Monoclonal gammopathy-associated pure red cell aplasia

被引:19
作者
Korde, Neha [1 ,2 ]
Zhang, Yong [2 ]
Loeliger, Kelsey [3 ]
Poon, Andrea [3 ]
Simakova, Olga [4 ]
Zingone, Adriana [2 ]
Costello, Rene [2 ]
Childs, Richard [3 ]
Noel, Pierre [5 ]
Silver, Samuel [6 ]
Kwok, Mary [7 ]
Mo, Clifton [7 ]
Young, Neal [3 ]
Landgren, Ola [1 ,2 ]
Sloand, Elaine [3 ]
Maric, Irina [5 ]
机构
[1] Mem Sloan Kettering Canc Ctr, Myeloma Serv, 1275 York Ave, New York, NY 10065 USA
[2] NCI, Multiple Myeloma Sect, Metab Branch, Silver Spring, MD USA
[3] NHLBI, Hematol Branch, Silver Spring, MD 20892 USA
[4] US FDA, Off Blood Res & Review, Ctr Biol Evaluat & Res, Silver Spring, MD USA
[5] NIH, Dept Lab Med, Ctr Clin, Ann Arbor, MI USA
[6] Univ Michigan, Sch Med, Internal Med Hematol Oncol, Ann Arbor, MI USA
[7] Walter Reed Natl Mil Med Ctr, Murtha Canc Ctr, Bethesda, MD USA
关键词
anaemia; monoclonal gammopathy of undetermined significance; myeloma; plasmacytosis; pure red cell aplasia; MULTIPLE-MYELOMA; CYCLOSPORINE-A; ANEMIA; ERYTHROPOIETIN; INHIBITOR; HEPCIDIN; PATIENT; DISEASE;
D O I
10.1111/bjh.14012
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:876 / 883
页数:8
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