Monoclonal gammopathy‐associated pure red cell aplasia

Summary 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 u...

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Bibliographic Details
Published in:British journal of haematology 2016-06, Vol.173 (6), p.876-883
Main Authors: Korde, Neha, Zhang, Yong, Loeliger, Kelsey, Poon, Andrea, Simakova, Olga, Zingone, Adriana, Costello, Rene, Childs, Richard, Noel, Pierre, Silver, Samuel, Kwok, Mary, Mo, Clifton, Young, Neal, Landgren, Ola, Sloand, Elaine, Maric, Irina
Format: Article
Language:English
Subjects:
Adult
Aged
anaemia
Bone Marrow - pathology
Dexamethasone - therapeutic use
Diagnosis, Differential
Female
Humans
Immunoglobulins - blood
Male
Middle Aged
monoclonal gammopathy of undetermined significance
Multiple Myeloma - diagnosis
Multiple Myeloma - pathology
myeloma
Paraproteinemias - diagnosis
Paraproteinemias - pathology
Plasma Cells - physiology
plasmacytosis
pure red cell aplasia
Red-Cell Aplasia, Pure - diagnosis
Red-Cell Aplasia, Pure - pathology
Reticulocyte Count
Reticulocytes - physiology
Thalidomide - analogs & derivatives
Thalidomide - therapeutic use
Young Adult
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Summary:Summary 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.
ISSN:0007-1048
1365-2141
DOI:10.1111/bjh.14012