Paroxysmal Nocturnal Hemoglobinuria Follow-up

Updated: Jun 29, 2016
  • Author: Emmanuel C Besa, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Follow-up

Further Outpatient Care

In many patients with paroxysmal nocturnal hemoglobinuria (PNH), the abnormal clone may eventually disappear. This usually takes at least 5 years, and often as long as 15-20 years. Reactivation of PNH in these patients has been observed with acute infections. Patients with chronic anemia alone, without thrombotic complications, can live relatively normal lives for many years.

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Further Inpatient Care

In severe cases with of paroxysmal nocturnal hemoglobinuria (PNH) with an aplastic phase, referral to a bone marrow transplantation center is indicated for possible allogeneic bone marrow transplantation. Umbilical cord stem cell transplantation from the patient's own cord blood, from a related donor, or from the registry for HLA–matched unrelated donors may be an option in pediatric patients.

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Inpatient & Outpatient Medications

Outpatient RBC transfusion often is necessary to keep a patient with chronic anemia and paroxysmal nocturnal hemoglobinuria (PNH) able to function and live a normal life.

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Complications

Primary care physicians should be aware of the thrombotic complications of paroxysmal nocturnal hemoglobinuria (PNH) and how to diagnose them when they occur.

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Prognosis

The prognosis in patients with paroxysmal nocturnal hemoglobinuria (PNH) is variable, depending on the severity of symptoms and the presence of complications. An aplastic phase is a serious prognostic factor, because the resulting pancytopenia and thrombosis of hepatic, abdominal, and cerebral veins can have life-threatening consequences. Prophylactic anticoagulation has not been shown to be of benefit because of a lack of data from a clinical trial setting.

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