Improvement in the symptoms of smooth muscle dystonia during eculizumab therapy in paroxysmal nocturnal hemoglobinuria.
نویسندگان
چکیده
Aberrant smooth muscle dystonia during hemolytic episodes in paroxysmal nocturnal hemoglobinuria (PNH) is implicated in the symptoms of abdominal pain, dysphagia and erectile dysfunction. Here we report two PNH patients treated with the complement inhibitor, eculizumab. Complement inhibition has been sustained for over 2 years and results in resolution of intravascular hemolysis and amelioration of symptoms associated with smooth muscle contractions.
منابع مشابه
Fatigue Improvement after Eculizumab in Paroxysmal Nocturnal Hemoglobinuria: Apropos of a Case
A 54-year-old male presented with shortness of breath and was diagnosed with pulmonary emboli. Laboratory studies revealed hemolytic anemia. Flow cytometry showed a prominent white blood cell clone without CD59 in 85% (NV 0-3.0) and red blood cells with 8.5% CD59 absence (NV 0-3.0) confirming a diagnosis of paroxysmal nocturnal hemoglobinuria. Eculizumab was started to decrease the possibility ...
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Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant clonal disorder resulting from somatic mutation in the PIG-A gene leading to a deficiency of the membrane-anchoring molecule glycosylphosphatidylinositol. The lack of expression of two glycosylphosphatidylinositol-anchored proteins involved in the regulation of the complement system renders PNH erythrocytes susceptible to complement-me...
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متن کاملParoxysmal nocturnal hemoglobinuria: a single Spanish center’s experience over the last 40 yr
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disease. To date, many reviews and series have been described. We report the experience of our center by presenting a review of 56 PNH patient cases with an average age at diagnosis of 38 yr and follow-ups beginning at approximately 40 yr; the median survival rate was 11 yr. The average clonal size upon diagnosis was 48%, presenting a v...
متن کاملIncreased eculizumab requirements during pregnancy in a patient with paroxysmal nocturnal hemoglobinuria: case report and review of the literature
Paroxysmal nocturnal hemoglobinuria (PNH) results from reduced complement regulatory proteins on hematopoietic cells, predisposing patients to intravascular hemolysis, thrombophilia, and cytopenias. Women diagnosed in pregnancy can experience significant maternofetal complications. Trials of eculizumab in PNH excluded pregnant women. Here, we report the first Canadian patient taken through preg...
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ورودعنوان ژورنال:
- Haematologica
دوره 90 12 Suppl شماره
صفحات -
تاریخ انتشار 2005