Paroxysmale nächtliche Hämoglobinurie (PNH) Pathogenese, Diagnostik und Therapie
A. Röth, U. Dührsen, H. Schrezenmeier, J. Schubert
Literatur 1 Dacie JV, Lewis SM. Paroxysmal nocturnal haemoglobinuria: clinical manifestations, haematology, and nature of
the disease. Ser Haematol 1972; 5: 3–23
2 Fachinformation Soliris® 2007
Frickhofen N, Heimpel H, Kaltwasser JP, Schrezenmeier H. Antithymocyte globulin with or without cyclosporin A:
11-year follow-up of a randomized trial comparing treatments of aplastic anemia. Blood 2003; 101: 1236–42
3 Hall C, Richards S, Hillmen P. Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal
hemoglobinuria (PNH). Blood 2003; 102: 3587–91
4 Hegenbart U, Niederwieser D, Forman S et al. Hematopoietic cell transplantation from related and unrelated
donors after minimal conditioning as a curative treatment modality for severe paroxysmal nocturnal
hemoglobinuria. Biol Blood Marrow Transplant 2003; 9: 689–97
5 Hill A, Rother RP, Hillmen P. Improvement in the symptoms of smooth muscle dystonia during eculizumab
therapy in paroxysmal nocturnal hemoglobinuria. Haematologica 2005; 90: ECR40–
6 Hillmen P, Hall C, Marsh JC et al. Effect of eculizumab on hemolysis and transfusion requirements in patients
with paroxysmal nocturnal hemoglobinuria. N Engl J Med 2004; 350: 552–9
7 Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. 8 Hillmen P, Muus P, Dührsen U et al. Effect of the complement inhibitor eculizumab on thromboembolism in
patients with paroxysmal nocturnal hemoglobinuria. Blood 2007; 110: 4123–4128
9 Hillmen P, Young NS, Schubert J et al. The complement inhibitor eculizumab in paroxysmal nocturnal
hemoglobinuria. N Engl J Med 2006; 355: 1233–43
10 Hugel B, Socié G, Vu T et al. Elevated levels of circulating procoagulant microparticles in patients with
paroxysmal nocturnal hemoglobinuria and aplastic anemia. Blood 1999; 93: 3451–6
11 Issaragrisil S, Piankijagum A, Tang-naitrisorana Y. Corticosteroids therapy in paroxysmal nocturnal
hemoglobinuria. Am J Hematol 1987; 25: 77–83
12 Luzzatto L, Bessler M. The dual pathogenesis of paroxysmal nocturnal hemoglobinuria. Curr Opin Hematol 13 Parker C, Omine M, Richards S et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. 14 Richards SJ, Hill A, Hillmen P. Recent advances in the diagnosis, monitoring, and management of patients with
paroxysmal nocturnal hemoglobinuria. Cytometry B Clin Cytom 2007; 72: 291–8
15 Rosse WF. Paroxysmal nocturnal hemoglobinuria as a molecular disease. Medicine (Baltimore) 1997; 76: 63–93 16 Rosse WF, Hillmen P, Schreiber AD. Immune-mediated hemolytic anemia. Hematology (Am Soc Hematol Educ 17 Rother RP, Bell L, Hillmen P, Gladwin MT. The clinical sequelae of intravascular hemolysis and extracellular
plasma hemoglobin: a novel mechanism of human disease. JAMA 2005; 293: 1653–62
18 Saso R, Marsh J, Cevreska L et al. Bone marrow transplants for paroxysmal nocturnal haemoglobinuria. 19 Schubert J, Alvarado M, Uciechowski P et al. Diagnosis of paroxysmal nocturnal haemoglobinuria using
immunophenotyping of peripheral blood cells. Br J Haematol 1991; 79: 487–92
20 Strübing P. Paroxysmale Haemoglobinurie. Dtsch Med Wochenschr 1882; 8: 1–3 21 Strübing P. Paroxysmale Haemoglobinurie. Dtsch Med Wochenschr 1882; 8: 17–19
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