Pulmonary hypertension in POEMS syndrome

Li, Jian; Tian, Zhuang; Zheng, Haoyi; Zhang, Wei; Duan, Minghui; Liu, Yongtai; Cao, Xinxin; Zhou, Daobin

doi:10.3324/haematol.2012.073031

Cited by 55 publications

(90 citation statements)

References 27 publications

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“…1 The survival of patients with pulmonary hypertension and POEMS is worse than those with POEMS alone, with median survival time of 54 months in large case series. 4 Although reversibility of pulmonary hypertension has been reported with corticosteroids, [1][2][3][4][5] this is, to our knowledge, the first case reported in which PH has resolved following radical radiotherapy to bone lesions. It is important to consider associated syndromes when a diagnosis of PH is made, because treatment of the underlying condition may lead to improvement in PH, not just in POEMS but in other conditions, such as human immunodeficiency virus infection and systemic lupus erythematosus.…”

Section: Case Descriptionmentioning

confidence: 95%

“…1,2 The suggested prevalence of associated PH is 27%-48%, although these figures were obtained using TTE to estimate pulmonary artery pressure. [3][4][5] In addition, thrombotic events can be associated with POEMS, with reports of an association with chronic thromboembolic pulmonary hypertension. 1 The survival of patients with pulmonary hypertension and POEMS is worse than those with POEMS alone, with median survival time of 54 months in large case series.…”

Section: Case Descriptionmentioning

confidence: 99%

“…Reversal of PH with corticosteroids has been documented in the literature. [1][2][3][4][5] We report a unique case of PH associated with POEMS syndrome with poor initial response to PH-targeted therapy with pulmonary vasodilators, subsequently successfully treated with radical radiotherapy locally to bone lesions with resolution of PH at repeat right heart catheterization (RHC). …”

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confidence: 99%

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Pulmonary Hypertension in Poems Syndrome: Resolution following Radiotherapy

et al. 2014

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Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary artery pressure (mPAP) ≥25 mmHg. It may be idiopathic or arise as a consequence of a number of diverse conditions. PH has been reported in association with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes), with reversal following systemic treatment with corticosteroids. We report a case of pulmonary hypertension associated with POEMS syndrome treated with radical radiotherapy locally to bone lesions with resolution of systemic disease.Keywords: plasmacytoma, sildenafil, bosentan. Pulmonary hypertension (PH), defined by a mean pulmonary artery pressure (mPAP) ≥25 mmHg, may be idiopathic or arise as a consequence of a number of diverse conditions. PH has been reported in association with POEMS syndrome, a rare paraneoplastic syndrome characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Reversal of PH with corticosteroids has been documented in the literature. [1][2][3][4][5] We report a unique case of PH associated with POEMS syndrome with poor initial response to PH-targeted therapy with pulmonary vasodilators, subsequently successfully treated with radical radiotherapy locally to bone lesions with resolution of PH at repeat right heart catheterization (RHC). CASE DESCRIPTIONA 46-year-old female nonsmoker presented in September 2011 with a 3-month history of breathlessness. Her medical history consisted of treated hypothyroidism and benign intracranial hypertension. She was noted to have peripheral edema and a palpable supraclavicular lymph node.Lung function was normal with the exception of a reduced carbon monoxide diffusing capacity, which was 40% of the predicted value. Chest radiograph findings (Fig. 1) Computed tomography (CT) pulmonary angiogram showed no evidence of thromboembolic disease but confirmed right heart dilatation, extravascular volume overload with subcutaneous edema, pericardial effusion, small left pleural effusion, and ascites. A 15-cm splenomegaly with widespread small volume lymphadenopathy (maximally measuring 15 mm in the mediastinum) was also present. Ventilation perfusion scan indicated low probability for thromboembolic disease.PH was confirmed at RHC with mPAP of 70 mmHg (systolic PAP, 88 mmHg; diastolic PAP, 50 mmHg), right atrial pressure (RAP) of 20 mmHg, cardiac index (CI) of 2.5 L/min/m 2 , pulmonary vascular resistance (PVR) of 13.8 Wood units, and pulmonary artery wedge pressure (PAWP) of 16 mmHg. The diastolic pressure gradient was significantly elevated, which suggests precapillary pulmonary vascular disease. 6

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Section: Case Descriptionmentioning

confidence: 95%

Section: Case Descriptionmentioning

confidence: 99%

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Pulmonary Hypertension in Poems Syndrome: Resolution following Radiotherapy

et al. 2014

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“…The course of POEMS syndrome is usually chronic with modern estimated median survivals of nearly 14 years [48,58] Only fingernail clubbing, extravascular volume overload-i.e., effusions, edema, and ascites [48], respiratory symptoms [58], pulmonary hypertension [80] impaired DLCO, and papilledema [52] have been associated with a significantly shorter overall survival. The number of POEMS features does not affect survival [5,10].…”

Section: Risk Stratificationmentioning

confidence: 99%

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

2015

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Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome.Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy.Risk‐adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3–6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. The benefit of anti‐VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 90:951–962, 2015. © 2015 Wiley Periodicals, Inc.

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“…6 PH occurs in 27%-48% of patients with POEMS syndrome and may resolve after POEMS treatment. 7 The mechanism of PH associated with myeloproliferative disorders is not known. Recently, we identified two patients with smoldering MM (SMM) who developed severe, symptomatic PH that resolved after treatment of their SMM.…”

Section: Case Descriptionmentioning

confidence: 99%

Severe Reversible Pulmonary Hypertension in Smoldering Multiple Myeloma: Two Cases and Review of the Literature

et al. 2015

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An association between pulmonary hypertension (PH) and POEMS syndrome (characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) as well as other plasma cell dyscrasias, including multiple myeloma (MM), has been shown to exist. Recent case reports have identified a reversible form of PH that occurs outside of previously identified etiologies. We report two cases of PH in the setting of smoldering MM (SMM) that resolved with chemotherapy and stem cell transplantation. Although other features were individualized among the cases (dermatomyositis, scleromyxedema), treatment of MM and SMM resulted in a normalization of right ventricular systolic pressure and improvement in right ventricular dysfunction that was previously unresponsive to PH therapies. The magnitude and sustained nature of reversibility in these four cases could offer clues about the pathophysiology and treatment of PH.Keywords: hypertension pulmonary, multiple myeloma, outcomes. CASE DESCRIPTIONThe two recent updates to the clinical classification of pulmonary hypertension (PH) 1,2 state that hematologic disorders-and, more specifically, myeloproliferative disorders-can be associated with PH (group 5.1). It has been reported that PH can occur in the chronic myeloproliferative disorders of myeloid metaplasia with myelofibrosis, polycythemia vera, and essential thrombocythemia. 3 PH can also occur in patients with plasma cell dyscrasias, including those with POEMS syndrome (characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) 4 and amyloidosis, 5 as well as after thalidomide treatment of multiple myeloma (MM). 6 PH occurs in 27%-48% of patients with POEMS syndrome and may resolve after POEMS treatment. 7 The mechanism of PH associated with myeloproliferative disorders is not known. Recently, we identified two patients with smoldering MM (SMM) who developed severe, symptomatic PH that resolved after treatment of their SMM. and an increase in his abdominal girth. Serum protein electrophoresis and immunofixation revealed an immunoglobulin G (IgG) λ of 2.1 g/dL and a λ immunoglobulin free light chain of 53.6 mg/dL (reference range, 0.57-2.63 mg/dL). A 24-hour urine protein was 737 mg, with a urine M-spike of 35 mg/dL on protein electrophoresis. A workup for POEMS syndrome was negative. Bone survey was negative for lytic or sclerotic bone lesions. Bone marrow biopsy showed 20%-30% plasma cells, while renal biopsy was consistent with membranoproliferative glomerulonephritis (Table 1). He was diagnosed as having SMM. Echocardiography showed evidence of severe PH. The estimated right ventricular systolic pressure (RVSP) by Doppler was 84 mmHg, the right ventricle was moderately to severely enlarged, there was mild RV systolic dysfunction, and there was severe tricuspid regurgitation. He had a left ventricular ejection fraction of 65% without pericardial effusion. Echocardiographic and hemodynamic catheterization parameters are presented in Table 2. Mild ...

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Pulmonary hypertension in POEMS syndrome

Cited by 55 publications

References 27 publications

Pulmonary Hypertension in Poems Syndrome: Resolution following Radiotherapy

Pulmonary Hypertension in Poems Syndrome: Resolution following Radiotherapy

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

Severe Reversible Pulmonary Hypertension in Smoldering Multiple Myeloma: Two Cases and Review of the Literature

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