eMedicine Specialties > Hematology > Immune System and Disorders

Hemoglobinuria, Paroxysmal Cold: Follow-up

Author: Corinne Goldberg, MD, Fellow in Transfusion Medicine/Blood Banking, Transfusion Medicine Department, Hoxworth Blood Center, University of Cincinnati
Coauthor(s): Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Contributor Information and Disclosures

Updated: Nov 21, 2008

Follow-up

Further Inpatient Care

  • Hospitalization is required for patients with paroxysmal cold hemoglobinuria to monitor and treat complications that are associated with severe anemia secondary to massive hemolysis.
  • Maintain the patient's cardiorespiratory function and hydration status.
  • Daily laboratory evaluation of anemia should include CBC counts, LDH levels, and reticulocyte counts.
  • Monitor the hemoglobinuria with routine urine analysis.
  • Evaluate for secondary causes of paroxysmal cold hemoglobinuria (ie, viral and bacterial serologic and culture assays, imaging studies).

Further Outpatient Care

When the acute phase of paroxysmal cold hemoglobinuria is over, several follow-up visits for assessment of blood counts to ensure recovery may be all that is necessary, with instructions to patients to avoid cold exposure. Confirming that the D-L antibody test result is no longer positive may be valuable on subsequent regular checkups; however, note that low titers of the antibody may persist for several years after an acute episode. Appropriate treatment and follow-up care for syphilis or other infections are needed until the conditions are deemed cured or in remission.

Deterrence/Prevention

In patients with the chronic form of paroxysmal cold hemoglobinuria, avoiding exposure to cold is essential to prevent recurrent episodes of hemolysis. These patients should avoid activities that would increase their likelihood of being chilled, such as jogging outside in cold weather and handling of cold objects that can alter the body's peripheral thermal property.

Complications

  • Urticarial eruptions generally resolve spontaneously or with antihistamine therapy.
  • Severe anemia, particularly in older patients with atherosclerotic disease, could result in exacerbation or precipitation of ischemic symptoms.
  • Renal failure is possible if patients with paroxysmal cold hemoglobinuria are inadequately hydrated or have a predisposition to renal disease.
  • Mortality is rare in paroxysmal cold hemoglobinuria and is most commonly due to multiorgan failure from severe anemia secondary to massive acute hemolysis.

Prognosis

  • Acute episodes are generally transitory in nature and rarely recur. The prognosis in paroxysmal cold hemoglobinuria is excellent with supportive therapy. 
  • The chronic idiopathic forms may persist for years with variable morbidity.
  • Paroxysmal cold hemoglobinuria usually ameliorates if the underlying disease responds to specific therapy.

Patient Education

Educating patients about the need to avoid cold exposure is essential. Explain the role that chilling the body plays in the development of the acute hemolytic event in paroxysmal cold hemoglobinuria. Patients should also understand the need to take folate supplements to assist erythrocyte production.

Miscellaneous

Medicolegal Pitfalls

  • Failure to recognize the acute hemolytic event and/or correlate the event with paroxysmal cold hemoglobinuria
  • Failure to institute hydration and other measures needed to prevent renal failure or cardiopulmonary dysfunction in a patient with massive intravascular hemolysis
  • Failure to diagnose underlying or associated disease states

Special Concerns

  • Avoid biphasic D-L antibody activity by providing warmed RBC units.
  • Exacerbation of hemolysis can be avoided by monitoring the patient while slowly transfusing blood components.
  • Monitor the patient's hydration status, particularly in those with cardiac complications.
 
Acknowledgments

The authors and editors gratefully acknowledge the contributions of previous authors, Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems, and Harry L Messmore, Jr, MD, Professor, Department of Medicine, Division of Hematology/Oncology, Loyola University Stritch School of Medicine, to the development and writing of this article.



More on Hemoglobinuria, Paroxysmal Cold

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References
Further Reading
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Further Reading

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Keywords

paroxysmal cold hemoglobinuria, hemolytic anemia, myelodysplastic syndromes, Donath-Landsteiner hemolytic anemia, cold-induced immune hemolytic anemia, PCH, paroxysmal nocturnal hemoglobinuria, autoimmune hemolytic anemia, Marchiafava-Micheli syndrome, AIHA, intravascular hemolysis, anemia, blood disorder, blood disease, cold exposure, biphasic hemolysin, hematologic disorders, syphilis, measles, mumps, influenza, varicella-zoster virus, VZV, cytomegalovirus, CMV, Epstein-Barr virus, EBV, adenovirus, parvovirus B19, Coxsackie A9, , , , , ,

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Contributor Information and Disclosures

Author

Corinne Goldberg, MD, Fellow in Transfusion Medicine/Blood Banking, Transfusion Medicine Department, Hoxworth Blood Center, University of Cincinnati
Disclosure: Nothing to disclose.

Coauthor(s)

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Medical Editor

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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