Severe Pulmonary Hypertension Caused by Smoldering Plasma Cell Myeloma: An Autopsy Case of POEMS Syndrome

Chinen, Katsuya; Fujioka, Yasunori

doi:10.1155/2012/836893

Cited by 8 publications

(5 citation statements)

References 30 publications

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“…Such patients characteristically present with back pain, radicular pain, sensory-motor disturbances, and bladder and rectal disturbances (7). We com-pared four cases of MM complicated by POEMS syndrome reported previously (Table 2) (8)(9)(10)(11). Including our case, all five reported cases had a middle-aged onset, gait disturbance, and numbness in the lower limbs at the onset.…”

Section: Discussionmentioning

confidence: 67%

Improvement in the Symptoms and VEGF Levels after Resection of an Extrame Dullary Spinal Tumor and Additional Chemotherapy in a Patient with Multiple Myeloma Complicated with POEMS Syndrome

et al. 2021

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We herein report a case of multiple myeloma and polyneuropathy, organomegaly, endocrinopathy, myeloma protein, and skin changes (POEMS) syndrome. The patient experienced exacerbated gait disturbance due to weakness and numbness in the lower limbs. Thoracic magnetic resonance imaging revealed an extramedullary tumor with spinal compression that required surgical resection. Plasmacytoma was diagnosed based on a biopsy. Radiation, betamethasone, and chemotherapy were therefore administered. Surgical removal of extramedullary tumors improved his symptoms, motor conduction velocity, and amplitude of the muscle action potential in the peroneal and tibial nerves, as shown by the nerve conduction study. Surgery also decreased the serum vascular endothelial growth factor levels. The patient required additional chemotherapy due to multiple myeloma and showed better outcomes nine months after discharge. The benefits of some treatments remain controversial due to the small number of patients. However, our findings reveal that an early diagnosis and comprehensive treatment may result in better outcomes in such patients.

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Section: Discussionmentioning

confidence: 67%

Improvement in the Symptoms and VEGF Levels after Resection of an Extrame Dullary Spinal Tumor and Additional Chemotherapy in a Patient with Multiple Myeloma Complicated with POEMS Syndrome

et al. 2021

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“…The postmortem examination of the lung of a patient with POEMS syndrome revealed pulmonary vascular remodelling, including plexiform lesions, which are consistent with pulmonary arterial hypertension (PAH) . On the other hand, an autopsy study of another such patient revealed stenosis and occlusion of pulmonary arteries due to plasma cell infiltration, different from the histology of PAH . These reports indicate that histological findings of PH associated with POEMS could be heterogeneous.…”

Section: Discussionmentioning

confidence: 93%

Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome

Ohashi

Nishimura

Sugimoto

et al. 2019

Respirology Case Reports

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Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell disease. Patients with POEMS syndrome are considered to be at a high risk of developing pulmonary hypertension (PH). We report a 51‐year‐old woman diagnosed with PH associated with POEMS syndrome. She was started on dexamethasone and thalidomide. Although, the plasma vascular endothelial growth factor (VEGF) level decreased, systolic pulmonary artery pressure (sPAP) remained high. Auto‐peripheral blood stem cell transplantation improved the plasma VEGF and sPAP levels. Four years later, she presented with dyspnoea on exertion, and elevated plasma VEGF and sPAP levels. Subsequently, on administering sildenafil and macitentan, the plasma VEGF and PH levels improved. Pulmonary vasodilators can be considered when PH remains after treatment of POEMS syndrome.

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“…In one report of POEMS syndrome, direct infiltration of the pulmonary vasculature by plasma cells was also reported. 36 Although none of our 3 patients exhibited characteristic features of POEMS syndrome, the underlying mechanisms of angiogenesis and inflammation in this disease may help elucidate the pathogenesis of PH in MM. The POEMS syndrome is associated with elevated VEGF levels, and this has been incorporated into the diagnostic criteria for the disease.…”

Section: Role Of Inflammation and Poems Syndromementioning

confidence: 87%

“…In one report of POEMS syndrome, direct infiltration of the pulmonary vasculature by plasma cells was also reported. 36…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Hypertension Complicating Multiple Myeloma

et al. 2015

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Pulmonary hypertension (PH) is an infrequently reported complication of multiple myeloma (MM). PH has been more commonly associated with amyloidosis, myeloproliferative diseases, and the POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome. PH in MM is typically mild to moderate and may be secondary to a variety of conditions, which include left ventricular dysfunction, high-output cardiac failure, chronic kidney disease, treatment-related toxicities, and precapillary involvement. We describe 3 patients with MM and severe PH. Each patient underwent right heart catheterization. All patients demonstrated elevated pulmonary pressures, transpulmonary gradients, and pulmonary vascular resistance. Each patient was ultimately treated with pulmonary vasodilator therapy with improvement in cardiopulmonary symptoms. Additional studies are needed to define the prevalence, prognosis, and pathogenesis of PH in this complex population and to help clarify who may benefit from targeted PH therapy.Keywords: pulmonary hypertension, multiple myeloma, pulmonary vasculature, amyloidosis. Multiple myeloma (MM) is a hematological malignancy characterized by the neoplastic proliferation of plasma cells within bone marrow and extramedullary sites. It is distinguished from other disorders of plasma cell proliferation by the presence of CRAB features, defined as hypercalcemia, renal insufficiency, anemia, and lytic bone lesions. 1 Although cardiovascular pathology has been frequently associated with MM, pulmonary hypertension (PH), which is characterized by elevated pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR), is not a commonly recognized complication. We report the clinical and hemodynamic features of 3 patients with established MM and severe PH. We also provide a review of the literature and discuss the different pathways for development of PH in this mixed population. CASE DESCRIPTION Case 1Patient 1 was a 72-year-old man who presented to the PH clinic with exertional dyspnea. His medical history was significant for immunoglobulin A (IgA) κ MM treated with hematopoietic autologous stem cell transplantation (HSCT) 3 years earlier. At the time of presentation, the patient was receiving maintenance chemotherapy with lenalidomide to help extend clinical remission. In addition, he had a history of permanent atrial fibrillation and severe aortic stenosis. Physical examination revealed a 3/6 crescendo-decrescendo murmur at the upper sternal base radiating to the carotids and a soft 1/6 holosystolic murmur at the lower sternal base. Transthoracic echocardiography (TTE) revealed an ejection fraction (EF) of 62%, a dilated left atrium (LA), pulmonary artery systolic pressure (PASP) of 83 mmHg, flattening of the interventricular septum, a severely dilated right ventricle (RV) and right atrium (RA), mildly reduced RV function, and an aortic valve area of 0.73 cm 2 . A right heart catheterization (RHC) showed PAP of 96/32 mmHg (mean, 53 mmHg) with a left ventricular (L...

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Severe Pulmonary Hypertension Caused by Smoldering Plasma Cell Myeloma: An Autopsy Case of POEMS Syndrome

Cited by 8 publications

References 30 publications

Improvement in the Symptoms and VEGF Levels after Resection of an Extrame Dullary Spinal Tumor and Additional Chemotherapy in a Patient with Multiple Myeloma Complicated with POEMS Syndrome

Improvement in the Symptoms and VEGF Levels after Resection of an Extrame Dullary Spinal Tumor and Additional Chemotherapy in a Patient with Multiple Myeloma Complicated with POEMS Syndrome

Effectiveness of pulmonary vasodilators on pulmonary hypertension associated with POEMS syndrome

Pulmonary Hypertension Complicating Multiple Myeloma

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