Pediatric Cold Agglutinin Disease Follow-up

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

Further Inpatient Care

Hospitalization is indicated in patients with severe cold agglutinin disease.

Next

Further Outpatient Care

Weekly until stable

  • Clinical examination
  • CBC count
  • Reticulocyte count
  • Urinalysis

Monthly until resolution

  • Clinical examination
  • CBC count
  • Reticulocyte count
  • Urinalysis
  • Direct antiglobulin test
  • Cold agglutinin titer
Previous
Next

Transfer

Transfer patients with severe anemia to a facility where pediatric hematology/oncology, blood bank, and pediatric intensive care services are available.

Previous
Next

Deterrence/Prevention

Currently, prevention is not possible.

Previous
Next

Complications

Complications are rare. Severe hemolysis and anemia can result in congestive heart failure or shock.

Previous
Next

Prognosis

Secondary postinfectious cold agglutinin disease is a transient self-limiting condition.

Secondary cold agglutinin disease associated with lymphoid neoplasms is chronic, and the prognosis depends on the underlying disorder. Treatment of the underlying lymphoid neoplasm may control the hemolysis. Lymphoid neoplasms can result in significant complications and death.

Primary cold agglutinin disease is a chronic indolent disease, with remissions and exacerbations of hemolysis. B-cell neoplasms may eventually develop.

Previous
Next

Patient Education

Teach patients to observe for signs of anemia, such as dyspnea, palpitations, and pallor, and to observe for signs of hemolysis, such as jaundice and dark urine.

Instruct patients to avoid exposure to cold and strenuous exercise.

For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center. Also, see eMedicine's patient education article Anemia.

Previous
 
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

  1. Khan FY, A yassin M. Mycoplasma pneumoniae associated with severe autoimmune hemolytic anemia: case report and literature review. Braz J Infect Dis. Feb 2009;13(1):77-9. [Medline].

  2. Roy-Burman A, Glader BE. Resolution of severe Donath-Landsteiner autoimmune hemolytic anemia temporally associated with institution of plasmapheresis. Crit Care Med. Apr 2002;30(4):931-4. [Medline].

  3. Giulino LB, Bussel JB, Neufeld EJ. Treatment with rituximab in benign and malignant hematologic disorders in children. J Pediatr. Apr 2007;150(4):338-44, 344.e1. [Medline].

  4. Berentsen S, Randen U, Vågan AM, Hjorth-Hansen H, Vik A, Dalgaard J, et al. High response rate and durable remissions following fludarabine and rituximab combination therapy for chronic cold agglutinin disease. Blood. Oct 28 2010;116(17):3180-4. [Medline].

  5. [Best Evidence] [Guideline] Berentsen S. How I manage cold agglutinin disease. Br J Haematol. May 2011;153(3):309-17. [Medline].

  6. Berentsen S, Ulvestad E, Gjertsen BT, et al. Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients. Blood. Apr 15 2004;103(8):2925-8. [Medline]. [Full Text].

  7. Camou F, Viallard JF, Pellegrin JL. [Rituximab in cold agglutinin disease]. Rev Med Interne. Aug 2003;24(8):501-4. [Medline].

  8. Cushing M, Degtyaryova D, Lomas-Francis C. Transfusion medicine illustrated. The role of plasmapheresis in the multimodal treatment of anti-Pr cold agglutinin disease. Transfusion. Oct 2010;50(10):2100-1. [Medline].

  9. Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. Am J Hematol. Apr 2002;69(4):258-71. [Medline].

  10. Gertz MA. Cold agglutinin disease and cryoglobulinemia. Clin Lymphoma. Mar 2005;5(4):290-3. [Medline].

  11. Hadnagy C. Agewise distribution of idiopathic cold agglutinin disease. Z Gerontol. May-Jun 1993;26(3):199-201. [Medline].

  12. Hamblin T. Management of cold agglutination syndrome. Transfus Sci. Feb-Apr 2000;22(1-2):121-4. [Medline].

  13. Inaba H, Geiger TL, Lasater OE, Wang WC. A case of hemoglobin SC disease with cold agglutinin-induced hemolysis. Am J Hematol. Jan 2005;78(1):37-40. [Medline].

  14. McNicholl FP. Clinical syndromes associated with cold agglutinins. Transfus Sci. Feb-Apr 2000;22(1-2):125-33. [Medline].

  15. Nanan R, Scheurlen W, Gerlich M, Huppertz HI. Severe low-titer cold-hemagglutinin disease responsive to steroid pulse therapy. Ann Hematol. Aug 1995;71(2):101-2. [Medline].

  16. Ness PM, Bell WR, Shirey RS. Transfusion medicine illustrated. Novel management of cold agglutinin disease. Transfusion. Jul 2003;43(7):839. [Medline].

  17. Ng PC, Lee KK, Lo AF, Li CK, Fok TF. Anti B cell targeted immunotherapy for treatment of refractory autoimmune haemolytic anaemia in a young infant. Arch Dis Child. Apr 2003;88(4):337-9. [Medline].

  18. Nydegger UE, Kazatchkine MD, Miescher PA. Immunopathologic and clinical features of hemolytic anemia due to cold agglutinins. Semin Hematol. Jan 1991;28(1):66-77. [Medline].

  19. Potter KN. Molecular characterization of cold agglutinins. Transfus Sci. Feb-Apr 2000;22(1-2):113-9. [Medline].

  20. Rituxan Product Information Sheet. FDA: FDA; 2002. [Full Text].

  21. Rosse WF, Hillmen P, Schreiber AD. Immune-mediated hemolytic anemia. Hematology (Am Soc Hematol Educ Program). 2004;48-62. [Medline].

  22. Stone MJ. Heating up cold agglutinins. Blood. Oct 28 2010;116(17):3119-20. [Medline].

  23. Teachey DT, Felix CA. Development of cold agglutinin autoimmune hemolytic anemia during treatment for pediatric acute lymphoblastic leukemia. J Pediatr Hematol Oncol. Jul 2005;27(7):397-9. [Medline].

  24. Terada K, Tanaka H, Mori R, et al. Hemolytic anemia associated with cold agglutinin during chickenpox and a review of the literature. J Pediatr Hematol Oncol. Mar-Apr 1998;20(2):149-51. [Medline].

  25. Vassou A, Alymara V, Chaidos A, Bourantas KL. Beneficial effect of rituximab in combination with oral cyclophosphamide in primary chronic cold agglutinin disease. Int J Hematol. Jun 2005;81(5):421-3. [Medline].

  26. Zecca M, Nobili B, Ramenghi U, Perrotta S, Amendola G, Rosito P, et al. Rituximab for the treatment of refractory autoimmune hemolytic anemia in children. Blood. May 15 2003;101(10):3857-61. [Medline].

 
Previous
Next
 
Blood smear showing spherocytic and agglutinated RBCs.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.