Dr. Erwin Budi Cahyono, SpPD

Gejala yg ditemukan saat pasien ke dokter Dicari latar belakang penyebabnya Pengobatan diberikan bila diagnosis sdh jelas Masalah klinik & Public Health

Penurunan Sel Darah Merah (SDM) dan kadar Hemoglobin (Hb) dibawah nilai normal

Menurut WHO 1972 :Ketinggian permukaan laut Hb lebih rendah11 gr% anak umur 6 bln 6 thn12 gr% Usia 6 thn 14 thn13 gr% Pria dewasa12 gr% Wanita dewasa tidak hamil11 gr% Wanita dewasa hamil

Pendekatan Penderita AnemiaRiwayat penyakit sekarang :(History of Present Illness)Status performanceObat-obatanDemamPenurunan BBGejala umum anemiaGejala sisten tertentu sesuai penyakit dasar

Riwayat Penyakit Keluarga Informasi penyakit darah yang diturunkan :

Autosomal dominant Autosomal recessive Sex linked traits

Kulit Mata Mulut Kelenjar limfe Tulang dada Organomegali

Penyebab / Etiologi Morfologik (Berdasarkan indeks Eritrosit, MCV, MCH, MCHC) Berdasarkan nilai hemoglobin

INDEKS ERITROSIT MCV =

HtEX 10% ( 80 -96 fl) MCH =

HbEX 10% ( 27,5 - 33,2 pg ) MCHC =

HbHtX 100% ( 34,4 - 35,5 )

Kehilangan darah (Hemorrhagik) Gangguan pembentukan (Aplasia, Defisiensi, penyakit kronik) penghancuran (Hemolitik)

Mikrositik hipokromik Normokromik mikrositik Makrositik

Ringan (mild) : 8 10 gr%Sedang (moderate): 5 8 gr%Berat (severe): < 5 gr%

Anemia gravis istilah utk Anemia BeratAnemia Refrakter adalah Anemia yg berulang-ulang

Jarang Barat : 1,4 2,3 / 1 jt pddk / thn Asia timur & tenggara : 7 10/ 1 jt pddk/ thn

50% unknowm Klorampenikol, Benzene, Radiasi, Virus Kongenital (Fancony Syndrome)

Gangguan sel induk Kegagalan stroma mikroenvironment Defisiensi growth factor Supresi imun sumsum tulang

Gejala Anemia Gejala Perdarahan Gejala Infeksi

Gejala Anemia & Infeksi Organomegali ( - ) Bila Organomegali (+) pikirkan pnykt lain

Pansitopenia Retikulosit Eritrosil mungkin makrositik Neutrofil absolut

(ANC = Absolute Neutrophil Count)

Immunosupresi

- Prednison 1 mg/ KgBB/ hari/ oral - Anti Thymocyte globulin (ATG) 15 40 mg/ KgBB/ iv 4 10 hari - Cyclosporine (CSP) 3 7 mg/KgBB/hari/oral 4 6 bln - Kombinasi ATG & CSPTerapi Androgen Hemopoetic Growth Factor Supportive (Transfusi, Antibiotika)Transplantasi sumsum tulang

(Bone Marrow Transplantation)

Medium Survival 3 6 bulan

( Tanpa pengobatan )Meninggal krn Sepsis & Perdarahan

Gangguan kronik yg sering dijumpai1/3 s/d wanita sehat di USA cadangan besi nol10% dari jml ini menderita Anemia defisiensi Fe

Tahapan Defisiensi FeIron depletion (cadangan besi / - )Iron deficiency (SI saturasi transferrin rendah)Iron deficiency Anemia

Intake yg non adekwatMalabsorbsiPerdarahan kronik (Chronic Blood Loss)Hamil & menyusuiHemolisis intravaskular & HemoglobinuriaKombinasi

Fe sintesis Hb eritropoesis aktifitas sitokromDisfungsi neurologik sekresi asam lambungAtrofi mukosa mulut & lambung

Gejala umum AnemiaKorelasi nilai Hb dgn gejala tidak jelasIrritabilitas, nyeri kepalaPerlambatan respons neurologik

Pucat, lidah licin & merahStomatitis, cheilitis angularisKoilonychiaPerdarahan retinaSplenomegalia

Laboratorium (1)Anisositosis, ovalositosis ringan, sel targetElongasi Elliptositosis Hipokromik (sel pensil)Hipokromia progresif (MCH rendah)Mikrositosis (MCV rendah)Retikulosit rendah /

Laboratorium (2)Jumlah E, Hb, Ht Trombositosis (50-75% penderita)BMA : - Selularitas & ratio ME bervariasi

- Sideroblast / nol- Eritroblast kecilSI : / normalTIBC : Serum Ferritin : < 10 mg/L

SI = Serum Iron, TIBC = Total Iron binding Capacity, Sat = SaturationBMSI = Bone Marrow Storage Iron* SI and TIBC occasionally normal in iron deficiencyLaboratorium (1)Anamnesis & laboratoriumBila dijumpai Chronic Blood Loss diperlukan pemeriksaan endoskopik saluran cerna

Cause of Hypochromic AnemiaSITIBC%SatBMSIIron DeficiencyThalassemiasSideroblastic AnemiasChronic disease* - N* - N - N* - N - N

Pengobatan Oral Iron Therapy Diet rendah tdk cukup Aman & murah Hindari hematinic ganda Jgn diberikan bersama makanan, antasida, H2 antagonis Diteruskan selama 12 bln sesudah Hb normal Dosis total 150-200 mg besi elemental (1 tablet sulfas ferron (325 mg) mengandung 65 mg besi elemental) Dosis 3 4 kali/hari 1 jam sebelum makan Efek samping : Intoleransi gastrointestinal

Parenteral Iron Therapy

Keadaan malabsorbsi tidak kooperatif Intoleransi thd preparat oral Iron Dextram : berisi 50 mg besi elemental im / iv Diteruskan selama 12 bln sesudah Hb normal Hati-hati reaksi AnaphylaxisPengobatan

CLASSIFICATION of MACROCYTIC ANEMIASRETICULOCYTE COUNTNormal or DecreasedIncreasedHemolytic disorderHemorrhageTreated B12/Folate Deficiency

NORMAL OR DECREASED RETICULOCYTE COUNTMacrocytes round;no hypersegmented PMNs on smearMacrocytes oval; hypersegmentedPMNs on smearBone marrownonmegaloblasticSerum B12 / folate,Red cell folate ( suspecttreatable megaloblastic disorderRule out refractory anemiaSideroblastic anemiaMyelodysplasiaDrug/Toxin effectAplastic AnemiaLiver diseaseMacrocytic anemia...

CLASSIFICATION PROTOCOL FOR MICROCYTIC ANEMIASSMEAR REVIEWNO CHANGESSUGGESTIVE or DIAGNOSTICWHITE CELL/RED CELL CHANGES

NO CHANGES SMEAR REVIEWMicrocytic Anemia...RDW, Normal ( 5x1012/LSuspect Iron DeficiencySuspect ThalassemiaAbnormal HgbHb A2> 4,0% N(

SUGGESTIVE OR DIAGNOSTICWHITE CELL/RED CELL CHANGES

Microcytic Anemia... Sikling,targeting Hb SS, double heterozygote for S Thalassemia MinorTargeting, stipping Complication of Thalassemia MinorMarked targeting HbE, HbC, Obstructive liver diseaseRed cell fragments/polychromatism Unsuspected hemolysisRouleaux Increase in globulins or decreasealbumin (benign/malignant)Neutrophils Hypersegmentation with or withoutmacrocytes