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 Blood, 15 October 2008, Vol. 112, No. 8, pp. 3099-3106.
Prepublished online as a Blood First Edition Paper on June 5, 2008; DOI 10.1182/blood-2008-01-133918.

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CLINICAL TRIALS AND OBSERVATIONS

Paroxysmal nocturnal hemoglobinuria: natural history of disease subcategories

Régis Peffault de Latour1, Jean Yves Mary2, Célia Salanoubat3, Louis Terriou4, Gabriel Etienne5, Mohamad Mohty6, Sophie Roth7, Sophie de Guibert8, Sebastien Maury9, Jean Yves Cahn10, Gerard Socié1, on behalf of the French Society of Hematology and of the French Association of Young Hematologists

1 Service d'Hématologie-Greffe and 2 Unité Inserm 717, Université Paris 7, Departement de Biostatistique et Informatique Médicale (DBIM), Hôpital Saint Louis, Paris; 3 Service d'Hématologie Clinique, Hôpital Hôtel Dieu, Paris; 4 Service des Maladies du Sang, Hôpital Claude Huriez, Lille; 5 Service de Médecine Interne, Groupe Hospitalier Sud, Pessac; 6 Unité de Transplantation et de Thérapie Cellulaire, Institut Paoli Calmettes, Marseille; 7 Service de Médecine Interne, Hôpital Archet, Nice; 8 Service d'Hématologie Clinique, Centre hospitalier Pontchaillou, Rennes; 9 Service d'Hématologie Clinique, Hôpital Henri-Mondor, Créteil; and 10 Clinique Universitaire d'Hematologie, Hôpital Michallon, Grenoble, France

The natural history of paroxysmal nocturnal hemoglobinuria (PNH) clinical subcategories (classic PNH and aplastic anemia [AA]/PNH syndrome) is still unknown. We retrospectively studied 460 PNH patients diagnosed in 58 French hematologic centers from 1950 to 2005. The median (SE) follow-up time was 6.8 (0.5) years. The median survival time (SE) was 22 (2.5) years. We identified 113 patients with classic PNH, 224 patients with AA-PNH syndrome, and 93 (22%) intermediate patients who did not fit within these 2 categories. At presentation, classic PNH patients were older, with more frequent abdominal pain and displayed higher levels of GPI-AP–deficient granulocytes. A time-dependent improved survival was observed. In classic PNH, diagnoses before 1986 (hazard ratio [HR]: 3.6; P = .01) and increasing age (P < .001) were associated with worse survival prognoses, whereas use of androgens within the first year after diagnosis was protective (HR, 0.17; P = .01). Transfusions before 1996 (HR, 2.7; P = .007) led to lower survival rates in patients with AA-PNH syndrome, whereas immunosuppressive treatment was associated with better outcomes (HR, 0.33; P = .03). Evolution to thrombosis affected survival in both subcategories (classic PNH: HR, 7.8 [P < .001]; AA-PNH syndrome: HR, 33.0 [P < .001]). Evolution to bicytopenia or pancytopenia for classic PNH (HR, 7.3, P < .001) and malignancies for AA-PNH syndrome (HR, 48.8; P < .001) were associated with worse outcomes. Although clinical presenta-tion and prognosis factors are different, classic PNH and AA-PNH syndrome present roughly similar outcomes, affected mainly by complications.


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