title: Monoclonal gammopathy-associated pure red cell aplasia.
abstract: Pure red cell aplasia (PRCA) is a rare disorder characterized by inhibition of erythroid precursors in the bone marrow and normochromic, normocytic anaemia with reticulocytopenia. Among 51 PRCA patients, we identified 12 (24%) patients having monoclonal gammopathy, monoclonal gammopathy of undetermined significance or smouldering multiple myeloma, with presence of monoclonal protein or abnormal serum free light chains and atypical bone marrow features of clonal plasmacytosis, hypercellularity and fibrosis. Thus far, three patients treated with anti-myeloma based therapeutics have responded with reticulocyte recovery and clinical transfusion independence, suggesting plasma cells play a key role in the pathogenesis of this specific monoclonal gammopathy-associated PRCA.
authors.affiliation: Myeloma Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA.|Multiple Myeloma Section, Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Neha
authors.initials: N
authors.lastname: Korde
authors.affiliation: Multiple Myeloma Section, Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Yong
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: National Heart, Lung, and Blood Institute, Hematology Branch, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Kelsey
authors.initials: K
authors.lastname: Loeliger
authors.affiliation: National Heart, Lung, and Blood Institute, Hematology Branch, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Andrea
authors.initials: A
authors.lastname: Poon
authors.affiliation: Office of Blood Research and Review/Center for Biologics Evaluation and Research, Food and Drug Administration, Silver Spring, MD, USA.
authors.forename: Olga
authors.initials: O
authors.lastname: Simakova
authors.affiliation: Multiple Myeloma Section, Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Adriana
authors.initials: A
authors.lastname: Zingone
authors.affiliation: Multiple Myeloma Section, Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Rene
authors.initials: R
authors.lastname: Costello
authors.affiliation: National Heart, Lung, and Blood Institute, Hematology Branch, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Richard
authors.initials: R
authors.lastname: Childs
authors.affiliation: Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Pierre
authors.initials: P
authors.lastname: Noel
authors.affiliation: Internal Medicine/Hematology-Oncology, University of Michigan Medical School, Ann Arbor, MI, USA.
authors.forename: Samuel
authors.initials: S
authors.lastname: Silver
authors.affiliation: Murtha Cancer Center, Walter Reed National Military Medical Center, Bethesda, MD, USA.
authors.forename: Mary
authors.initials: M
authors.lastname: Kwok
authors.affiliation: Murtha Cancer Center, Walter Reed National Military Medical Center, Bethesda, MD, USA.
authors.forename: Clifton
authors.initials: C
authors.lastname: Mo
authors.affiliation: National Heart, Lung, and Blood Institute, Hematology Branch, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Neal
authors.initials: N
authors.lastname: Young
authors.affiliation: Myeloma Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA.|Multiple Myeloma Section, Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Ola
authors.initials: O
authors.lastname: Landgren
authors.affiliation: National Heart, Lung, and Blood Institute, Hematology Branch, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Elaine
authors.initials: E
authors.lastname: Sloand
authors.affiliation: Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Irina
authors.initials: I
authors.lastname: Maric
mesh_terms: D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology; D003907:Dexamethasone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000097:blood; D000077269:Lenalidomide; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000175:diagnosis* / Q000473:pathology; D010265:Paraproteinemias / Q000175:diagnosis* / Q000473:pathology; D010950:Plasma Cells / Q000502:physiology; D012010:Red-Cell Aplasia, Pure / Q000175:diagnosis* / Q000473:pathology; D017701:Reticulocyte Count; D012156:Reticulocytes / Q000502:physiology; D013792:Thalidomide / Q000031:analogs & derivatives / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D007136:Immunoglobulins; C009823:M-proteins (Myeloma); D013792:Thalidomide; D003907:Dexamethasone; D000077269:Lenalidomide
keywords: anaemia; monoclonal gammopathy of undetermined significance; myeloma; plasmacytosis; pure red cell aplasia