APLASTIC ANEMIA Divisi Hemato-Onkologi Bagian Ilmu Kesehatan Anak Universitas Sumatera Utara

Inherited bone marrow failure syndrome  Pancytopenia fanconi’s anemia dyskeratosis congenita amegakaryocytic anemia other genetic syndrome  unilineaqe cytopenia Diamond- Blackfan anemia Kostman’s syndrome Thrombocytopenia with absent radii

CONSTITUSIONAL APLASTIC ANEMIA DIAGNOSE Thrombocytopenia or neutropenia progressing to pancytopenia Macrocytosis Multiple congenital anomalies Increased chromosome breakage in peripheral blood lympocytes

Important single cytopenias Red cell aplasia congenital hypoplastic anemia (Diamond- Blackfan anemia) Transient erythroblastopenia of childhood Transient aplasia with chronic hemolysis Neutropenia Kostmann syndrome shwachman-Diamond syndrome Cyclic neutropenia Thrombocytopenia Thrombocytopenia with absent radii Amegakaryocytic thrombocytopenia

Laboratory findings Thrombocytopenia/ leucopenia Macrocytosis BMP  hipoplasia or aplasia

treatment supportive care Parentral broad spectrum antibiotics transfusion Bone marrow transplantation

ACQUIRED APLASTIC ANEMIA The International Agranulocytosis and Aplastic Anemia Study (IAAAS) Aplastic Anemia:  Hemoglobin ≤ 100 gr/dl or hematocrit ≤ 0,30  Platelet count ≤ 50x10 9/l  White blood cell count ≤3,5x10 9 /l or granulocyt ≤1,5x10 9 /l

DIAGNOSE Weakness and pallor Petechie purpura and bleeding Frequent or severe infection Pancytopenia or cellular bone marrow

Lab. findings Bone marrow biopsy  decrease in cellularity and the absence of significant fibrosis or neoplastic infiltration Severe aplastic anemia defined as < 25% moderate (25-50% with < 30% of cells being hematopoietic)

etiology Viral infection, notably hepatitis Drugs and toxin Immune disorders Thymoma

PATOPHYSIOLOGY The phenotype of aplastic anemia reflects the ultimate failure of hematopoietic progenitors to differentiated into erythroid, megacaryocytic or myeloid elements Failure of the stem cell seems more probable, as borne out by study have demonstrated reduce numbers of CD34 + peripheral blood and marrows cell

TREATMENT supportive care antibiotics red blood cell transplantation bone marrow transplantation

Treatment Supportive modalities should be used to treat infection and to prevent bleeding Use of blood products must be judicious to minimize donor For CMV- negative recipients, the blood products should either be CMV negative or filtered Whenever possible, single donor platelets should be used as opposed to pooled randomdonor product HLA typing of the patient and family should be done on an urgent basis to facilitate early BMT or the searching of unrelated donor registries