Hongbing Ma1, Hong Chang. 1. Department of Hematology, West China Hospital, Sichuan University, Chengdu 610041, China.
Abstract
RATIONALE: Acquired hemophilia A (AHA) is a rare bleeding disorder with prolonged activated partial thromboplastin time (aPTT). Severe hemorrhage may occur, especially in refractory AHA. PATIENT CONCERNS: We reported a 63-year-old man who suffered from life-threatening bleeding after the onset in lower limbs. DIAGNOSES: The patient was diagnosed as AHA which was related to pemphigoid. INTERVENTIONS: The patient had no response to the first-line treatment with corticosteroid and cyclophosphamide. Meanwhile, fatal hemorrhage occurred successively in thoracic cavity and right frontal lobe. rFVIIa and rituximab were administered. OUTCOMES: The patient survived from the life-threatening hemorrhage with a normal aPTT. His aPTT and FVIII:C level was normal during the follow-up of 6 months. LESSONS: Rituximab and rFVIIa can play a critical role in rescuing AHA that is refractory to the first-line treatment.
RATIONALE: Acquired hemophilia A (AHA) is a rare bleeding disorder with prolonged activated partial thromboplastin time (aPTT). Severe hemorrhage may occur, especially in refractory AHA. PATIENT CONCERNS: We reported a 63-year-old man who suffered from life-threatening bleeding after the onset in lower limbs. DIAGNOSES: The patient was diagnosed as AHA which was related to pemphigoid. INTERVENTIONS: The patient had no response to the first-line treatment with corticosteroid and cyclophosphamide. Meanwhile, fatal hemorrhage occurred successively in thoracic cavity and right frontal lobe. rFVIIa and rituximab were administered. OUTCOMES: The patient survived from the life-threatening hemorrhage with a normal aPTT. His aPTT and FVIII:C level was normal during the follow-up of 6 months. LESSONS: Rituximab and rFVIIa can play a critical role in rescuing AHA that is refractory to the first-line treatment.
Authors: Peter W Collins; Sybil Hirsch; Trevor P Baglin; Gerard Dolan; John Hanley; Michael Makris; David M Keeling; Ri Liesner; Simon A Brown; Charles R M Hay Journal: Blood Date: 2006-10-17 Impact factor: 22.113
Authors: Angela Huth-Kühne; Francesco Baudo; Peter Collins; Jørgen Ingerslev; Craig M Kessler; Hervé Lévesque; Maria Eva Mingot Castellano; Midori Shima; Jean St-Louis Journal: Haematologica Date: 2009-04 Impact factor: 9.941