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

Fina Climent,Joan Cid,Anna Sureda

doi:10.3390/hemato3010014

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.

Highlights

Apheresis & Cellular Therapy Unit, Department of Hemotherapy and Hemostasis, ICMHO, IDIBAPS, Department of Hematology, Institut Català d’Oncologia, IDIBELL, L’Hospitalet de Llobregat, Abstract: Cold agglutinin disease (CAD) is a distinct clinicopathologic entity characterized by clonal
Dressler is generally credited with being the first person to give a clear description of an autoimmune hemolytic anemia (AIHA), probably paroxysmal cold hemoglobinuria (PCH), in 1854 [1]
The authors confirmed that the direct antiglobulin test (DAT) tube test was the gold standard in diagnosing AIHA, they found that some negative results with the DAT tube test were identified with microcolumn test, reflecting the different accuracy of these laboratory tests to detect the low quantity of IgG bound on the red blood cells (RBC) membrane [37]

Summary

Introduction

Dressler is generally credited with being the first person to give a clear description of an autoimmune hemolytic anemia (AIHA), probably paroxysmal cold hemoglobinuria (PCH), in 1854 [1]. In the early years of the twentieth century, a distinction of congenital and acquired forms of hemolytic anemias was well defined, though the underlying mechanism was not well understood [2]. The direct antiglobulin test (DAT) distinguished between the familial and acquired forms of hemolytic anemia, and demonstrated a difference in their etiology. In 1951, Young et al were the first to coin the term AIHA [4] Those authors theorized that the production of an autoantibody leads to autoimmunization

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Conclusion