Zusammenfassung
Eine erworbene Hämophilie stellt eine seltene Komplikation bei autoimmunen Erkrankungen und Malignomen dar. Sie kann zu Blutungen in Haut und Muskel führen, wobei intrakranielle Blutungen bei Erwachsenen bislang nicht beschrieben sind. Wir berichten über eine Patientin mit akuter intrakranieller Blutung aufgrund einer erworbenen Hemmkörperhämophilie Faktor VIII betreffend. Trotz rascher Behandlung mit Faktor VIIa gefolgt von operativer Hämatomausräumung und immunmodulatorischer Therapie mit Steroiden und Cyclophosphamid verstarb die Patientin im Verlauf an einem Blutungsrezidiv.
Summary
Acquired hemophilia is a rare complication in autoimmune disorders and malignancies. It can result in bleedings into skin and muscle, whereas intracranial hemorrhage in adults has so far not been described. We report a patient with acute intracerebral hemorrhage due to acquired hemophilia with factor VIII inhibition. The patient was treated with recombinant factor VIIa and open hematoma evacuation followed by administration of cortisone and cyclophosphamide. After good initial recovery, intracerebral rebleeding occurred and the patient died from brainstem compression.
Literatur
Arkin S, Cooper HA, Hutter JJ et al. (1998) Activated recombinant human coagulation factor VII therapy for intracranial hemorrhage in patients with hemophilia A or B with inhibitors. Haemostasis 28:93–98
Boggio LN, Green D (2001) Acquired hemophilia. Rev Clin Exp Hematol 5:389–404
Haya S, Lorenzo JI, Dasi MA et al. (1998) Development of a factor VII inhibitor in a newborn haemophiliac. Haemophilia 4:755–756
Kueh YK, Koh DR, Chan TB et al. (1988) Acquired factor VIIIC deficiency due to circulating factor VIIIC inhibitors. Ann Acad Med Singapore 17:589–594
Nishino Y, Ueki K, Suto M et al. (2001) Successful treatment of patients with rheumatic disorders and acquired factor VIII inhibitors with cyclophosphamide and prednisolone combination therapy: two case reports. J Int Med Res 29:432–436
Papadaki HA, Xylouri I, Valatas W et al. (1998) Severe acquired hemophilia A successfully treated with activated recombinant human factor VII. Ann Hematol 77:123–125
Quinones-Hinojosa A, Gulati M, Singh V et al. (2003) Spontaneous intracerebral hemorrhage due to coagulation disorders. Neurosurg Focus 15:E3
Ries M, Wolfel D, Maier-Brandt B (1995) Severe intracranial hemorrhage in a newborn infant with transplacental transfer of an acquired factor VII:C inhibitor. J Pediatr 127:649–650
Soriano RM, Matthews JM, Guerado-Parra E (1987) Acquired haemophilia and rheumatoid arthritis. Br J Rheumatol 26:382–383
Trotta F, Bajocchi G, La Corte R et al. (1999) Long-lasting remission and successful treatment of acquired factor VIII inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus. Rheumatology 38:1007–1009
Von Depka M (2002) NovoSeven: mode of action and use in acquired haemophilia. Intensive Care Med 28:222–227
Von Depka Prondzinski M (2003) Acquired haemophilia due to autoantibodies against factor VIII. Hamostaseologie 23:28–35
Zeitler H, Ulrich-Merzenich G, Walger P et al. (2006) Das modifizierte Bonn-Malmö-Protokoll in der Behandlung der erworbenen Hemmkörperhämophilie. Dtsch Med Wochenschr 131:141–147
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Marquardt, L., Haubelt, H., Gass, S. et al. Intrakranielle Blutung bei erworbener Hemmkörperhämophilie. Nervenarzt 77, 1480–1482 (2006). https://doi.org/10.1007/s00115-006-2181-0
Issue Date:
DOI: https://doi.org/10.1007/s00115-006-2181-0