A single-center clinical study of 22 patients with acquired hemophilia.

Chunchen Yang; Ziqiang YU; Wei Zhang; Lijuan Cao; Jian SU; Pingping Sha; Xia Bai; Zhaoyue Wang; Changgeng Ruan

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A single-center clinical study of 22 patients with acquired hemophilia.

Author: Chunchen YANG ¹ ; Ziqiang YU ¹ ; Wei ZHANG ¹ ; Lijuan CAO ¹ ; Jian SU ¹ ; Pingping SHA ¹ ; Xia BAI ¹ ; Zhaoyue WANG ¹ ; Changgeng RUAN ¹
Author Information

1. Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Jiangsu Institute of Hematology, the First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Soochow University, Suzhou 215006, China.
Publication Type:Journal Article
MeSH: Adolescent; Adult; Aged; Aged, 80 and over; Antibodies, Monoclonal, Murine-Derived; Child; Child, Preschool; Female; Hematoma; Hemophilia A; Hemorrhage; Hemostatics; Humans; Immunosuppressive Agents; Male; Middle Aged; Retrospective Studies; Rituximab; Young Adult
From: Chinese Journal of Hematology 2015;36(2):107-111
CountryChina
Language:Chinese
Abstract:
OBJECTIVETo investigate the feature of underlying disorders, clinical symptoms, diagnosis and treatment strategies of patients with acquired hemophilia(AH).
METHODSThe clinical data and laboratory tests results of 22 patients with AH from March 2010 to June 2014 were retrospectively analyzed.
RESULTSA total of 22 patients with AH were enrolled in our study, including 20 patients diagnosed as acquired hemophilia A (AHA) and 2 as acquired hemophilia B (AHB). Among the AHA patients, there were 10 males and 10 females with the median age of 37.5 (range, 2-95) years old. The median activity of FVIII (FVIII:C) of the 20 AHA patients was 1.9% (0.5%-39.0%). Soft tissue hematoma (80.0%) and subcutaneous bleeding (75.0%) were the most common clinical symptoms. Two male children were diagnosed as AHB (age 1 and 3 years old, respectively) with mild bleeding symptoms, and the activities of FIX (FIX:C) were 5.0% and 16.0%, respectively. In addition, an underlying disorder was found in 7 patients (31.8%). In laboratory testing, all patients had prolonged APTT, normal PT, decreased FVIII:C or FIX:C, positive antibody screening test or antibody titer (2-32 BU), and negative for lupus anticoagulant and anticardiolipin antibody. Nineteen out of 20 patients were treated with blood products to stop acute bleeding episodes. Corticosteroid alone was applied to 7 patients, corticosteroid combined with other immunosuppressive agents to 11 patients, rituximab to 3 patients. Nineteen patients responded well to hemostatic treatment, except 1 patient who died of fatal bleeding. The FVIII:C of 8 patients increased to a normal level with the median time of 42.5(21-145) days. After treatment, the activity of FIX:C of the 2 AHB patients achieved 35% and 24% in 48 and 60 days, respectively.
CONCLUSIONAcquired hemophilia is not an uncommon disease in clinical practices, which can occur in people of all ages. AH is a bleeding disorder with heterogeneous characteristics. Compared with adult, the clinical symptoms of children patients were mild, which lead to underdiagnosis.