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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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| Published in: | British journal of haematology 2016-06, Vol.173 (6), p.876-883 |
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| Main Authors: | , , , , , , , , , , , , , , , |
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| Language: | English |
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| cites | cdi_FETCH-LOGICAL-c5842-5793730c6a257f85f753256c6666e44f98fca82b73cdc2ca9e5e122f271d73be3 |
| container_end_page | 883 |
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| container_title | British journal of haematology |
| container_volume | 173 |
| creator | 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 |
| description | 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. |
| doi_str_mv | 10.1111/bjh.14012 |
| format | article |
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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.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1111/bjh.14012</identifier><identifier>PMID: 26999424</identifier><language>eng</language><publisher>England</publisher><subject>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</subject><ispartof>British journal of haematology, 2016-06, Vol.173 (6), p.876-883</ispartof><rights>Published 2016. This article is a U.S. Government work and is in the public domain in the USA.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5842-5793730c6a257f85f753256c6666e44f98fca82b73cdc2ca9e5e122f271d73be3</citedby><cites>FETCH-LOGICAL-c5842-5793730c6a257f85f753256c6666e44f98fca82b73cdc2ca9e5e122f271d73be3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,780,784,885,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26999424$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Korde, Neha</creatorcontrib><creatorcontrib>Zhang, Yong</creatorcontrib><creatorcontrib>Loeliger, Kelsey</creatorcontrib><creatorcontrib>Poon, Andrea</creatorcontrib><creatorcontrib>Simakova, Olga</creatorcontrib><creatorcontrib>Zingone, Adriana</creatorcontrib><creatorcontrib>Costello, Rene</creatorcontrib><creatorcontrib>Childs, Richard</creatorcontrib><creatorcontrib>Noel, Pierre</creatorcontrib><creatorcontrib>Silver, Samuel</creatorcontrib><creatorcontrib>Kwok, Mary</creatorcontrib><creatorcontrib>Mo, Clifton</creatorcontrib><creatorcontrib>Young, Neal</creatorcontrib><creatorcontrib>Landgren, Ola</creatorcontrib><creatorcontrib>Sloand, Elaine</creatorcontrib><creatorcontrib>Maric, Irina</creatorcontrib><title>Monoclonal gammopathy‐associated pure red cell aplasia</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>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.</description><subject>Adult</subject><subject>Aged</subject><subject>anaemia</subject><subject>Bone Marrow - pathology</subject><subject>Dexamethasone - therapeutic use</subject><subject>Diagnosis, Differential</subject><subject>Female</subject><subject>Humans</subject><subject>Immunoglobulins - blood</subject><subject>Male</subject><subject>Middle Aged</subject><subject>monoclonal gammopathy of undetermined significance</subject><subject>Multiple Myeloma - diagnosis</subject><subject>Multiple Myeloma - pathology</subject><subject>myeloma</subject><subject>Paraproteinemias - diagnosis</subject><subject>Paraproteinemias - pathology</subject><subject>Plasma Cells - physiology</subject><subject>plasmacytosis</subject><subject>pure red cell aplasia</subject><subject>Red-Cell Aplasia, Pure - diagnosis</subject><subject>Red-Cell Aplasia, Pure - pathology</subject><subject>Reticulocyte Count</subject><subject>Reticulocytes - physiology</subject><subject>Thalidomide - analogs & derivatives</subject><subject>Thalidomide - therapeutic use</subject><subject>Young Adult</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><recordid>eNp1kE1OwzAQhS0EoqWw4AIoS1ik9W-cbJCgAgoCsYG15ThOm8qJg52AuuMInJGTkJJSwYLZzEjz6b2ZB8AxgmPU1SRdLsaIQoR3wBCRiIUYUbQLhhBCHiJI4wE48H4JISKQoX0wwFGSJBTTIYgfbGWVsZU0wVyWpa1ls1h9vn9I760qZKOzoG6dDlw3KG1MIGsjfSEPwV4ujddHmz4Cz9dXT9NZeP94czu9uA8ViykOGU8IJ1BFEjOexyznjGAWqagrTWmexLmSMU45UZnCSiaaaYRxjjnKOEk1GYHzXrdu01JnSleNk0bUriilWwkrC_F3UxULMbevgjGa8M59BE43As6-tNo3oiz8-hNZadt6gWIYR4gzBDv0rEeVs947nW9tEBTrpEWXtPhOumNPft-1JX-i7YBJD7wVRq_-VxKXd7Ne8gt2aYjy</recordid><startdate>201606</startdate><enddate>201606</enddate><creator>Korde, Neha</creator><creator>Zhang, Yong</creator><creator>Loeliger, Kelsey</creator><creator>Poon, Andrea</creator><creator>Simakova, Olga</creator><creator>Zingone, Adriana</creator><creator>Costello, Rene</creator><creator>Childs, Richard</creator><creator>Noel, Pierre</creator><creator>Silver, Samuel</creator><creator>Kwok, Mary</creator><creator>Mo, Clifton</creator><creator>Young, Neal</creator><creator>Landgren, Ola</creator><creator>Sloand, Elaine</creator><creator>Maric, Irina</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>5PM</scope></search><sort><creationdate>201606</creationdate><title>Monoclonal gammopathy‐associated pure red cell aplasia</title><author>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</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5842-5793730c6a257f85f753256c6666e44f98fca82b73cdc2ca9e5e122f271d73be3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>Adult</topic><topic>Aged</topic><topic>anaemia</topic><topic>Bone Marrow - pathology</topic><topic>Dexamethasone - therapeutic use</topic><topic>Diagnosis, Differential</topic><topic>Female</topic><topic>Humans</topic><topic>Immunoglobulins - blood</topic><topic>Male</topic><topic>Middle Aged</topic><topic>monoclonal gammopathy of undetermined significance</topic><topic>Multiple Myeloma - diagnosis</topic><topic>Multiple Myeloma - pathology</topic><topic>myeloma</topic><topic>Paraproteinemias - diagnosis</topic><topic>Paraproteinemias - pathology</topic><topic>Plasma Cells - physiology</topic><topic>plasmacytosis</topic><topic>pure red cell aplasia</topic><topic>Red-Cell Aplasia, Pure - diagnosis</topic><topic>Red-Cell Aplasia, Pure - pathology</topic><topic>Reticulocyte Count</topic><topic>Reticulocytes - physiology</topic><topic>Thalidomide - analogs & derivatives</topic><topic>Thalidomide - therapeutic use</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Korde, Neha</creatorcontrib><creatorcontrib>Zhang, Yong</creatorcontrib><creatorcontrib>Loeliger, Kelsey</creatorcontrib><creatorcontrib>Poon, Andrea</creatorcontrib><creatorcontrib>Simakova, Olga</creatorcontrib><creatorcontrib>Zingone, Adriana</creatorcontrib><creatorcontrib>Costello, Rene</creatorcontrib><creatorcontrib>Childs, Richard</creatorcontrib><creatorcontrib>Noel, Pierre</creatorcontrib><creatorcontrib>Silver, Samuel</creatorcontrib><creatorcontrib>Kwok, Mary</creatorcontrib><creatorcontrib>Mo, Clifton</creatorcontrib><creatorcontrib>Young, Neal</creatorcontrib><creatorcontrib>Landgren, Ola</creatorcontrib><creatorcontrib>Sloand, Elaine</creatorcontrib><creatorcontrib>Maric, Irina</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>British journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Korde, Neha</au><au>Zhang, Yong</au><au>Loeliger, Kelsey</au><au>Poon, Andrea</au><au>Simakova, Olga</au><au>Zingone, Adriana</au><au>Costello, Rene</au><au>Childs, Richard</au><au>Noel, Pierre</au><au>Silver, Samuel</au><au>Kwok, Mary</au><au>Mo, Clifton</au><au>Young, Neal</au><au>Landgren, Ola</au><au>Sloand, Elaine</au><au>Maric, Irina</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Monoclonal gammopathy‐associated pure red cell aplasia</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>2016-06</date><risdate>2016</risdate><volume>173</volume><issue>6</issue><spage>876</spage><epage>883</epage><pages>876-883</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><abstract>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.</abstract><cop>England</cop><pmid>26999424</pmid><doi>10.1111/bjh.14012</doi><tpages>8</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | British journal of haematology, 2016-06, Vol.173 (6), p.876-883 |
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| source | Wiley |
| 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 |
| title | Monoclonal gammopathy‐associated pure red cell aplasia |
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