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APLASTIK ANEMI

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APLASTIK ANEMI

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    8. Akut Lsemi

    10. MPH

    12. Aplastik Anemi

    15. Insidans: 2/milyon/yil

    17. Figure 3. Antigenic Stimuli Initiating the Destruction of Bone Marrow. A virus (Panel A) may infect hematopoietic cells (or hepatocytes in hepatitis), leading to the production of novel viral proteins and the excessive or aberrant production of normal cellular proteins, which are released and then taken up by antigen-presenting cells, in which the proteins are processed to become peptides, form complexes with major-histocompatibility-complex molecules, and are presented to naive T cells. The immune response results in the destruction of infected cells. In rare cases, the lymphocyte response persists and affects normal cells, resulting in autoimmune organ destruction. A similar process may occur in patients with drug-induced aplastic anemia (Panel B). Reactive drug metabolites, formed in marrow cells, may bind to cellular proteins, leading the immune system to recognize them as foreign, thereby initiating the sequence shown in Panel A. A generic substituted hydrocarbon is symbolized by a benzene ring with an R moiety. Presentation of an altered or aberrantly expressed protein -- for example, a product of a chromosomal translocation or an oncogene (Panel C) -- may similarly initiate a T-cell response that broadens to include nonmalignant cells, leading to marrow hypocellularity in myelodysplasia through a similar sequence.Figure 3. Antigenic Stimuli Initiating the Destruction of Bone Marrow. A virus (Panel A) may infect hematopoietic cells (or hepatocytes in hepatitis), leading to the production of novel viral proteins and the excessive or aberrant production of normal cellular proteins, which are released and then taken up by antigen-presenting cells, in which the proteins are processed to become peptides, form complexes with major-histocompatibility-complex molecules, and are presented to naive T cells. The immune response results in the destruction of infected cells. In rare cases, the lymphocyte response persists and affects normal cells, resulting in autoimmune organ destruction. A similar process may occur in patients with drug-induced aplastic anemia (Panel B). Reactive drug metabolites, formed in marrow cells, may bind to cellular proteins, leading the immune system to recognize them as foreign, thereby initiating the sequence shown in Panel A. A generic substituted hydrocarbon is symbolized by a benzene ring with an R moiety. Presentation of an altered or aberrantly expressed protein -- for example, a product of a chromosomal translocation or an oncogene (Panel C) -- may similarly initiate a T-cell response that broadens to include nonmalignant cells, leading to marrow hypocellularity in myelodysplasia through a similar sequence.

    20. Figure. Relationship of PNH to Aplastic Anemia and Myelodysplasia. An unknown mechanism, probably an environmental toxin, damages hematopoietic stem cells and induces a mutation of PIG-A. The type and extent of damage determine whether aplastic anemia, PNH, or a myelodysplastic syndrome results.Figure. Relationship of PNH to Aplastic Anemia and Myelodysplasia. An unknown mechanism, probably an environmental toxin, damages hematopoietic stem cells and induces a mutation of PIG-A. The type and extent of damage determine whether aplastic anemia, PNH, or a myelodysplastic syndrome results.

    24.

    32. Kanama egilimi

    33. Infeksiyon egilimi

    34. Solukluk

    35. Fanconi Aplastik Anemisi APLASTIK ANEMI SOMATIK BOZUKLUKLAR Byme geriligi Hiperpigmentasyon Iskelet bozukluklari Kalp-bbrek-gz defektleri Mikrosefali Hipogonadizm ARTMIS KANSER RISKI

    36. Hb< 10 g/dL Ntrofil< 1500/mL Trombosit: 50 000/mL

    37. KLASIK ASOSIYASYONLAR TA-GVHD Hemofagositik Sendrom/Aplazi Hepatitis/Aplazi

    38. APLASTIK ANEMI SIDDETININ TANIMLANMASI

    50. Figure 1. Cumulative Incidence of Observed Cancers in 860 Patients with Aplastic Anemia Who Received Immunosuppressive Therapy and 748 Patients Who Underwent Bone Marrow Transplantation. MDS denotes myelodysplastic syndrome, AL acute leukemia, and ST malignant solid tumors.Figure 1. Cumulative Incidence of Observed Cancers in 860 Patients with Aplastic Anemia Who Received Immunosuppressive Therapy and 748 Patients Who Underwent Bone Marrow Transplantation. MDS denotes myelodysplastic syndrome, AL acute leukemia, and ST malignant solid tumors.

    60. IS TEDAVIYE YANIT KRITERLERI

    62. TRANSPLANTASYON ve IST NCESI

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