Severe reversible pulmonary hypertension in smoldering multiple myeloma: Two cases and review of the literature

Wayne L. Feyereisn, Eric R. Fenstad, Robert B. McCully, Martha Lacy

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)211-216
Number of pages6
JournalPulmonary Circulation
Volume5
Issue number1
DOIs
StatePublished - Mar 1 2015

Fingerprint

Multiple Myeloma
Pulmonary Hypertension
POEMS Syndrome
Paraproteinemias
Scleromyxedema
Right Ventricular Dysfunction
Dermatomyositis
Stem Cell Transplantation
Ventricular Pressure
Therapeutics
Blood Pressure
Drug Therapy
Skin

Keywords

  • Hypertension pulmonary
  • Multiple myeloma
  • Outcomes

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Severe reversible pulmonary hypertension in smoldering multiple myeloma : Two cases and review of the literature. / Feyereisn, Wayne L.; Fenstad, Eric R.; McCully, Robert B.; Lacy, Martha.

In: Pulmonary Circulation, Vol. 5, No. 1, 01.03.2015, p. 211-216.

Research output: Contribution to journalArticle

Feyereisn, Wayne L. ; Fenstad, Eric R. ; McCully, Robert B. ; Lacy, Martha. / Severe reversible pulmonary hypertension in smoldering multiple myeloma : Two cases and review of the literature. In: Pulmonary Circulation. 2015 ; Vol. 5, No. 1. pp. 211-216.
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