TUGAS PENDAHULUAN

HEMOLISIS (INTRAVASKULAR DAN EKSTRAVASKULAR)

Riskullah Makmur

Pembimbing I : dr. Fierna Darmawanti Hanafi

Pembimbing II : dr. Darwati Muhadi, Sp.PK(K)

PROGRAM STUDI ILMU PATOLOGI KLINIK

PROGRAM PENDIDIKAN DOKTER SPESIALIS
FAKULTAS KEDOKTERAN UNIVERSITAS HASANUDDIN / RSUP DR. WAHIDIN SUDIROHUSODO
 PENDAHULUAN
• Hemolisis = Pemecahan/penghancuran eritrosit secara abnormal -> Hb
pembawa O2 dibebaskan ke plasma.
• Hemolisis terbagi dua, yaitu :
1. Hemolisis intravaskular = didalam pembuluh darah
2. Hemolisis ekstravaskular = didalam organ seperti hepar dan lien

1. Siddon AJ, Tormey CA. The chemical and laboratory investigation of hemolysis. In: Advances in Clinical Chemistry. Academic Press Inc.; 2019. p. 215–58.
2. Hendrickson JE, Tormey CA. The RBC as a Target of Damage. In: Pathobiology of Human Disease. Elsevier; 2016.
3. Barcellini W, Fattizzo B. Clinical Applications of Hemolytic Markers in the Differential Diagnosis and Management of Hemolytic Anemia. Vol. 2015, Disease Markers. Hindawi Limited; 2015. 3
4. Jameson et al. Harrison’s, Principles of Internal Medicine. 20 ed. Boston; The McGraw-Hill Companies; 2018.
 PATOFISOLOGI HEMOLISIS INTRAVASKULAR
Aktivasi Gangguan
Komplemen Mekanik Membran /
Jalur Klasik Hemoglobin

Sferositosis
Opsonisasi Makroangiopati
Herediter

Anafilatoksin Mikroangiopati Luka Bakar

Aktivasi Anemia Sickle

Komplemen Cell

Defisiensi G6PD

6. Risitano AM, et al. Anti-complement Treatment for Paroxysmal Nocturnal Hemoglobinuria: Time for Proximal Complement Inhibition? A Position Paper From the SAAWP of the EBMT. J Front. Immunol.
2019; 10(1157). Diakses di : https://www.frontiersin.org/article/10.3389/fimmu.2019.01157 4
 PATOFISOLOGI HEMOLISIS INTRAVASKULAR

5. Hermening, Denise. Modern Blood Banking and Transfusion Practices, 7th edition. In Hartwell, B (Eds) Apheresis. Philadelphia; F.A. Davis Company; 2019. 5
 PATOFISOLOGI HEMOLISIS EKSTRAVASKULAR
• Mekanisme destruksi eritrosit dimediasi oleh makrofag jaringan dalam Reticuloenothelial System
(RES) / Eritrocyte Phagocytosis (EP)
• EP -> pengenalan dan engulfment eritrosit tua / rusak oleh makrofag dalam limfa, liver (sel Kupffer)
dan sumsum tulang -> dicetuskan eryptosis
• Klirens eritrosit -> 2 proses komponen (retensi mekanik dan deformabilitas eritrosit) -> penentuan
eritrosit disingkirkan dari sirkulasi / tidak

13. Turpin C, et al. Erythrocytes: Central Actors in Multiple Scenes of Atherosclerosis. International Journal of Molecular Sciences. 29 Mei 2021;22:5843.
14. Li H, et al. How the spleen reshapes and retains young and old red blood cells: A computational investigation. PLOS Computational Biology. 1 November 2021;17(11):e1009516.
6
16. Klei TRL, et al. From the Cradle to the Grave: The Role of Macrophages in Erythropoiesis and Erythrophagocytosis. Frontiers in Immunology [Internet]. 2017 [dikutip 14 Maret 2022];8. Diakses di :
https://www.frontiersin.org/article/10.3389/fimmu.2017.00073
 PATOFISOLOGI HEMOLISIS EKSTRAVASKULAR

13. Turpin C, et al. Erythrocytes: Central Actors in Multiple Scenes of Atherosclerosis. International Journal of Molecular Sciences. 29 Mei 2021;22:5843.
14. Li H, et al. How the spleen reshapes and retains young and old red blood cells: A computational investigation. PLOS Computational Biology. 1 November 2021;17(11):e1009516.
7
16. Klei TRL, et al. From the Cradle to the Grave: The Role of Macrophages in Erythropoiesis and Erythrophagocytosis. Frontiers in Immunology [Internet]. 2017 [dikutip 14 Maret 2022];8. Diakses di :
https://www.frontiersin.org/article/10.3389/fimmu.2017.00073
 MANIFESTASI KLINIS HEMOLISIS INTRAVASKULAR
• ↑ LDH
• ↑ Bil. T
• ↓ Hp
• ↑ Hemoglobinuria
• ↑ Hemosidenuria

1. Siddon AJ, Tormey CA. The chemical and laboratory investigation of hemolysis. In: Advances in Clinical Chemistry. Academic Press Inc.; 2019. p. 215–58. 8
 MANIFESTASI KLINIS HEMOLISIS EKSTRAVASKULAR

Ikterus, Hepatosplenomegali, Limfadenopati : Riw. Anemia Hemolitik : SCA, SH, Thalassemia, An.
Umum Def. G6PD

Diare : HUS Riw. Kanker : AIHA Warm

Riw. Infeksi Mononukleosis / M. Pneumoniae :

Demam : AIHA, DIC, HUS, infeksi
AIHA Cold

Hematuria : PNH Riw. Transfusi : Reaksi Transfusi hemolitik

Obat-obatan : Anemia Mikroangiopatik Trombotik /

Ikterus : Anemia Hemolitik
Hemolitik, Anemia Def. G6PD

3. Barcellini W, Fattizzo B. Clinical Applications of Hemolytic Markers in the Differential Diagnosis and Management of Hemolytic Anemia. Vol. 2015, Disease Markers. Hindawi Limited; 2015.
8. Jokiranta TS. HUS and atypical HUS. Blood. 2017 May;129(21):2847–56. 9
 PENUTUP
• Hemolisis dapat terjadi akibat hemolisis ekstravaskular bersamaan dengan hemolitik
intravaskular.
• Hemolisis intravaskular adalah lisisnya eritrosit di dalam pembuluh darah.
• Mekanisme utama hemolisis intravaskular adalah aktivasi komplemen jalur klasik,
mekanik dan gangguan membran/hemoglobin.
• Manifestasi dari hemolisis intravaskular adalah peningkatan kadar bilirubin tak
terkonjugasi/total, peningkatan LDH dan hemosiderinuria.
• Hemolisis ekstravaskular adalah lisisnya eritrosit di dalam organ (hepar dan lien).
• Mekanisme utama hemolisis ekstrvasakular adalah melalui sekuestrasi dan
fagositosis eritrosit oleh makrofag dalam limpa, liver dan sumsum tulang akibat
deformabilitas yang buruk.
• Manifestasi hemolisis ekstravaskular dapat berupa limfadenopati,
hepatosplenomegali, kolestasis, dan koledokolithiasis.

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TERIMA KASIH