Paroxysmal Cold Hemoglobinuria Follow-up

Updated: Apr 01, 2015
  • Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Follow-up

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.

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Further Inpatient Care

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  • 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).
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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.

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Complications

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  • 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.
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Prognosis

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  • 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.
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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.

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