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Anemia HemolitikAnemia Hemolitik

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Anemia hemolitikAnemia hemolitik ANEMIA yg disebabkan proses hemolisisANEMIA yg disebabkan proses hemolisis

Hemolisis : pemecahan eritrosit dlm pembuluh darah sebelum waktunya(<120 Hemolisis : pemecahan eritrosit dlm pembuluh darah sebelum waktunya(<120 hr)hr)

Hemolisis berbeda dg proses penuaan(eritrosit sdh cukp umur)Hemolisis berbeda dg proses penuaan(eritrosit sdh cukp umur)

Hemolisis dpt terjadi di dalam pembuluh darah(intra vaskuler) atau diluar Hemolisis dpt terjadi di dalam pembuluh darah(intra vaskuler) atau diluar pebuluh darah(ekstra vaskuler).pebuluh darah(ekstra vaskuler).

Normal,hemolisis akan di respon tubuh dg peningkatan eritropoesis dlm Normal,hemolisis akan di respon tubuh dg peningkatan eritropoesis dlm sumsum tulang.Kemampuan maksimum sumsum tulang untk meningkatkan sumsum tulang.Kemampuan maksimum sumsum tulang untk meningkatkan eritropoesis adalah 6-8 x normal.Apabila derajad hemolisis tdk terlalu eritropoesis adalah 6-8 x normal.Apabila derajad hemolisis tdk terlalu berat(pemendekan masa hidup eritrosit sekitar 50 hari),maka sumsum tlng berat(pemendekan masa hidup eritrosit sekitar 50 hari),maka sumsum tlng masih mampu kompensasi,shg tdk,akn timbul anemia.Keadaan ini dsbt masih mampu kompensasi,shg tdk,akn timbul anemia.Keadaan ini dsbt keadaan hemolisis yg terkompensasi(compensated hemolytic state)keadaan hemolisis yg terkompensasi(compensated hemolytic state)

Apabila kemampuan kompensasi sumsum tulang dilampaui,akan terjadi anemi Apabila kemampuan kompensasi sumsum tulang dilampaui,akan terjadi anemi yg dsbt ANEMI HEMOLITIK.yg dsbt ANEMI HEMOLITIK.

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Anemia hemolitik merupakan anemia yg Anemia hemolitik merupakan anemia yg tidak terlalu sering dijumpai,bila tidak terlalu sering dijumpai,bila dijumpai,hrs dg pendekatan diagnosis yg dijumpai,hrs dg pendekatan diagnosis yg tepat.tepat.

Dilaporkan anemia hemilitik merupakan Dilaporkan anemia hemilitik merupakan 6% dari kasus anemia,menempati urutan ke 6% dari kasus anemia,menempati urutan ke tiga stlh anemia aplastik dan keganasan tiga stlh anemia aplastik dan keganasan hematologik.hematologik.

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KLASIFIKASI ANEMIA KLASIFIKASI ANEMIA HEMOLITIKHEMOLITIK

Klasifikasi anemia hemolitik berdasarkan Klasifikasi anemia hemolitik berdasarkan penyebabpenyebabnyanya,dibagi dua gol.besar:,dibagi dua gol.besar:

1,Anemia hemolitik ok faktor di dalam eritrosit 1,Anemia hemolitik ok faktor di dalam eritrosit sendiri(intrakorpuskuler),yg sbgn besar heriditer-familier.sendiri(intrakorpuskuler),yg sbgn besar heriditer-familier.

2.Anemia hemolitik ok faktor di luar eritrosit(ekstra 2.Anemia hemolitik ok faktor di luar eritrosit(ekstra korpuskuler),yg sbgn besar bersifat didapat(acquired).korpuskuler),yg sbgn besar bersifat didapat(acquired).

Di klinik,khususnya di peny.dalam,anemia yg paling Di klinik,khususnya di peny.dalam,anemia yg paling banyak dijumpai adalah anemia hemolitik banyak dijumpai adalah anemia hemolitik autoimun.Agaknya,anemia hemolitik heriditer-familier autoimun.Agaknya,anemia hemolitik heriditer-familier hanya sbgn kecil yg dpt mencapai usia dewasa shg banyak hanya sbgn kecil yg dpt mencapai usia dewasa shg banyak dijumpai pd anak.dijumpai pd anak.

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PATOFISIOLOGIPATOFISIOLOGIANEMIA HEMOLITIKANEMIA HEMOLITIK

1.Penurunan kadar hemoglobin y akan mengakibatkan 1.Penurunan kadar hemoglobin y akan mengakibatkan anemia.Hemolisis akan terjadi perlahan-lahan,shg dapat anemia.Hemolisis akan terjadi perlahan-lahan,shg dapat diatasi oleh mekanisme kompensasi tubuh,tetapi dp juga diatasi oleh mekanisme kompensasi tubuh,tetapi dp juga terjadi tiba-tiba shg segera menurunkankadar hemoglobin. terjadi tiba-tiba shg segera menurunkankadar hemoglobin. Normal,hemolisis akan di respon tubuh dg peningkatan Normal,hemolisis akan di respon tubuh dg peningkatan eritropoesis sumsum tulang.dpt 6-8 x normal,shg tidak eritropoesis sumsum tulang.dpt 6-8 x normal,shg tidak terjadi anemia,keadaan ini dsbt hemolitik terjadi anemia,keadaan ini dsbt hemolitik terkompensasi.Umur eritrosit sktr 50 hr,sumsum tlng terkompensasi.Umur eritrosit sktr 50 hr,sumsum tlng masih mampu kompensasi,bila tdk,akn timbul anemia masih mampu kompensasi,bila tdk,akn timbul anemia hemolitik.hemolitik.

2 Peningkatan hasil pemecahan eritrosit dlm tubuh.2 Peningkatan hasil pemecahan eritrosit dlm tubuh. Hemolisis berdasar Hemolisis berdasar tempattempatnya di bagi dlm 2 bagian : nya di bagi dlm 2 bagian :

a.Hemolisis ekstravaskulera.Hemolisis ekstravaskuler b.Hemolisis intravaskuler.b.Hemolisis intravaskuler.

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A.Hemolisis ekstra vaskulerA.Hemolisis ekstra vaskuler A.Hemolisis ekstra vaskulerA.Hemolisis ekstra vaskuler:lebih sering dijumpai dp :lebih sering dijumpai dp

intravaskuler.Hemolisis terjadi pd sel Makrofag dari sistem retikulo intravaskuler.Hemolisis terjadi pd sel Makrofag dari sistem retikulo endotelial(RES),pd Lien,hepar,ssm tulang,ok sel ini mengandung endotelial(RES),pd Lien,hepar,ssm tulang,ok sel ini mengandung heme oksigenase.heme oksigenase.

Pemecahan eritrosit ini menghasilkan globin yg akan dikembalikan ke Pemecahan eritrosit ini menghasilkan globin yg akan dikembalikan ke protein pool,besi di kembalikan ke Makrofag(cadangan besi)yg protein pool,besi di kembalikan ke Makrofag(cadangan besi)yg dipakai kembali,sedang protoporfirin menghasilkan gas CO dan dipakai kembali,sedang protoporfirin menghasilkan gas CO dan bilirubin.bilirubin.

Bilirubin dlm darah berikatan dg albumin menjadi Bilirubin Bilirubin dlm darah berikatan dg albumin menjadi Bilirubin Indirek,mengalami konyugasi,dlm hati,menjadi bilirubin Direk,di Indirek,mengalami konyugasi,dlm hati,menjadi bilirubin Direk,di buang melalui empedu,meningkatkan sterkobilinogen dlm feses dan buang melalui empedu,meningkatkan sterkobilinogen dlm feses dan urobilinogen dlm urinurobilinogen dlm urin

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B.Hemolisis intravaskuler B.Hemolisis intravaskuler

Pemecahan eritrosit intravaskuler menyebabkan Pemecahan eritrosit intravaskuler menyebabkan lepasnya hemoglobin bebas kedalam lepasnya hemoglobin bebas kedalam plasma.Hemoglobin bebas ini di ikat oleh plasma.Hemoglobin bebas ini di ikat oleh Haptoglobin(globulin alfa),hg kdr Haptoglobin Haptoglobin(globulin alfa),hg kdr Haptoglobin akan turun.Komplek Haptoglobin akan akan turun.Komplek Haptoglobin akan dibersihkan oleh hati dan RES dl bbrp menit.dibersihkan oleh hati dan RES dl bbrp menit.

Apabila kapasitas haptoglobin dilampaui maka Apabila kapasitas haptoglobin dilampaui maka akan tejadilah hemoglobin bebas dlm plasma yg akan tejadilah hemoglobin bebas dlm plasma yg dsbt hemoglobinemia.dsbt hemoglobinemia.

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PATOFISIOLOGIPATOFISIOLOGIANEMIA HEMOLITIKANEMIA HEMOLITIK

3. Kompensasi sumsum tulang untk meningkatkan 3. Kompensasi sumsum tulang untk meningkatkan eritropoesis.eritropoesis.

Destruksi eritrosit dlm darah tepi akan merangsang Destruksi eritrosit dlm darah tepi akan merangsang mekanisme biofeedback(melalui eritropoetin)shg sumsum mekanisme biofeedback(melalui eritropoetin)shg sumsum tulang meningkatkan eritropoesis.Sumsum tulang normal dpt tulang meningkatkan eritropoesis.Sumsum tulang normal dpt meningkatkan kemampuan eritropoesisnya 6-8 kali lipat.meningkatkan kemampuan eritropoesisnya 6-8 kali lipat.

Peningkatan ini ditandai oleh penigkatan jumlah Peningkatan ini ditandai oleh penigkatan jumlah eritroblast(normoblast)dlm sumsum tulang shg terjadi eritroblast(normoblast)dlm sumsum tulang shg terjadi hiperplasia normoblastik..hiperplasia normoblastik..

Normoblast sering dilepaskan ke darah shg terjadi Normoblast sering dilepaskan ke darah shg terjadi normoblastemia.normoblastemia.

Sel eritrosit muda yg masih mengandung sisa inti(RNA)dsbt Sel eritrosit muda yg masih mengandung sisa inti(RNA)dsbt sbg retikulosit,akan dilepas ke darah tepi shg terjadi sbg retikulosit,akan dilepas ke darah tepi shg terjadi retikulosis dlm darah tepi.retikulosis dlm darah tepi.

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Gejala Klinik Anemia Hemolitik Gejala Klinik Anemia Hemolitik

Bervariasi:ok perjalanan Bervariasi:ok perjalanan penyakit(akut,kronik),dan tempat penyakit(akut,kronik),dan tempat kejadian(intra atau ekstra vaskuler).kejadian(intra atau ekstra vaskuler).

Gejala klinik dibagi dua:Gejala klinik dibagi dua: A.Anemia hemolitik kronik,heriditer A.Anemia hemolitik kronik,heriditer

familierfamilier B.Anemia hemolitik akut didapat(acquired)B.Anemia hemolitik akut didapat(acquired)

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Gejala Klinik( Anemia Gejala Klinik( Anemia hemolitik)hemolitik)

Gambaran klinik anemia hemolitik sangat berfariasi Gambaran klinik anemia hemolitik sangat berfariasi disebabkan oleh perjalanan penyakit(akut atau kronik)dan disebabkan oleh perjalanan penyakit(akut atau kronik)dan tempat kejadian hemolisis(intra/ekstravaskuler)shg pd tempat kejadian hemolisis(intra/ekstravaskuler)shg pd umumnya dilihat dari gejala kliniknya anemia hemolitik umumnya dilihat dari gejala kliniknya anemia hemolitik dpt dibagi menjadi 2 golongan besar:dpt dibagi menjadi 2 golongan besar:

a.Anemia hemolitik kronik heriditer-familiera.Anemia hemolitik kronik heriditer-familier b.Anemia hemolitik akut didapat(acquired)b.Anemia hemolitik akut didapat(acquired) Kedua jenis hemolitik in mempunyai gambaran klinik yg Kedua jenis hemolitik in mempunyai gambaran klinik yg

berbeda,di mana anemia hemolitik kronk heriditer-familier berbeda,di mana anemia hemolitik kronk heriditer-familier di dominasi gejala akibat hemolisis ekstravaskuler yg di dominasi gejala akibat hemolisis ekstravaskuler yg berlangsung perlahan lahan,sedang pd anemia hemolitik berlangsung perlahan lahan,sedang pd anemia hemolitik akut didapat terjadi hemolisis ekstravaskuler masif atau akut didapat terjadi hemolisis ekstravaskuler masif atau hemolisis intravaskuler,walau tdk bisa dipisahkan secara hemolisis intravaskuler,walau tdk bisa dipisahkan secara tegas.tegas.

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Gejala Klinik( Anemia hemolitik)Gejala Klinik( Anemia hemolitik)dapat dibagi menjadi tiga,:dapat dibagi menjadi tiga,:

1.Gejala Umum Anemia(anemic Syndrome)1.Gejala Umum Anemia(anemic Syndrome) 2.Gejala hemolisis ekstra/intra vaskuler2.Gejala hemolisis ekstra/intra vaskuler 3.Gejala penyakit dasar(penyebab)masing 3.Gejala penyakit dasar(penyebab)masing

masing anemia hemoliktik. masing anemia hemoliktik.

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1.GEJALA UMUM ANEMIA1.GEJALA UMUM ANEMIA

Gejala timbul bila Hb <7-8 gr/dlGejala timbul bila Hb <7-8 gr/dl Makin berat penurunan,maka gejala mkn Makin berat penurunan,maka gejala mkn

brt,disamping tgt kecepatan penurunan brt,disamping tgt kecepatan penurunan hemoglobin.hemoglobin.

Pada anemia hemolitik akut,penurunan lbh Pada anemia hemolitik akut,penurunan lbh cepat daripada kronikcepat daripada kronik

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2.GEJALA HEMOLITIK2.GEJALA HEMOLITIK

Anemia Hemolitik Kronik familier heriditerAnemia Hemolitik Kronik familier heriditer Gejala: Gejala:

timbul:Ikterus,Splenomegali/Hepatomegali,timbul:Ikterus,Splenomegali/Hepatomegali,kolelitiasis,kelainan tulang,ulkus kaki,serta kolelitiasis,kelainan tulang,ulkus kaki,serta timbulnya krisis.timbulnya krisis.

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3.GEJALA HEMOLITIK 3.GEJALA HEMOLITIK BERDASAR PENYAKIT BERDASAR PENYAKIT

IKTERUS:Timbul ok peningkatan bilirubin IKTERUS:Timbul ok peningkatan bilirubin indirek(unconjugated)dlm darah,shg ikterus indirek(unconjugated)dlm darah,shg ikterus bersifat acholuric jaundice(dlm urin tdk bersifat acholuric jaundice(dlm urin tdk dijumpai bilirubin).dijumpai bilirubin).

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GEJALA HEMOLITIKBERDASAR PENYAKIT GEJALA HEMOLITIKBERDASAR PENYAKIT (lanjut)(lanjut)

Splenomegali,kholelithiasis,ulkus kaki,kelainan Splenomegali,kholelithiasis,ulkus kaki,kelainan tulangtulang

Splenomegali,hepatomegali:selalu ada pd anemia Splenomegali,hepatomegali:selalu ada pd anemia Hemolitik kronik.Hemolitik kronik.

Kholelitiasis,pd anemia Hemolitik Kronik Kholelitiasis,pd anemia Hemolitik Kronik familier-heriditer.Batunya black pigment stone,40 familier-heriditer.Batunya black pigment stone,40 % radiopak.% radiopak.

Ulkus kaki:ulkus pd proksimalUlkus kaki:ulkus pd proksimal Kelainan tulang:pd thalasemia mayor.Kelainan tulang:pd thalasemia mayor. Krisis :ok penurunan Hb yg tiba-tiba.: dpt Krisis Krisis :ok penurunan Hb yg tiba-tiba.: dpt Krisis

aplastik,Krisis hemolitik:Hb tiba-tiba turun,Krisis aplastik,Krisis hemolitik:Hb tiba-tiba turun,Krisis megaloblastik,ok krng as.folat,yg disebabkan ok megaloblastik,ok krng as.folat,yg disebabkan ok eritropoesis yg meningkat.eritropoesis yg meningkat.

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KRISISKRISIS

Pada anemia hemolitik kronik sering terjadi penurunan Pada anemia hemolitik kronik sering terjadi penurunan kadar hemoglobin secara tiba tiba yg dsbt krisis.Krisis kadar hemoglobin secara tiba tiba yg dsbt krisis.Krisis pada anemia hemolitik dpt berupa :pada anemia hemolitik dpt berupa :

a.Krisis aplastik:krisis yg paling sering dijumpai yg a.Krisis aplastik:krisis yg paling sering dijumpai yg menimbulkan kegagalan hemopoesis transien.menimbulkan kegagalan hemopoesis transien.

b.Krisis hemolitik,terjadi hemolisis masif,shg b.Krisis hemolitik,terjadi hemolisis masif,shg menimbulkan penurunan hemoglobin secara tiba2,disertai menimbulkan penurunan hemoglobin secara tiba2,disertai retikulositosis dan pembesaran limpa.retikulositosis dan pembesaran limpa.

c.Krisis megaloblastik:yg timbul ok relatif kekurangan c.Krisis megaloblastik:yg timbul ok relatif kekurangan asam folat ok kebutuhan akibat eritropoesis yg sngt asam folat ok kebutuhan akibat eritropoesis yg sngt meningkat.meningkat.

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Anemia Hemolitik Akut DidapatAnemia Hemolitik Akut Didapat

Misal:reaksi transfusi,defisiensi G6PD yg Misal:reaksi transfusi,defisiensi G6PD yg mendapat obat oksidan,terjadi hemolisis intra mendapat obat oksidan,terjadi hemolisis intra vaskuler masif,maka gejalanya menyerupai acute vaskuler masif,maka gejalanya menyerupai acute febrile ilness.febrile ilness.

Gejala dpt smpai syok,ggl ginjal akut,nyeri Gejala dpt smpai syok,ggl ginjal akut,nyeri pinggang dan perut,skt kepala,kram perut(gejala pinggang dan perut,skt kepala,kram perut(gejala mirip akut abdomen)mirip akut abdomen)

Anemia hemolitik autoimun ditandai oleh Anemia hemolitik autoimun ditandai oleh hemolisis ekstra vaskuler,sering dg anemia berat.hemolisis ekstra vaskuler,sering dg anemia berat.

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Kelainan LaboratoriumKelainan Laboratorium

Kelainan lab.pd anemia hemolitik di Kelainan lab.pd anemia hemolitik di golongkan menjadi:golongkan menjadi:

1.Anemia1.Anemia 2.Kelainan lab.akibat proses hemolisis,2.Kelainan lab.akibat proses hemolisis, 3.Kelainan akibat kompensasi sumsum 3.Kelainan akibat kompensasi sumsum

tulangtulang 4.Kelaian lab.akibat penyakit dasar.4.Kelaian lab.akibat penyakit dasar.

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AnemiaAnemia

Anemia pd anemia hemolitik sbgn besar bersifat Anemia pd anemia hemolitik sbgn besar bersifat normokromik normositer,dapat juga bersifat normokromik normositer,dapat juga bersifat hipokromik mikrositer spt pd hipokromik mikrositer spt pd thalassemia.Penurunan kadar hemoglobin sngt thalassemia.Penurunan kadar hemoglobin sngt bervariasi,mulai dari ringan,smp berat.bervariasi,mulai dari ringan,smp berat.

Penurunan kadar Hb dpt berlangsung cepat,dpt Penurunan kadar Hb dpt berlangsung cepat,dpt berlangsung perlahan,spt pd anemia hemolitik berlangsung perlahan,spt pd anemia hemolitik kronik.kronik.

Penurunan kadar Hb>1 g/dl dalam waktu Penurunan kadar Hb>1 g/dl dalam waktu semingu,tanpa disertai perdarahan,mrpkan satu semingu,tanpa disertai perdarahan,mrpkan satu petunjuk ke arah anemia hemolitik.petunjuk ke arah anemia hemolitik.

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PENDEKATAN DIAGNOSISPENDEKATAN DIAGNOSIS

Ada 2 tahap:Ada 2 tahap: 1.Menentukan adanya Anemia hemolitik.1.Menentukan adanya Anemia hemolitik. 2.Menetukan Penyebab Spesifik Anemia 2.Menetukan Penyebab Spesifik Anemia

hemolitik.hemolitik.

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Menentukan adanya Anemia Menentukan adanya Anemia hemolitik.hemolitik.

Anemia Hemolitik dpt di diagnosa jika trdpt anemia dg destruksi Anemia Hemolitik dpt di diagnosa jika trdpt anemia dg destruksi eritrosit dan/atau peningkatan eritropoesis,tdk ada tes tunggal,hrs dg eritrosit dan/atau peningkatan eritropoesis,tdk ada tes tunggal,hrs dg tes yg lain.tes yg lain.

Curigai Anemia Hemolitik (Wintrobe)bila:Curigai Anemia Hemolitik (Wintrobe)bila:

1.Destruksi eritrosit berlebihan,peningkatan eritropoesis.1.Destruksi eritrosit berlebihan,peningkatan eritropoesis.

2.Anemi persisten dg retikulosis tanpa ada tanda perdarahan yg jelas.2.Anemi persisten dg retikulosis tanpa ada tanda perdarahan yg jelas.

3.Terdapat penurunan Hb >1gr/dl dlm waktu 1 minggu(melebihi 3.Terdapat penurunan Hb >1gr/dl dlm waktu 1 minggu(melebihi kemampuan kompensasi eritropoesis)serta perdarahan akut dan kemampuan kompensasi eritropoesis)serta perdarahan akut dan

hemodilusi dpt disingkirkan,mk Anemia Hemolitik dpt ditegakkan.hemodilusi dpt disingkirkan,mk Anemia Hemolitik dpt ditegakkan.

4.Apabila dijumpai hemoglobinuria atau tanda hemolisis intra 4.Apabila dijumpai hemoglobinuria atau tanda hemolisis intra vaskuler yg lain.vaskuler yg lain.

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Menetukan Penyebab Spesifik Menetukan Penyebab Spesifik Anemia hemolitik.Anemia hemolitik.

Anamnese yg teliti,pemeriksaan apusan darah dan tes Anamnese yg teliti,pemeriksaan apusan darah dan tes Coombs.Maka pasien dpt dikelompokkan menjadi lima.Coombs.Maka pasien dpt dikelompokkan menjadi lima.

1.Ada riwayat terpapar infeksi,bahan kimia.1.Ada riwayat terpapar infeksi,bahan kimia. 2.Tes Coombs (+),ditetapkan sbg Anemia 2.Tes Coombs (+),ditetapkan sbg Anemia

imunohemolitik.Lalu cari penyakit dasar,tes serologi,untk imunohemolitik.Lalu cari penyakit dasar,tes serologi,untk tahu sifat antibodi yg dijumpaitahu sifat antibodi yg dijumpai

3.Kasus dg Anemia sferositik ,Coombs tes (-).3.Kasus dg Anemia sferositik ,Coombs tes (-). 4.Kasus dg kelainan morfologi yg lain.Sel target,akan 4.Kasus dg kelainan morfologi yg lain.Sel target,akan

mengarah thalassemia,sel sabit sbg patognomonik anemia mengarah thalassemia,sel sabit sbg patognomonik anemia sel sabit.sel sabit.

5.Kasus tanpa kelainan morfologi,tes Coombs,perlu tes yg 5.Kasus tanpa kelainan morfologi,tes Coombs,perlu tes yg lain.lain.

Bila semua diatas negatif,perlu pemeriksaan spesifik,pd Bila semua diatas negatif,perlu pemeriksaan spesifik,pd pusat lab.yg maju.pusat lab.yg maju.

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DIAGNOSIS BANDINGDIAGNOSIS BANDINGHEMOLITIK ANEMIAHEMOLITIK ANEMIA

1.Anemia perdarahan akut.Disini dpt di bedakan 1.Anemia perdarahan akut.Disini dpt di bedakan ok tdk ada ikterus,dan kadar Hb meningkat pd ok tdk ada ikterus,dan kadar Hb meningkat pd pemeriksaan berikutnya.pemeriksaan berikutnya.

2.Anemia ok eritropoetik inefektif,dg ikterus 2.Anemia ok eritropoetik inefektif,dg ikterus akholurik dan hiperplasi normoblastik.akholurik dan hiperplasi normoblastik.

3.Anemi ok perdarahan ke rongga 3.Anemi ok perdarahan ke rongga peritoneal,jaringan lain,sulit dibedakan dg anemia peritoneal,jaringan lain,sulit dibedakan dg anemia hemolitik.hemolitik.

4.Kasus ikterik tanpa anemia,spt sindrome Gilbert.4.Kasus ikterik tanpa anemia,spt sindrome Gilbert. 5.Adanya mioglobulinuria,spt pada kerusakan otot 5.Adanya mioglobulinuria,spt pada kerusakan otot

yg luas.yg luas.

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PENGOBATANPENGOBATAN

Pengobatan anemia hemolitika sangat Pengobatan anemia hemolitika sangat tergantung keadaan klinik,penyebabnya,jadi tergantung keadaan klinik,penyebabnya,jadi sngt bervariasi.Pada prinsipnya di bagi 3 sngt bervariasi.Pada prinsipnya di bagi 3 golongan:golongan:

1.Terapi Gawat Darurat1.Terapi Gawat Darurat 2.Terapi Suportif-simtomatik2.Terapi Suportif-simtomatik 3.Terapi Kausal.3.Terapi Kausal.

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1.Terapi Gawat Darurat1.Terapi Gawat Darurat

Pada hemolisis akut,tu intravaskuler,terjadi Pada hemolisis akut,tu intravaskuler,terjadi syok,ggl ginjal akut,maka hrs diatasi syok,ggl ginjal akut,maka hrs diatasi syok,pertahankan keseimbangan cairan dan syok,pertahankan keseimbangan cairan dan elektrolit,atasi fungsi ginjal.Anemi elektrolit,atasi fungsi ginjal.Anemi berat,pertimbangkan transfusi,dg hati-berat,pertimbangkan transfusi,dg hati-hati.Sebaiknya diberikan darah merah yg di hati.Sebaiknya diberikan darah merah yg di cuci(washed red cell),untk mengurangi beban cuci(washed red cell),untk mengurangi beban antibodi.Pada saat yg sama dpt diberikan steroid antibodi.Pada saat yg sama dpt diberikan steroid perenteral dosis tinggi,untk menekan fungsi perenteral dosis tinggi,untk menekan fungsi makrofagmakrofag

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2.Terapi Suportif-simtomatik2.Terapi Suportif-simtomatik

Untuk anemia hemolitik,diberikan spy menekan Untuk anemia hemolitik,diberikan spy menekan proses hemolisis terutama di limpa.Eritrosit dg proses hemolisis terutama di limpa.Eritrosit dg kerusakan ringan,di keluarkan lewat limpa,tetapi kerusakan ringan,di keluarkan lewat limpa,tetapi eritrosit dg kerusakan berat,dikeluarkan lewat eritrosit dg kerusakan berat,dikeluarkan lewat organ lain,tu hati.organ lain,tu hati.

Steroid memberikan respon pd kasus imuno Steroid memberikan respon pd kasus imuno hemolitik .hemolitik .

Pada anemi hemolitik kronik familier-Pada anemi hemolitik kronik familier-heriditer,sering perlu transfusi darah,agar kadar heriditer,sering perlu transfusi darah,agar kadar Hb terjaga.Hb terjaga.

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3.Terapi Kausal.3.Terapi Kausal.

Menjadi harapan untk dpt memberikan Menjadi harapan untk dpt memberikan kesembuhan total.kesembuhan total.

Sayangnya,sbgn kasus bersifat Sayangnya,sbgn kasus bersifat idiopatik,bagi yg kausanya jelas,terapi idiopatik,bagi yg kausanya jelas,terapi kausal harus segera diberikan,kausal harus segera diberikan,

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The Hemolytic Anemia may devided as :The Hemolytic Anemia may devided as :

1. Intracorpuscular:1. Intracorpuscular:2. 2. Extracorpuscular:Extracorpuscular:

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1. Intracorpuscular:1. Intracorpuscular:

Hereditary Spherocytosis, Elliptocytosis, and Related Hereditary Spherocytosis, Elliptocytosis, and Related DisorderDisorder

Hemolytic Anemia Related to Red Cell Enzyme Defect.Hemolytic Anemia Related to Red Cell Enzyme Defect. The ThalassemiaThe Thalassemia The Sickle Cell Diseases and Related Disorder.The Sickle Cell Diseases and Related Disorder. Hemoglobinopathies Associated with Unstable Hemoglobinopathies Associated with Unstable

HemoglobinHemoglobin

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22. Extracorpuscular:. Extracorpuscular:

2.1. Non Immunologic: 2.1. Non Immunologic: Traumatic Hemolytic Anemia, March Hemoglobinuria, Traumatic Hemolytic Anemia, March Hemoglobinuria,

Sports and Space-Related AnemiaSports and Space-Related Anemia Microangiopathic Hemolytic AnemiaMicroangiopathic Hemolytic Anemia Hemolytic Anemia Resulting from a Chemical or Physical Hemolytic Anemia Resulting from a Chemical or Physical

AgentAgent Hemolytic Anemia Resulting from a Infectious AgentHemolytic Anemia Resulting from a Infectious Agent

2.2. Immunologic2.2. Immunologic Hemolytic Anemia Resulting from Warm-Reacting Hemolytic Anemia Resulting from Warm-Reacting

Antibodies. Antibodies. Cryopathic Hemolytic AnemiaCryopathic Hemolytic Anemia Drug-induced Hemolytic Anemia Drug-induced Hemolytic Anemia Alloimmune Hemolytic Disease of the NewbornAlloimmune Hemolytic Disease of the Newborn

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LABORATORY FINDINGS:LABORATORY FINDINGS:Blood film : schistocytosis prominent, including, helmet cells, Blood film : schistocytosis prominent, including, helmet cells, triangular cells, spherocytestriangular cells, spherocytesEvaluation of reticulocyte count and increased concentration of Evaluation of reticulocyte count and increased concentration of plasma hemoglobin, urine hemoglobin, hemosiderin.plasma hemoglobin, urine hemoglobin, hemosiderin.Decreased serum haptoglobin levelDecreased serum haptoglobin levelCoagolation abnormalities due to consumption Coagolation abnormalities due to consumption coagulopathy.coagulopathy.Overt : decreased levels of factors V, VIII, antithrombin III,Overt : decreased levels of factors V, VIII, antithrombin III, fibrinogen ; elevated fibrin (ogen) degradation product.fibrinogen ; elevated fibrin (ogen) degradation product.TREATMENT:TREATMENT:Directed toward management of primary process Directed toward management of primary process underlying the microangiophaty.underlying the microangiophaty.Red cell transfusions to maintain adequate level of Red cell transfusions to maintain adequate level of hemoglobinhemoglobinPlatelet transfusions for bleeding caused by Platelet transfusions for bleeding caused by thrombocytopenia.thrombocytopenia.Heparin is controversialHeparin is controversial

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Hemolytic Anemia Resulting from Hemolytic Anemia Resulting from a Chemical or Physical Agenta Chemical or Physical Agent

Certain drugs can induce hemolysis in individuals with Certain drugs can induce hemolysis in individuals with abnormalities of erythrocytic enzymes such as glucose-6-abnormalities of erythrocytic enzymes such as glucose-6-phosphate dehydrogenase.phosphate dehydrogenase.

Other drugs induce hemolytic anamia through an immunological Other drugs induce hemolytic anamia through an immunological mechanism.mechanism.

Arsenic Hydride (ARSINE, AsH3)Arsenic Hydride (ARSINE, AsH3)Arsine gas is formed in many industrial processes.Arsine gas is formed in many industrial processes.Inhalation of arsine gas can lead to severe anemia, Inhalation of arsine gas can lead to severe anemia, hemoglobinuria and jaundice.hemoglobinuria and jaundice.

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LEADLEAD Lead poisioning in children usualy is result of Lead poisioning in children usualy is result of

ingestion of lead paint flakes of chewing lead-painted ingestion of lead paint flakes of chewing lead-painted objects. In adults, it usually is the result of industrial objects. In adults, it usually is the result of industrial exposure.exposure.

Lead intoxication leads to anemia largely caused by Lead intoxication leads to anemia largely caused by inhibition of hemesynthesis.inhibition of hemesynthesis.

The anemia is usually mild in adults but may be The anemia is usually mild in adults but may be severe in children.severe in children.

Copper:Copper: Hemolytic anemia may be induced by high levels of cooper in Hemolytic anemia may be induced by high levels of cooper in

patients hemodialyzed with fluid contaminated by copper patients hemodialyzed with fluid contaminated by copper tubing.tubing.

Tmh,tmbg

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Miscellanneus Drugs and ChemicalsMiscellanneus Drugs and Chemicals

Drugs and Chemicals that Have Been Reported to Cause Drugs and Chemicals that Have Been Reported to Cause Clinically Significant Hemolytic Anemia.Clinically Significant Hemolytic Anemia.

CHEMICALSCHEMICALSAnilineAnilineApiolApiolDichlorprop (herbicide)Dichlorprop (herbicide)FormaldehydeFormaldehydeHydroxylaminesHydroxylaminesLysolLysolMineral spiritMineral spiritNitrobenzeneNitrobenzeneResorcinResorcin

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DRUG:DRUG:

Amyl nitriteAmyl nitrite

MephenesinMephenesin

Methylene bule Methylene bule

OmeprazoleOmeprazole

Phenazopyridine (Pyridium)Phenazopyridine (Pyridium)

SalicylazosulfapydineSalicylazosulfapydine

Salicylazosulfapyridine (Azulfidine)Salicylazosulfapyridine (Azulfidine)

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Oxygen:Oxygen:Hemolytic anemia has developed in patients receiving Hemolytic anemia has developed in patients receiving hyperbaric oxygenation and in astronauts exposed to hyperbaric oxygenation and in astronauts exposed to 100% oxygen.100% oxygen.

Insect and Arachinid venomsInsect and Arachinid venomsSevere hemolysis may occur in some patients following Severe hemolysis may occur in some patients following bites by bees wasps, spiders, or scorpionsbites by bees wasps, spiders, or scorpionsSnake bites are only rarely a caused of hemolysisSnake bites are only rarely a caused of hemolysis

HeatHeatPatient with extensive burns may develop severe Patient with extensive burns may develop severe hemolytic anemia apparently as a result of direct hemolytic anemia apparently as a result of direct damage to the red cells by heatdamage to the red cells by heat

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Hemolytic Anemia Resulting from Infectious AgentHemolytic Anemia Resulting from Infectious Agent

Mechanisms:Mechanisms:

Hemolysis may be caused by:Hemolysis may be caused by:

Direct invasion by infecting organisms (malaria)Direct invasion by infecting organisms (malaria)

Elaboraion of hemolytis toxins (Clostridium Elaboraion of hemolytis toxins (Clostridium perfringens)perfringens)

Development of autoantibodies againt red blood Development of autoantibodies againt red blood cell antigens (Mycoplasma pneumoniae)cell antigens (Mycoplasma pneumoniae)

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Malaria :Malaria :The world’s most common cause of hemolytic anemia.The world’s most common cause of hemolytic anemia.Transmitted by bite of an infected female Transmitted by bite of an infected female AnophelesAnopheles mosquito. mosquito.Parasites grow intracellularly and parasitized cells are detroyed in Parasites grow intracellularly and parasitized cells are detroyed in the spleen.the spleen.

Clinical Featrues :Clinical Featrues :Febrile paroxysms are characteristically cyclic Febrile paroxysms are characteristically cyclic Splenomegaly in typicaly present in chronic infection.Splenomegaly in typicaly present in chronic infection.Falcifarum malaria is occasionaly associated with very severe Falcifarum malaria is occasionaly associated with very severe hemolysis and dark, almost black urine (black water fever).hemolysis and dark, almost black urine (black water fever).Diagnosis depends on demonstration of parasites on the blood film.Diagnosis depends on demonstration of parasites on the blood film.

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Hemolytic Anemia Resulting from Warm-Hemolytic Anemia Resulting from Warm-Reacting AntibodiesReacting Antibodies

In autoimmune hemolytic anemia (AHA), shortened red In autoimmune hemolytic anemia (AHA), shortened red blood cell (RBC) survival is the result of host antibodies blood cell (RBC) survival is the result of host antibodies that react with autologous RBC. that react with autologous RBC. AHA may be classified :AHA may be classified :SecondarySecondaryPrimary or idiopathic.Primary or idiopathic.AHA may also be classified by the nature of the antibody.AHA may also be classified by the nature of the antibody.““Warm-reacting”Warm-reacting”““Cold-reacting”Cold-reacting”Occasionally, mixed disorder occur.Occasionally, mixed disorder occur.Warm antibody autoimune hemolytic anemia (AHA) is Warm antibody autoimune hemolytic anemia (AHA) is the most common typethe most common type

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AHA occurs in all age groups.AHA occurs in all age groups.Calssification of Autoimmune Hemolytic Anemia:Calssification of Autoimmune Hemolytic Anemia:

I.I. On basic of serologic characterisitics of involved autoimmune proccesOn basic of serologic characterisitics of involved autoimmune procces

A.A. Warm –autoantibody type: autoantibody maximally active at body temperature 37Warm –autoantibody type: autoantibody maximally active at body temperature 37ooC.C.B.B. Cold-autoantibody type: autoantibody active at temperature below 37Cold-autoantibody type: autoantibody active at temperature below 37ooCCC.C. Mixed cold and warm autoantibodiesMixed cold and warm autoantibodies

II. On basic of presence or absence of underlying or significanly associated dissorder.II. On basic of presence or absence of underlying or significanly associated dissorder.

A.A. Primary or idiopathic AHAPrimary or idiopathic AHAB.B. Secondary AHASecondary AHA1.1. Assciated with lymphoproliferative disoreder (e.g. Hodgkin or non Hodgkin lymphoma)Assciated with lymphoproliferative disoreder (e.g. Hodgkin or non Hodgkin lymphoma)2.2. Assciated with the rheumatic disorders, particulary SLE.Assciated with the rheumatic disorders, particulary SLE.3.3. Assciated with certain infectionAssciated with certain infection4.4. Assciated with certain nonlymphoid neoplasms (e.g. ovarian tumors).Assciated with certain nonlymphoid neoplasms (e.g. ovarian tumors).5.5. Assciated with chronic inflamatory disease (e.g. ulcerative colitis).Assciated with chronic inflamatory disease (e.g. ulcerative colitis).6.6. Assciated with ingestion of certain drugs (e.g. alfa-methyldopa)Assciated with ingestion of certain drugs (e.g. alfa-methyldopa)

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In secondary AHA the autoantibody most likely develops In secondary AHA the autoantibody most likely develops from an immunoregulatory defect.from an immunoregulatory defect.Certain drugs (e.g. alfa-methildopa) can induce specific Certain drugs (e.g. alfa-methildopa) can induce specific antibodies in otherwise normal individuals by unknowm antibodies in otherwise normal individuals by unknowm mechanism. These subside spontaneously when the drug mechanism. These subside spontaneously when the drug is stopped.is stopped.RBC autoantibodies in AHA are pathogenic.RBC autoantibodies in AHA are pathogenic.

CLINICAL FEATURES:CLINICAL FEATURES:Symptoms are usually slow in onset, but rapidly Symptoms are usually slow in onset, but rapidly developing anemia can occurdeveloping anemia can occurPhysical examination may be normal if the anemia is Physical examination may be normal if the anemia is mild . Splenomegaly is common but not always observed . mild . Splenomegaly is common but not always observed . Jaundice and physical finding related to more Jaundice and physical finding related to more pronounced anemia may be noticed.pronounced anemia may be noticed.

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LABORATORY FEATURESLABORATORY FEATURESGeneral :General : Anemia can range mild to life –threatening.Anemia can range mild to life –threatening. Blood film reveals polychromasiaBlood film reveals polychromasia With severe cases, nucleated RBC,RBC fragments and With severe cases, nucleated RBC,RBC fragments and

occaionally erythrophagocytosis by monocyte may be seen.occaionally erythrophagocytosis by monocyte may be seen. Reticulocytosis is usually present if marow has not been injured.Reticulocytosis is usually present if marow has not been injured. Occasionally neutropenia and thrombocytopenia occur.Occasionally neutropenia and thrombocytopenia occur. Evans syndrome is a rare condition in which both immune-Evans syndrome is a rare condition in which both immune-

mediated RBC and platelet destructions occur.mediated RBC and platelet destructions occur. Bone marrow examination usually reveals erythroid hyperplasia.Bone marrow examination usually reveals erythroid hyperplasia. Unconjugated hyperbilirubinemia is often present.Unconjugated hyperbilirubinemia is often present. Haptoglobin levels are usually low, and LDH activity is elevated.Haptoglobin levels are usually low, and LDH activity is elevated. Urinary urobilinogen is routinely increased.Urinary urobilinogen is routinely increased.

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The diagnosis of AHA :The diagnosis of AHA : Usually achieved by the direct antiglobulin test (DAT)Usually achieved by the direct antiglobulin test (DAT) RBC may be coated with :RBC may be coated with :- IgG aloneIgG alone- IgG and complementIgG and complement- Complement only.Complement only. Rarely, anti-IgA and anti-IgM reactions are encountered.Rarely, anti-IgA and anti-IgM reactions are encountered. Free autoantibody may be detected by the indirect antiglobulin test.Free autoantibody may be detected by the indirect antiglobulin test. A positive indirect test with a negative direct tes probably does not indicated A positive indirect test with a negative direct tes probably does not indicated

autoimune disease but an alloantibody generated by a prior transfusion or autoimune disease but an alloantibody generated by a prior transfusion or pregnancy.pregnancy.

Occasional patients exhibit all the features of AHA but have negative DAT.Occasional patients exhibit all the features of AHA but have negative DAT. The relationship between the amount of bound antibody and degree of The relationship between the amount of bound antibody and degree of

hemolysis is variable.hemolysis is variable. Autoantibodies from AHA patient usually bind to all the types of RBC used Autoantibodies from AHA patient usually bind to all the types of RBC used

laboratory screening and therefore appear to be “nonspecific”. “public” laboratory screening and therefore appear to be “nonspecific”. “public” antigens. antigens.

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Differential Diagnosis:Differential Diagnosis:Paroxymal nocturnal hemoglobinuria.Paroxymal nocturnal hemoglobinuria.If the DAT is positive, further serologic If the DAT is positive, further serologic

charateriziations are warranted distinguish charateriziations are warranted distinguish

cold-reacting from warm-reacting autoantibodies.cold-reacting from warm-reacting autoantibodies.In recently transfused patient, alloantibody In recently transfused patient, alloantibody againts donor RBC may be detected by a positif againts donor RBC may be detected by a positif DATDAT

DAT: direck antiglobulin test

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Therapy Therapy Occasional patients have a possitive DAT but minimal hemolysis Occasional patients have a possitive DAT but minimal hemolysis and stable hematocrit. These patients need no treatment.and stable hematocrit. These patients need no treatment.TransfusionTransfusionTransfusion RBC are destroyed as fast or faster than host RBC Transfusion RBC are destroyed as fast or faster than host RBC but may tide the patient through a dangerous time.but may tide the patient through a dangerous time.Glucocorticoids quikly slow or stop hemolysis .Glucocorticoids quikly slow or stop hemolysis .Oral prednisolon at 60 to 100 mg/dOral prednisolon at 60 to 100 mg/dIntravemous methylprednisolone at 300 mg daily .Intravemous methylprednisolone at 300 mg daily .When the hematocrit stabilizes, prednisone may be tapered to 15 When the hematocrit stabilizes, prednisone may be tapered to 15 to 20 mg/d and continued for 2 to 3 month before tapering off the to 20 mg/d and continued for 2 to 3 month before tapering off the drugs entirely.drugs entirely.

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Cold-agglutinin-mediated hemolysis account for 10 to 20 percent of all Cold-agglutinin-mediated hemolysis account for 10 to 20 percent of all cases of autoimmune hemolytic anemia.cases of autoimmune hemolytic anemia.

Hemolysis is generally chronicHemolysis is generally chronicAcrocyanuosis is frequently observed.Acrocyanuosis is frequently observed.Anemia is usually mild to moderate.Anemia is usually mild to moderate.

Therapy :Therapy :Keeping the patient warm is importantKeeping the patient warm is importantChlorambucil and Cyclophosphamide are usefull for more severe, Chlorambucil and Cyclophosphamide are usefull for more severe,

chronic cases.chronic cases.Splenectomy ang glucocrticoid generally are not helpful , very high Splenectomy ang glucocrticoid generally are not helpful , very high

dose glucocorticoids may be useful in the ill.dose glucocorticoids may be useful in the ill.In critical ill patient, plasmapheresis may provide temporary relief.In critical ill patient, plasmapheresis may provide temporary relief.

Paroxysmal Cold hemoglubiuriaParoxysmal Cold hemoglubiuriaA very rare from of hemolytic anemia characterized by recurrent A very rare from of hemolytic anemia characterized by recurrent

massive hemolysis following exposure to cold.THE ENDmassive hemolysis following exposure to cold.THE END

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Thanks for your attention!!Thanks for your attention!!