Pediatric Cold Agglutinin Disease Clinical Presentation

  • Author: James L Harper, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Aug 3, 2011
 

History

Transient cold agglutinin disease may appear abruptly with anemia and hemoglobinuria. Recent or current symptoms of infection may be present. Chronic cold agglutinin disease has a gradual onset.

Symptoms of anemia include the following:

  • Pallor
  • Fatigue
  • Dyspnea
  • Poor feeding
  • Anemia, which may be mild, moderate, or severe

Symptoms of hemolysis include the following:

  • Jaundice
  • Dark urine caused by hemoglobinuria

Acrocyanosis, a purplish discoloration of the fingers, toes, nose, and ears, is precipitated by exposure to cold. Acrocyanosis occurs mostly in the chronic form of the disease and is due to intra-arteriolar agglutination of RBCs in relatively cool areas of the body.

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Physical

Anemia causes pallor. Signs of congestive heart failure or shock are rare but may be present when anemia is severe.

Hemolysis causes jaundice, splenomegaly, and sometimes fever.

Acrocyanosis may be evident in the fingertips, feet, earlobes, and nose.

Fever, lymphadenopathy, or rash may reflect an underlying condition.

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Causes

Cold agglutinin disease may be primary (idiopathic) or secondary, caused by infection, systemic autoimmunity, or neoplasm. Primary (idiopathic) cold agglutinin disease is associated with monoclonal autoantibodies. Secondary cold agglutinin disease can be associated with either monoclonal or polyclonal autoantibodies.

Primary (idiopathic) cold agglutinin disease, monoclonal - Usually chronic, occurring in adults

Secondary cold agglutinin disease, monoclonal - Usually chronic, occurring in adults (Cases of cold agglutinin disease in children with B-cell lymphoproliferative diseases [eg, acute lymphoblastic leukemia] induced by an associated infection have been reported.)

  • B-cell neoplasms - Waldenström macroglobulinemia, lymphoma, chronic lymphoid leukemia, myeloma
  • Nonhematologic neoplasms

Secondary cold agglutinin disease, polyclonal - Usually acute, transient, and postinfectious, occurring in children and young adults

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Contributor Information and Disclosures
Author

James L Harper, MD  Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Gary R Jones, MD  Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Gary D Crouch, MD  Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

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Blood smear showing spherocytic and agglutinated RBCs.
 
 
 
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