性别比例、年龄及体质量指数（BMI）均无显著差异（P〉0.05）。超声心动图检查结果提示：5例（5/53,9%）OSAHS患者、23例（23/36,67%）OS患者存在肺动脉高压;多导睡眠监测结果提示,OSAHS组与OS组患者夜间均存在明显低氧血症,表现为夜间最低血氧饱和度的降低及氧减指数的增加,但以OS组患者更为显著;日问的动脉血气分析结果提示,与OSAHS患者比较,OS组患者日间氧饱和度（SaO_2）、动脉氧分压（PaO_2）降低、二氧化碳分压（PaCO_2）增高。结论相对于OSAHS患者,OS患者发展为肺动脉高压和Ⅱ型呼吸衰竭的风险更高。     

Portal hypertension as the initial manifestation of POEMS syndrome: a case report

Background

Portal hypertension has a broad differential diagnosis. POEMS syndrome is an uncommon cause of it. POEMS syndrome is a rare disease involving multiple organs. In differential diagnosis of portal hypertension, POEMS syndrome should be considered especially when other symptoms such as numbness, organomegaly, endocrine alteration and skin changes also present, as it is highlighted by our case.

Case presentation

We report a 46-year-old Chinese male, a teacher, presenting with portal hypertension. Electromyography revealed peripheral neuropathy. Immunofixation showed monoclonal immunoglobulin A lambda protein. The diagnosis of POEMS syndrome was established. After treatment of lenalidomide combined with dexamethasone over 2 years, the patient achieved a considerable improvement.

Conclusion

This case highlights the manifestation of portal hypertension in POEMS syndrome. Lenalidomide with or without dexamethasone is effective for portal hypertension due to POEMS syndrome, though esophageal and gastric varices seems not reversible so easily.

     

Acquired blue sclerae in POEMS syndrome

High‐grade transformation of chronic lymphocytic leukaemia [Richter syndrome (RS)] is rare and represents a unique and uncommon clinical challenge. Clonally related diffuse large B cell type RS is a chemotherapy‐resistant and devastating disease. Patients are typically elderly, immunosuppressed and present with a rapidly deteriorating performance status. Historical outcomes suggest a median overall survival of approximately 8 months. RS remains is an area of high unmet clinical need. The molecular profile and treatment needs of patients are likely to change over time with the advent of novel B cell receptor inhibitors, monoclonal antibodies and BH3 mimetics. Herein, we summarise what is known regarding the molecular drivers of RS and the existing clinical trial data, including the recently published CHOP‐OR (cyclophosphamide, doxorubicin, vincristine, prednisolone and ofatumumab followed by ofatumumab maintenance in newly diagnosed RS) trial. We discuss novel agents in development with a focus on the second‐generation Bruton tyrosine kinase inhibitor acalabrutinib, checkpoint inhibition and the potential role of precision medicine in future trials of RS.     

Pulmonary hypertension in primary Sj?gren's syndrome.

The clinical course of a patient with pulmonary hypertension associated with Sjögren''s syndrome is reported. The clinical features and outcome of this case are compared with those of two other reported cases in the Spanish and Japanese published work. Thus far, pulmonary hypertension has not been reported with primary Sjögren''s syndrome in English publications. These three cases suggest that the development of pulmonary hypertension in primary Sjögren''s syndrome is uncommon but should be included as part of the clinical spectrum.     

POEMS syndrome-related pulmonary hypertension is steroid-responsive

BACKGROUND: The POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal immunoglobulin, Skin changes) syndrome is a rare disease that entails a specific risk for pulmonary arterial hypertension. METHODS AND RESULTS: We report on 2 patients who suffered from POEMS syndrome and pulmonary arterial hypertension for whom a detailed hemodynamic pulmonary study was obtained before and after steroid treatment. Patient 1 had defined post-capillary pulmonary hypertension (PH) ascribed to an abnormally high cardiac output. Patient 2 suffered from severe pre-capillary PH. Under high-dose steroids treatment, clinical symptoms disappeared and PH assessment by sequential hemodynamic study showed a clear-cut improvement in both patients. CONCLUSION: First-line therapy should include corticosteroids in POEMS syndrome-related PH.