Non-thromboembolic pulmonary hypertension in multiple myeloma, after thalidomide treatment: A pilot study

Lafaras, Christos; Mandala, Evdokia; Verrou, Evgenia; Platogiannis, Dimitrios; Barbetakis, Nikolaos; Bischiniotis, Theodoros; Zervas, Konstantinos

doi:10.1093/annonc/mdn287

Cited by 15 publications

(17 citation statements)

References 42 publications

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“…PH in the absence of thromboembolic events has also been described in 3 patients with MM during thalidomide treatment [5, 6, 7] and in a pilot study [1]. We also experienced occurrence of PH in a MM patient during treatment with thalidomide.…”

Section: Introductionsupporting

confidence: 65%

“…Furthermore, in the pilot study of Lafaras et al [1], 4 of the 82 treated patients had PH and improved after discontinuation of therapy. Our patient showed clinical and echocardiographic signs of PH after a few days of thalidomide therapy.…”

Section: Discussionmentioning

confidence: 99%

“…Multiple myeloma (MM) is thrombogenic as a consequence of multiple hemostatic effects, including elevated interleukin-6 levels, procoagulant antibody formation, paraprotein interference with fibrin structure, activated protein C resistance and endothelial damage [1]. …”

Section: Introductionmentioning

confidence: 99%

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Reversible Pulmonary Hypertension Related to Thalidomide Treatment for Multiple Myeloma

et al. 2011

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Multiple myeloma (MM) is thrombogenic as a consequence of multiple hemostatic effects. Thalidomide is an effective treatment; however, it has been associated with an increased risk of thromboembolic events including pulmonary hypertension (PH). PH in the absence of thromboembolic events has also been described in some patients with MM during thalidomide treatment. We experienced occurrence of PH in a MM patient during treatment with thalidomide. A 79-year-old woman was diagnosed with IgG lambda MM and was started on thalidomide treatment. About a month later, she presented with asthenia, palpitation and dyspnoea on exertion and was hospitalized. An echocardiography revealed severe PH (systolic pulmonary artery pressure 75 mm Hg) without paradoxic movement of ventricular septum or right ventricular dysfunction signs; a previous echocardiography was normal. Pulmonary computed tomography and perfusion scan were negative for pulmonary embolism. Based on the hypothesis of a pharmacological pathogenesis, thalidomide was promptly interrupted. About a month later, she was hospitalized for further investigations. Physical examination documented absence of dyspnoea or other respiratory signs, and echocardiography showed normal right ventricular function and normal pulmonary artery pressure. As in the cases reported in the literature, we suggest a possible direct correlation between thalidomide and PH, since in all cases a rapid decrease of pulmonary artery pressure after thalidomide discontinuation was observed.

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Section: Introductionsupporting

confidence: 65%

Section: Discussionmentioning

confidence: 99%

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Reversible Pulmonary Hypertension Related to Thalidomide Treatment for Multiple Myeloma

et al. 2011

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“…Of note, carfilzomib has been associated with a rate of PH of approximately 3%, with 1% of patients having severe PH. 32 Patient 3 was treated with numerous chemotherapeutic agents, including carfilzomib both before and after PH was detected (Table 2).…”

Section: Treatment-related Vasculopathymentioning

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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“…This outcome suggests a possible association between MM and PH. In addition, Lafaras et al reported that some MM patients receiving thalidomide develop non-thromboembolic PH (21). Therefore, lenalidomide, not thalidomide, was chosen as the third drug in the combination therapy used in this case, in which the patient had severe PH.…”

Section: Introductionmentioning

confidence: 98%

Successful Bortezomib/dexamethasone Induction Therapy with Lenalidomide in an Elderly Patient with Primary Plasma Cell Leukemia Complicated by Renal Failure and Pulmonary Hypertension

et al. 2014

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Primary plasma cell leukemia (PPCL) is a rare disease that progresses rapidly. In such cases, it is difficult to achieve remission, and early intensive chemotherapy is recommended. We herein describe the case of a 76-year-old man with PPCL complicated by renal failure and pulmonary hypertension. Bortezomib/dexamethasone induction therapy with lenalidomide was administered in association with continuous hemodiafiltration (CHDF). Complete remission was achieved after a single course of treatment, resulting in the cessation of CHDF. With the patient in remission, the administration of beraprost and bosentan resulted in improvements in the pulmonary hypertension. The results of this case report support the use of bortezomib/lenalidomide/dexamethasone combination therapy as an effective treatment for elderly PPCL patients with various complications.

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Non-thromboembolic pulmonary hypertension in multiple myeloma, after thalidomide treatment: A pilot study

Cited by 15 publications

References 42 publications

Reversible Pulmonary Hypertension Related to Thalidomide Treatment for Multiple Myeloma

Reversible Pulmonary Hypertension Related to Thalidomide Treatment for Multiple Myeloma

Pulmonary Hypertension Complicating Multiple Myeloma

Successful Bortezomib/dexamethasone Induction Therapy with Lenalidomide in an Elderly Patient with Primary Plasma Cell Leukemia Complicated by Renal Failure and Pulmonary Hypertension

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