Hemophilia A yang Didapat: Suatu Tinjauan tentang Diagnosis dan Manajemen

  • I Made Bakta (1) Divisi Hematologi dan Onkologi Medik (2) Bagian Penyakit Dalam Fakultas Kedokteran Universitas Udayana/ RS Sanglah, Denpasar, Bali

Abstract

Hemophilia A yang didapat atau acquired hemophilia A (AHA) adalah penyakit perdarahan yang didapat (acquired), suatu penyakit yang jarang,  penyakit autoimun yang ditandai oleh autoantibodi terhadap faktor VIII. Yang khas, penderita tidak menunjukkan riwayat perdarahan sebelumnya, datang dengan keluhan utama perdarahan spontan, terutama perdarahan kulit atau mukosa. AHA lebih banyak terjadi pada umur tua, baik pada laki maupun wanita dan dihubungkan dengan berbagai penyakit, seperti penyakit autoimun (lupus erithematosus sistemik, penyakit limfoproliferatif, artritis rematoid), keganasan atau kehamilan.  Sekitar setengah dari penderita bersifat idiopatik. AHA didiagnosis jika dijumpai penderita tanpa riwayat perdarahan sebelumnya, baik personal atau keluarga, dimana pemanjangan aPTT (activated plasma thromboplastin time) tidak dapat dikoreksi setelah pencampuran dengan sejumlah volume yang sama dari plasma penderita dan plasma normal yang diinkubasi selama 2 – 4 jam pada suhu 37oC. Kadar FVIII:C menurun.  Aktivitas titer inhibitor  FVIII:C yang spesifik dapat dideteksi dan diukur dengan Bethesda assay atau dengan modifikasinya Nijmegen assay. Dalam pengelolaan AHA, pengendalian terhadap perdarahan merupakan prioritas pertama. Pada penderita dengan kadar inhibitor yang rendah, desmopressin dapat digunakan. Tetapi FVIII by passing agents (activated prothrombin complex concentrate atau recombinant FVIIa) diperlukan pada penderita dengan kadar inhibitor yang tinggi atau penderita dengan episode perdarahan yang berat. Secara bersamaan dilakukan eradikasi terhadap inhibitor (autoantibodi) untuk mengurangi risiko perdarahan berikutnya. Dianjurkan terapi lini pertama dengan kortikosteroid atau terapi kombinasi kortikosteroid dengan siklofosfamid. Untuk terapi lini kedua dapat dipakai rituximab, jika terapi lini pertama gagal atau terdapat kontraindikasi.

Kata kunci: hemophilia yang didapat, diagnosis, manajemen.

 

(Acquired Hemophilia A: An Overview on Diagnosis and Management)

Acquired hemophilia A (AHA) is a rare acquired bleeding disorder, an autoimmune disease characterized by autoantibody directed against factor VIII. Typically, patients with no prior history ofbleeding disorder, present with spontaneous bleeding, especially skin and mucosal bleeding. AHA occurs more frequently in the elderly, both in male and female and may be associated  with several conditions, such as autoimmune diseases (systemic lupus erythematosus, lymphoproliferative disease, rheumatoid arthritis), malignancies and pregnancy.  Around half of the patients are idiopathic. AHA is diagnosed in patients without previous personal or family bleeding history in which prolonged activated partial thromboplastin time  is not corrected after mixing and incubating for 2 – 4 hours at 37oC equal volumes of patient and normal plasma.  FVIII:C levels are reduced  and a specific FVIII inhibiting activity is detected and measured by the Bethesda  assay or its Nijmegen modification. In the management of AHA, control of acute bleeding is the first priority. In patients with low level inhibitor, desmopressin may be used. But FVIII by passing agents (activated prothrombin complex concentrate and recombinant activated FVII) are required for patients with high titre of antibody or with serious bleeding episode. Immediate autoantibody eradication to reduce subsequent bleeding risk should be performed. It is recommended initial treatment with corticosteroid or combination therapy with corticosteroid and cyclophosphamide and suggest second-line  therapy with rituximab if first-line therapy is fails or is contraindicated.

Keywords: acquired hemophilia A, diagnosis, management.

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Published
2018-01-31
How to Cite
Bakta, I. M. (2018). Hemophilia A yang Didapat: Suatu Tinjauan tentang Diagnosis dan Manajemen. WMJ (Warmadewa Medical Journal), 2(2), 44-51. https://doi.org/10.22225/wmj.2.2.230.44-51
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