Profil Pasien Anemia Hemolitik Auto Imun (AHAI) dan Respon Pengobatan Pasca Terapi Kortikosteroid di Rumah Sakit Umum Pusat Nasional dr. Cipto Mangunkusumo

Wulyo Rajabto, Djumhana Atmakusuma, Siti Setiati

Abstract

Pendahuluan. Anemia hemolitik auto imun (AHAI) merupakan salah satu penyakit imunologi yang menyebabkan hemolisis. Data mengenai karakteristik demografi dan respon pengobatan AHAI, khususnya pemberian kortikosteroid belum banyak didapatkan di Indonesia. Penelitian ini dilakukan untuk mengetahui profil pasien-pasien AHAI dan respon pengobatannya setelah mendapatkan kortikosteroid.

Metode. Desain studi potong lintang dilakukan menggunakan status rekam medik pasien AHAI yang berobat jalan di Poliklinik Hematologi-Onkologi Medik Departemen Ilmu Penyakit Dalam RSUPN Dr. Cipto Mangunkusumo selama 5 tahun (2004- 2008). Variabel yang diteliti meliputi karakteristik demografi, klasifikasi AHAI dan etiologinya, serta respon pengobatannya setelah mendapatkan kortikosteroid dosis inisial setara prednison 1-1,5 mg/kgbb/hari selama 3-4 minggu, respon positif ditandai oleh meningkatkan kadar Hb ≥10 g/dL.

Hasil. Dari total 50 subjek, didapatkan 92% AHAI tipe hangat, 6% tipe dingin dan 2% campuran. Etiologi AHAI tipe hangat adalah idiopatik atau primer (54,3%), sedangkan etiologi tipe sekunder adalah lupus eritematosus sistemik/LES (41,3%), hepatitis autoimun (2,2%) dan leukemia limfositik kronik (2,2%). Karakteristik serologis tes Coombs pada AHAI tipe hangat adalah kombinasi anti-IgG + anti-C3 (84,8%) dan anti IgG (15,2%). Proporsi subjek AHAI tipe hangat yang memiliki positif dengan kortikosteroid adalah 71,7%. Etiologi AHAI tipe dingin adalah idiopatik atau primer (66,7%) dan mieloma multipel (33,3%). Tes Coombs menunjukkan anti-C3 dan ditemukannya cold antibody. Semua subjek AHAI tipe dingin berespon negatif setelah mendapatkan kortikosteroid. Didapatkan etiologi AHAI tipe campuran primer yang berespon positif setelah mendapatkan kortikosteroid dan tes Coombs menunjukkan anti-IgG + anti-C3 disertai antibody non-specific dengan titer yang tinggi.

Simpulan. Mayoritas subjek AHAI adalah AHAI tipe hangat. Proporsi subjek AHAI tipe hangat yang berespon positif setelah mendapatkan kortikosteroid 71,7%, AHAI tipe campuran juga berespon positif, sedangkan semua subjek AHAI tipe dingin berespon negatif.

Kata Kunci: AHAI tipe dingin, AHAI tipe hangat, AHAI tipe campuran, kortikosteroid, respon pengobatan

 

Auto Immune Hemolytic Anemia (AIHA) Patients Profile and Treatment Response to Corticosteroids in Cipto Mangunkusumo Hospital

Introduction. Autoimmune hemolytic anemia (AIHA) is one of the immunological diseases that causes hemolysis. Data on demographic characteristics and treatment response of AIHA patients has not available in Indonesia. This study was conducted to identify the profile of autoimmune hemolytic anemia (AIHA) patients and their response of treatment after receiving corticosteroid treatment.

Methods. This cross-sectional study used data from the medical records of AIHA patients in the outpatient clinic division of the Hematology-Medical Oncology, Department of Internal Medicine, Dr. Cipto Mangunkusumo National Hospital between 2004-2008 which included: patient demography characteristics, AIHA classification and etiology, as well as the response of treatment to initial dose of corticosteroid, equivalent to prednisone 1-1,5 mg/kgbw/day for 3-4 weeks; with positive response marked by increase of Hb >10 g/dL.

Results. From total of 50 subjects, the proportion of warm type, cold type and mix type were 92%, 6% and 2%, respectively. Most of the etiology of AIHA warm type was idiopathic or primary (54.3%), whereas the etiology of secondary type were systemic lupus erythematosus/LES (41.3%), autoimmune hepatitis (2.2%) and chronic lymphocytic leukemia (2.2%). Characteristics of serological Coombs tests on AIHA warm type were combination of anti-IgG + anti-C3 (84.8%) and the anti-IgG (15.2%). Meanwhile, the proportion of subjects with AIHA warm type that has a positive response to corticosteroids were 71.7%. This study found that the etiology of AIHA cold type were idiopathic or primary (66.7%) and multiple myeloma (33.3%). Coombs tests showed an anti-C3 and cold antibody and all subjects had negative response to corticosteroids. This study also found the etiology of AIHA mixed type was primary, had positive response to corticosteroids and Coombs test demonstrated anti-IgG + anti-C3 accompanied by a non-specific antibody with high titer.

Conclusions. The majority of AIHA subjects are warm type AIHA, with a small portion being cold and mixed type. The proportion of warm type AIHA that respond positively to corticosteroids is 71,7%. All cold type AIHA subjects do not respond to corticosteroids while mixed type AIHA subjects show positive response.

Keywords

cold type AIHA, mixed type AIHA, response to steroid treatment, warm type AIHA

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