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Volume 3
Issue 1
10.3390/hemato3010014
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Review

Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow

by
Fina Climent
1,*,
Joan Cid
2 and
Anna Sureda
3
1
Department of Pathology, Hospital Universitari de Bellvitge, IDIBELL, L’Hospitalet de Llobregat, 08907 Barcelona, Spain
2
Apheresis & Cellular Therapy Unit, Department of Hemotherapy and Hemostasis, ICMHO, IDIBAPS, Hospital Clínic, University of Barcelona, 08036 Barcelona, Spain
3
Department of Hematology, Institut Català d’Oncologia, IDIBELL, L’Hospitalet de Llobregat, Universitat de Barcelona, 08907 Barcelona, Spain
*
Author to whom correspondence should be addressed.
Hemato 2022, 3(1), 163-173; https://doi.org/10.3390/hemato3010014
Submission received: 23 January 2022 / Revised: 5 February 2022 / Accepted: 10 February 2022 / Published: 13 February 2022
(This article belongs to the Special Issue Classification of Lymphomas and Hematological Neoplasia in the Era of Genomic Research: A Themed Issue in Honor of Dr. Elaine S. Jaffe)
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Abstract

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.
Keywords: autoimmune hemolytic anemia; cold agglutinin; B-cell lymphoproliferative disorder autoimmune hemolytic anemia; cold agglutinin; B-cell lymphoproliferative disorder

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MDPI and ACS Style

Climent, F.; Cid, J.; Sureda, A. Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow. Hemato 2022, 3, 163-173. https://doi.org/10.3390/hemato3010014

AMA Style

Climent F, Cid J, Sureda A. Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow. Hemato. 2022; 3(1):163-173. https://doi.org/10.3390/hemato3010014

Chicago/Turabian Style

Climent, Fina, Joan Cid, and Anna Sureda. 2022. "Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow" Hemato 3, no. 1: 163-173. https://doi.org/10.3390/hemato3010014

APA Style

Climent, F., Cid, J., & Sureda, A. (2022). Cold Agglutinin Disease: A Distinct Clonal B-Cell Lymphoproliferative Disorder of the Bone Marrow. Hemato, 3(1), 163-173. https://doi.org/10.3390/hemato3010014

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