Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow
Cold agglutinin disease (CAD) is a distinct clinicopathologic entity characterized by clonal B-cell lymphoproliferative disorder in the bone marrow. B-cell gene mutations affect NF-ΚB as well as chromatin modification and remodeling pathways. Clonal immunoglobulins produced by B cells bind to red ce...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-02-01
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| Series: | Hemato |
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| Online Access: | https://www.mdpi.com/2673-6357/3/1/14 |
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| author | Fina Climent Joan Cid Anna Sureda |
| author_facet | Fina Climent Joan Cid Anna Sureda |
| author_sort | Fina Climent |
| collection | DOAJ |
| description | Cold agglutinin disease (CAD) is a distinct clinicopathologic entity characterized by clonal B-cell lymphoproliferative disorder in the bone marrow. B-cell gene mutations affect NF-ΚB as well as chromatin modification and remodeling pathways. Clonal immunoglobulins produced by B cells bind to red cells (RBCs) at cold temperatures causing RBC aggregation, complement cascade activation and cold-autoantibody autoimmune hemolytic anemia (cAIHA). The clinical picture shows cold-induced symptoms and cAIHA. Therapeutic options include “wait and watch”, rituximab-based regimens, and complement-directed therapies. Steroids must not be used for treating CAD. New targeted therapies are possibly identified after recent molecular studies. |
| first_indexed | 2024-03-09T19:45:52Z |
| format | Article |
| id | doaj.art-f4b8091dca8f42a791b5f5256d8e7fe6 |
| institution | Directory Open Access Journal |
| issn | 2673-6357 |
| language | English |
| last_indexed | 2024-03-09T19:45:52Z |
| publishDate | 2022-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Hemato |
| spelling | doaj.art-f4b8091dca8f42a791b5f5256d8e7fe62023-11-24T01:24:18ZengMDPI AGHemato2673-63572022-02-013116317310.3390/hemato3010014Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone MarrowFina Climent0Joan Cid1Anna Sureda2Department of Pathology, Hospital Universitari de Bellvitge, IDIBELL, L’Hospitalet de Llobregat, 08907 Barcelona, SpainApheresis & Cellular Therapy Unit, Department of Hemotherapy and Hemostasis, ICMHO, IDIBAPS, Hospital Clínic, University of Barcelona, 08036 Barcelona, SpainDepartment of Hematology, Institut Català d’Oncologia, IDIBELL, L’Hospitalet de Llobregat, Universitat de Barcelona, 08907 Barcelona, SpainCold agglutinin disease (CAD) is a distinct clinicopathologic entity characterized by clonal B-cell lymphoproliferative disorder in the bone marrow. B-cell gene mutations affect NF-ΚB as well as chromatin modification and remodeling pathways. Clonal immunoglobulins produced by B cells bind to red cells (RBCs) at cold temperatures causing RBC aggregation, complement cascade activation and cold-autoantibody autoimmune hemolytic anemia (cAIHA). The clinical picture shows cold-induced symptoms and cAIHA. Therapeutic options include “wait and watch”, rituximab-based regimens, and complement-directed therapies. Steroids must not be used for treating CAD. New targeted therapies are possibly identified after recent molecular studies.https://www.mdpi.com/2673-6357/3/1/14autoimmune hemolytic anemiacold agglutininB-cell lymphoproliferative disorder |
| spellingShingle | Fina Climent Joan Cid Anna Sureda Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow Hemato autoimmune hemolytic anemia cold agglutinin B-cell lymphoproliferative disorder |
| title | Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow |
| title_full | Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow |
| title_fullStr | Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow |
| title_full_unstemmed | Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow |
| title_short | Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow |
| title_sort | cold agglutinin disease a distinct clonal b cell lymphoproliferative disorder of the bone marrow |
| topic | autoimmune hemolytic anemia cold agglutinin B-cell lymphoproliferative disorder |
| url | https://www.mdpi.com/2673-6357/3/1/14 |
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