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  • Anemia Sideroblastik: Patogenesis, Diagnosis, dan Tatalaksana

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    ariel valentino
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    1. Sideroblastic anemia is caused by a mutation in the ALAS gene, resulting in a defect in 5-aminolevulinic acid synthetase and abnormal iron deposition in red blood cell precursors called sideroblasts. 2. Diagnosis involves identifying increased serum ferritin and iron levels, increased transferrin saturation, and microscopic examination of bone marrow showing ringed sideroblasts. Treatment includes pyridoxine supplementation, iron chelation to prevent overload, and stem cell transplant in severe cases. 3. Anemia associated with chronic diseases is caused by increased inflammatory cytokines like IL-6 and IFN-γ, which induce hepcidin production, impair iron transport

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    Pleno1_DDx Anemia Mikrositik Part II

    Original Title

    Pleno1_DDx Anemia Mikrositik Part II

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    1. Sideroblastic anemia is caused by a mutation in the ALAS gene, resulting in a defect in 5-aminolevulinic acid synthetase and abnormal iron deposition in red blood cell precursors called sideroblasts. 2. Diagnosis involves identifying increased serum ferritin and iron levels, increased transferrin saturation, and microscopic examination of bone marrow showing ringed sideroblasts. Treatment includes pyridoxine supplementation, iron chelation to prevent overload, and stem cell transplant in severe cases. 3. Anemia associated with chronic diseases is caused by increased inflammatory cytokines like IL-6 and IFN-γ, which induce hepcidin production, impair iron transport

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    © All Rights Reserved
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    94 views6 pages

    Anemia Sideroblastik: Patogenesis, Diagnosis, dan Tatalaksana

    Uploaded by

    ariel valentino
    1. Sideroblastic anemia is caused by a mutation in the ALAS gene, resulting in a defect in 5-aminolevulinic acid synthetase and abnormal iron deposition in red blood cell precursors called sideroblasts. 2. Diagnosis involves identifying increased serum ferritin and iron levels, increased transferrin saturation, and microscopic examination of bone marrow showing ringed sideroblasts. Treatment includes pyridoxine supplementation, iron chelation to prevent overload, and stem cell transplant in severe cases. 3. Anemia associated with chronic diseases is caused by increased inflammatory cytokines like IL-6 and IFN-γ, which induce hepcidin production, impair iron transport

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
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    of 6

    Anemia Sideroblastik

    Sills R. Other microcytic anemias. In: Nelson textbook of


    paediatrics. 20th ed. Philadelphia: Elsevier; 2016. Chapter 456,
    • Fenomena sideroblas bercincin Other microcytic anemias; p. 2326-2327

    • Pewarnaan Prussian Blue (reaksi


    Perls)
    • Mikrositik – hipokrom

    • Kongenital  mutasi, x-linked


    • Didapat  timbal (Pb), isoniazid,
    defisiensi Cu, kemoterapi
    Anemia Sideroblastik: Patogenesis
    • Kongenital  X-linked
    • Mutasi gen ALAS  defek
    isozim 5-aminolevulinat
    sintetase

    • Manifestasi  pucat, takipnea,


    palpitasi, pusing,
    hepatomegaly, splenomegaly
    • Beberapa kasus  bronze-
    colored skin

    Cazzola M, Malcovati L. Diagnosis and treatment of sideroblastic anemias: from defective heme
    synthesis to abnormal RNA splicing. Hematology. 2015 Dec 1;2015(1):19–25.
    Anemia Sideroblastik: Diagnosis, Tatalaksana,
    Komplikasi
    Diagnosis Tatalaksana Komplikasi

    • Peningkatan ferritin serum • Piridoksin oral (50- • Iron overload:


    • Peningkatan Fe serum 100mg/hari) • Diabetes mellitus
    • Peningkatan saturasi Fe- • Pencegahan iron overload • Disfungsi hepar
    transferrin  deferoxamin
    • Darah lengkap  MCV dan • Nutrisi tembaga untuk
    MCH rendah  mikrosit defisiensi Cu
    • Analisis sumsum tulang  • Transplantasi stem cell 
    rule out myelodisplasia anemia sideroblastik berat
    • Reaksi Perls  sideroblas
    bercincin • Penelitian  TGF-β
    inhibitor
    Anemia akibat Penyakit Kronik: Etiologi dan
    Patogenesis
    • Osteomyelitis Kronik Peningkatan sitokin pro-
    Infeksi • Endokarditis Bakterialis Inflamasi Sistemik inflamasi

    Kronik • Abses Paru


    IL-1, IL-6, TNF, IFN

    IL-6 : Peningkatan hepcidin IFN-γ : Inhibisi eritropoietin


    Autoimun • Rheumatoid arthritis dan downregulasi
    ferroportin  gangguan IL-1 : Peningkatan destruksi
    transport eritrosit oleh makrofag

    • Limfoma Hodgkin
    Keganasan • Karsinoma paru Anemia
    • Karsinoma payudara
    Anemia akibat Penyakit Kronik: Diagnosis dan
    Tatalaksana
    • Definitif  diterapi penyakit
    kroniknya 
    • Administrasi zat besi dan
    eritropoietin
    • Erithropoietin stimulating
    agents (ESA)
    • Kombinasi ESA-besi  optimal

    • Dosis tinggi ESA  stroke,


    penyakit KV
    Referensi
    1. Sills R. Other microcytic anemias. In: Nelson textbook of paediatrics. 20th ed.
    Philadelphia: Elsevier; 2016. Chapter 456, Other microcytic anemias; p. 2326-2327.
    2. Ashorobi D, Chhabra A. Sideroblastic anemia. In: StatPearls [Internet]. Treasure Island
    (FL): StatPearls Publishing; 2019 Jan- [updated 2019 Feb 24; cited 2019 April 24].
    Available from: https://www.ncbi.nlm.nih.gov/books/NBK538287/
    3. Cazzola M, Malcovati L. Diagnosis and treatment of sideroblastic anemias: from
    defective heme synthesis to abnormal RNA splicing. Hematology. 2015 Dec
    1;2015(1):19–25.
    4. Kumar V, Abbas AK, Aster JC. Robbins basic pathology. 9th ed. Philadelphia: Elsevier;
    2013. Chapter 11, Hematopoietic & lymphoid systems; p. 421.
    5. Madu AJ, Ughasoro MD. Anaemia of chronic disease: An in-depth review. Med Princ
    Pract. 2016;26(1):1–9.
    6. Gangat N, Wolanskyj AP. Anemia of chronic disease. Semin Hematol. 2013
    Jul;50(3):232–8.

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