Anemia AplastikDr.Jalila Zamzam, Sp.A*

Gangguan hematopoisis ditandai : -penurunan produksi eritroid,mieloid, dan megakariosit dalam sumsum tulang Pansitopenia pada darah tepi - keganasan sistem hematopoitik (-) - kanker metastatik yg menekan sumsum tulang (-)

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Aplasia : eritropoitik granulopoitik megakariositik

Panmieloptisis = Anemia Aplastik

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DiagnosisThe International Agranulocytosis and Aplastic Anemia Study (IAAAS) Aplastic anemia :Haemoglobin 10 g/dl atau Hematokrit 30Thrombocytopenia, 50.000/mm3Leucocytopenia, 3500/mm3 granulositopenia, 1,5 x 109/L

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Herediter: Fanconi anemia Dyskeratosis congenital Schwachman-Diamond syndrome Amegakaryocytic thrombocytopenia Acquired: Obat-obatan :kloramfenikol, antirematik, antitiroid Zat Kimia : Benzene, insektisida Infeksi : virus hepatitis,tb milier Penyakit sistem imun *Transfusion associated Graft versus host disease * radioaktif : radiasi, sinar Rontgen70% kasus anemia aplastik : idiopatik

ETIOLOGI :*

Patofisiologi1.Kerusakan sel induk hematopoitik LTC-IC(Longterm Culture initiating cell), LTMC (Longterm MarrowCulture), jumlah sel induk CD34 menurun sampai 1-10% dari normal BMT berhasil pd 60-80%kasus2.Proses imunologi yg menekan hematopoisisth/Imunosupresif dgn Siklosporin atau Metilprednisolon menyembuhkan 70%3.Kerusakan mikro sumsum tulangbanyak penelitian tidak mendukung*

ClassificationA. Acquired aplastic anemia1. Idiopathic2. Associated with radiation, chemical, or drugs a. Ionazing radiation (accidental or therapeutic exposure)b. Antineoplastic drugsc. Drugs or chemical exposure*

3. Associated or following infections:a. Viruses: hepatitis C, EBV, CMV, parvovirus B19, HIVb. Miliary tuberculosisc. Chronic mucocutaneous candidiasis4. Paroxismal Nocturnal Hemoglobinuria (PNH)5. Systemic disease (e.g., pancreatic disease, Shwachman-Diamond syndrome)*

6. Graft Versus Host Disease (GVHD)7. Immunologic disorders (e.g., X-linked lymphoproliferative disease, Thymoma)8. Pregnancy9. Preleukemic syndrome*

B. Constitutional aplastic anemia1. With congenital anomalies (Fanconi anemia)2. Without congenital anomalies (delayed onset)3. Delayed onset aplasia with congenital Dysceratosis4. Constitutional congenital aplastic anemia (Tipe II)- congenital trombocytopenia with delayed onset pancytopenia without congenital anomalies*

Manifestasi KlinisPetekie, Echymosis, purpura, epistaksisAnemia pucat, anoreksia,palpitasi, dyspnoeFollowed by systemic manifestation or local infections with fever, sore throatTidak ditemukan adanya pembesaran organ hepar dan limpa(tanpa hepato/splenomegali)*

Peripheral blood: Decreased Hb Normocytic Normochromic Leucophenia Relative lymphocitosis Prolonged B.T B.M.P :Decreased in hematopoietic activityVery rare megakariosit /(-)>> fatty cell>> R.E.S cell*

TINGKAT KEPARAHAN ANEMIA APLASTIK :Menurut The Internatinal Agranulocytosis and Aplastic Anemia Study (IAAS) :

1.Mild / Moderate aplastic anemia (MAA) adanya penurunan jumlah sel darah dimana penurunan tidak seberat pada severe aplastic anemia (SAA)

Severe aplastic anemia (SAA) selularitas sum-sum tulang (produksi sel darah) kurang dari 25% dan diikuti sedikitnya dua gejala sebagai berikut :- Jumlah netropil : < 500 /mm3- Jumlah platelet : < 20.000/mm3- Jumlah retikulosit : < 20.000/mm3

Very severe aplastic anemia (VSAA) jumlah netropil : < 2.00/mm3*

I.T.P & A.T.PA.L.LPreleukemic state in acute leukemiaMyelofibrosisNeoplasmic infiltration

Differential Diagnosis*

Treatment1. Removal the causative agent2. Therapeutic approaches: a. Supportive care Blood transfusion, antibiotics b. Androgen dan glucocorticosteroid therapy 1. Fluoxymesterone,orally, 0,4-1mg/kg daily 2. Methyltestosteron, or testosteron propionate, 1-2 mg/kg, usually 30 to 50 mg daily in divided dose3. Testosteron enanthate, IM, 4 mg/kg once weekly or 9 mg/kg every 2 weeks *

4. Testosteron cypionate, IM, 4 mg/kg once weekly or 8 mg/kg every 2 weeks5. Oxymetholone, orally, 2 to 6,5mg/kg daily, max 100 mg daily6. Nadrolone Decanoate,IM, 25 to 50mg every 3 to 4 weekss3.Imunosuppresive therapyAntiThymociteGlobulin and dosis tinggi Cyclophosphamide sebelum bone marrow transplantation baik4.Bone marrow transplantationHLA-matchedHLA-identical sibling shows the best result*

5. Hematopoietic Growth Factors G-CSF, GM-CSF dan IL-3 : dapat meningkatkan jumlah granulosit dapat diberikan pada pasien yang menderita infeksi berat setelah terapi immunosuppresi atau BMT*

PROGNOSIS :Prognosis bergantung pada :Gambaran sum sum tulang hiposeluler atau aselulerKadar Hb F yang lebih dari 200 mg% memperlihatkan prognosis yang lebih baik3.Jumlah granulosit lebih dari 2000/mm3 menunjukkan prognosis yang lebih baik4.Pencegahan infeksi sekunder

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Sebab kematianInfeksi : bronkopneumonia atau sepsisPerdarahan otak atau abdomen*

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