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

被引：3
|
作者：
Climent, Fina [1 ]
Cid, Joan [2 ]
Sureda, Anna [3 ]
机构：
[1] Hosp Univ Bellvitge, Dept Pathol, IDIBELL, Lhospitalet De Llobregat 08907, Barcelona, Spain
[2] Univ Barcelona, Hosp Clin, Dept Hemotherapy & Hemostasis, Apheresis & Cellular Therapy Unit,ICMHO,IDIBAPS, Barcelona 08036, Spain
[3] Univ Barcelona, Inst Catala Oncol, Dept Hematol, IDIBELL, Lhospitalet De Llobregat 08907, Barcelona, Spain
来源：
HEMATO | 2022年 / 3卷 / 01期
关键词：
autoimmune hemolytic anemia; cold agglutinin; B-cell lymphoproliferative disorder; AUTOIMMUNE HEMOLYTIC-ANEMIA; PLASMA-EXCHANGE; RITUXIMAB; DIAGNOSIS; LYMPHOMA; THERAPY; FUTURE;
D O I：
10.3390/hemato3010014
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
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-Kappa B 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.
引用
收藏
页码：163 / 173
页数：11
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