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All Journal Jurnal Kedokteran Syiah Kuala Acta Interna : The Journal of Internal Medicine Journal of the Medical Sciences (Berkala Ilmu Kedokteran) The Indonesian Biomedical Journal Smart Medical Journal
Ibnu Purwanto
Department Of Internal Medicine, Faculty Of Medicine, Universitas Gadjah Mada/ Dr. Sardjito Hospital, Yogyakarta
Biochemistry, Genetics & Molecular Biology Health Professions Immunology & microbiology Medicine & Pharmacology Neuroscience Public Health

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Journal : Smart Medical Journal

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Tinjauan Terkini Hemofilia A yang Didapat : Aspek Diagnosis dan Manajemen Ibnu Purwanto
Smart Medical Journal Vol 3, No 2 (2020): Smart Medical Journal
Publisher : Faculty of Medicine Universitas Sebelas Maret

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.13057/smj.v3i2.45870

Abstract

Hemofilia A yang didapat adalah penyakit yang jarang terdiagnosis dan seringkali salah terdiagnosis namun berpotensi menyebabkan perdarahan yang mengancam nyawa. Penyakit autoimun akibat pembentukan autoantibodi (inhibitor) terhadap FVIII ini hampir setengahnya memiliki gangguan lain yang mendasari. Pemanjangan activated partial thromboplastin time, mixing test yang tidak terkoreksi, rendahnya aktivitas FVIII, dan bukti inhibitor FVIII mendukung penegakan diagnosis Hemofilia A yang didapat. Rintangan dalam manajemen pasien dimulai dari penegakan diagnosis hingga penentuan terapi, baik terapi hemostatik, imunosupresi, serta pengobatan penyakit penyerta. Pemilihan terapi serta pengendalian terhadap efek samping dari pengobatan memerlukan perhatian khusus agar tercapai hemostasis dan remisi yang bertahan lama.Acquired Hemophilia A can potentially cause life-threatening conditions due to profuse bleeding, but this autoimmune disease is mostly underdiagnosed. Hemophilia A occurs due to the development of an antibody against FVIII, moreover up to half of these cases have underlying conditions. Prolonged activated partial thromboplastin time, uncorrected mixing test, low FVIII activity, and detection of FVIII inhibitors support the diagnosis of acquired Hemophilia A. However, several challenges lay within patients’ management strategy, such as diagnosis workup and therapeutical choices. Treatment for acquired hemophilia A encompasses hemostatic therapy, immunosuppression, and treatment of underlying disease. Moreover, therapeutical choice and side effects control require special consideration to achieve hemostasis and durable remission.
Acquired Hemophilia A induced by Clopidogrel : A Case Report Chofi Qolbi NA; Ibnu Purwanto; Vita Yanti Anggraeni
Smart Medical Journal Vol 4, No 2 (2021): Smart Medical Journal
Publisher : Faculty of Medicine Universitas Sebelas Maret

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.13057/smj.v4i2.45329

Abstract

ABSTRACT                                                   ABSTRACTIntroduction: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by intervention of factor VIII by autoantibody. Most AHA cases arise from underlying medical conditions such as autoimmune disorders, cancers, drug/allergic reaction, with 50% cases are idiopathic.Case Presentation: 60-year old Asian male complained weakness worsened in the last two days accompanied by fatigue and pallor. Patients has history of percutaneous coronary intervention in proximal and distal of left anterior descending artery. Patient’s aPTT mixed with normal plasma before incubation is 41 seconds and after incubation became 59 seconds, it can be concluded that patient has time dependent FVIII antibody and his diagnosis became Acquired Hemophilia A. Methylprednisolone 0,5 mg/kg/day was added to his treatment following his diagnosisDiscussion and Conclusions : Drug induced AHA can be caused by several medications, including antibiotics (penicillin, sulfonamides, chloramphenicol), anticonvulsants (phenytoin), methyldopa, interferon-α, clopidogrel, fludarabine . In this case, patient has a history of percutaneus intervention with routine consumption of clopidogrel and aspirin for 3 months. Clopidogrel therapy for 3 months is suspected as a cause of AHA emergence in this patient. Short-term, high-dose immunosuppressive treatment with oral prednisone (1 mg/kg for 10 days, 0.5 mg/kg for 20 days, and 0.25 mg/kg for 15 days) can reduce the incidence of angiographic restenosis with minor secondary effects such as gastric pain, water and salt retention, and worsened hypertension in nearly 10% patients.Keywords: Acquired, Hemophilia, Clopidogrel, Coronary, Artery, Disesase ABSTRAKPendahuluan : Acquired hemophilia A (AHA) adalah kelainan perdarahan langka yang disebabkan oleh intervensi faktor VIII oleh autoantibodi. Sebagian besar kasus AHA timbul dari kondisi medis yang mendasari seperti gangguan autoimun, kanker, reaksi alergi / obat, dengan 50% kasus bersifat idiopatik.Persentasi Kasus : Laki-laki berusia 60 tahun mengeluh lemas memberat dalam dua hari terakhir disertai kelelahan dan pucat. Pasien memiliki riwayat percutaneuos coronary intervention di bagian proksimal dan distal arteri desenden anterior kiri. APTT pasien mixed dengan plasma normal sebelum inkubasi adalah 41 detik dan setelah inkubasi menjadi 59 detik, dapat disimpulkan bahwa pasien memiliki antibodi FVIII yang tergantung waktu dan diagnosisnya menjadi Acquired Hemophilia A. Methylprednisolone 0,5 mg / kg / hari ditambahkan pada pengobatan setelah diagnosis AHA tegak.Diskusi dan Kesimpulan : AHA yang diinduksi obat dapat disebabkan oleh beberapa obat, termasuk antibiotik (penisilin, sulfonamida, kloramfenikol), antikonvulsan (fenitoin), metildopa, interferon-α, clopidogrel, fludarabine. Dalam kasus ini, pasien memiliki riwayat percutaneous coronary intervention dengan konsumsi rutin clopidogrel dan aspirin selama 3 bulan. Terapi klopidogrel selama 3 bulan diduga sebagai penyebab munculnya AHA pada pasien tersebut. Pengobatan imunosupresif dosis tinggi jangka pendek dengan prednison oral (1 mg / kg selama 10 hari, 0,5 mg / kg selama 20 hari, dan 0,25 mg / kg selama 15 hari) dapat mengurangi kejadian restenosis angiografik dengan efek sekunder minor seperti sebagai nyeri lambung, retensi air dan garam, dan hipertensi yang memburuk pada hampir 10% pasien. Kata Kunci : Hemofilia yang di Dapat, Clopidogrel, Penyakit Jantung Koroner
Co-Authors A. Harijadi Agus Barmawi Ahmad Ghozali Ahmad Ghozali Chofi Qolbi NA Diah Ari Safitri Didik Setyo Heiyanto Eiji Tatsumi Hiroyuki Sakoda Irianiwati Widodo Iwan Dwiprahasto Iwan Dwiprahasto Johan Kurnianda Kaho Furuta Kartika W. Taroeno-Hariadi Kartika Widayati Taroeno Hariadi Katsuyasu Saigo Kayoko Masuda Ken-Ichi Nagai Mardiah Suci Hardianti Mardiah Suci Hardiyanti Maryatun Hasan Meilani Syampurnawati Merari Panti Astuti NEVIATY PUTRI ZAMANI Puntodewo Puntodewo Sagiri Mangunsudirjo Seiji Kawano Setsuki Isono Shin-Ichi Kondo Shunichi Kumagai Sofia M. Haryana Sofia Mubarika Haryana Sukendro Sendjaja Sunggoro, Agus Jati Takayuki Takahashi Teguh Aryandono Trisnawati, Lidwina Tika Vita Yanti Anggraeni Wiwiek Probowati Yoshitaka Hayashi