dasar penyakit hematologi.pdf
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Dasar Penyakit Hematologi
Tulus Widiyanto, MD Optima Bandung-Jakarta
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Erytrocyte Disorders
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ANEMIA Erythropoiesis Process
� Hematopoiesis is the process by which the formed elements of the blood are produced.
� The physiologic regulator of red cell production, the glycoprotein hormone EPO, is produced and released by peritubular capillary lining cells within the kidney.
Tulus Widiyanto, MD
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ANEMIA Diagnostic Approach
� The evaluation of the patient with anemia requires a careful history and physical examination.
� Physical examination may demonstrate a forceful heartbeat, strong peripheral pulses, and a systolic “flow” murmur.
Tulus Widiyanto, MD
Classification: � Marrow production defects
(hypoproliferation) � Red cell maturation defects
(ineffective eryth ropoiesis) � Decreased red cell survival
(blood loss/hemolysis). 1. Reticulocyte production
index > 2.5; hemolysis 2. A reticulocyte production
index < 2; hypoproliferative anemia or maturation disorder.
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ANEMIA Diagnostic Approach
Tulus Widiyanto, MD
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ANEMIA; Iron Deficiency Anemia
Iron Cycle Nutritional Requirement � The balance of iron
metabolism in the or- ganism is tightly controlled and designed to conserve iron for reutilization.
� Iron comes into the body is via absorption from food (dietary iron intake) or from medicinal iron taken orally.
Tulus Widiyanto, MD
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ANEMIA; Iron Deficiency Anemia
Stages of Iron Deficiency
Tulus Widiyanto, MD
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ANEMIA; Iron Deficiency Anemia
Diagnostic Approach
Treatment � A dose of 200 to 300 mg of elemental iron per day should result in the absorption of iron up to 50 mg/d.
� Typically, the reticulocyte count should begin to increase within 4 to 7 days after initiation of therapy and peak at 1½ weeks.
Tulus Widiyanto, MD
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ANEMIA; Megaloblastic Anemia
Definition Pathophysiology � The megaloblastic
anemias are disorders caused by impaired DNA synthesis.
� Most megaloblastic anemias are due to a deficiency of cobalamin (vitamin B12) and/or folic acid.
Tulus Widiyanto, MD
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ANEMIA; Megaloblastic Anemia
Diagnostic Approach
Laboratory � If the macrocytosis is marked (MCV > 110 fL), the patient is much more likely to have a megaloblastic anemia.
� Enhanced intramedullary destruction of erythroblasts results in an in- crease in unconjugated bilirubin and lactic acid dehydrogenase
Tulus Widiyanto, MD
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ANEMIA Megaloblastic Anemia
Cobalamine deficiency: � Oral replacement therapy with
2 mg crystalline B12 per day.
� Parenteral treatment begins with 1000 μg cobalamin per week for 8 weeks, followed by 1000 μg cyanocobala- min intramuscularly every month for the rest of the patient’s life.
Tulus Widiyanto, MD
Folate deficiency: � Oral folic acid
supplementation; the usual dose of folic acid is 1 mg/d.
� Higher dose can be given for malabsorbtion.
� Definitive treatment
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Thalassemia Hemoglobin chain
� The thalassemia syndromes are inherited disorders of α- or β-globin biosynthesis.
� Unbalanced accumulation of α and βsubunits occurs because the synthesis of the unaffected globin proceeds at a normal rate.
� Clinical severity varies widely.
Tulus Widiyanto, MD
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THALLASAEMIA Clinical Manifestation
� Hypochromia and microcytosis.
� Shortening the RBC life span.
� Massive bone marrow expansion.
� Hemolytic anemia causes hepatosplenomegaly, leg ulcers, gallstones, and high-output congestive heart failure
Tulus Widiyanto, MD
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THALLASAEMIA Workup & Treatment
Workup � CBC; Anemia � Peripheral blood
smear; hypochromasia and microcytosis, hypochromic macrocytes that represent the polychromatophilic cells, nucleated RBCs, basophilic stippling, and occasional immature leukocytes
� HB electroforesis
Tatalaksana � Blood transfusion � Iron-chelating
therapy � Hematopoietic
stem cell transplantation (HSCT)
Tulus Widiyanto, MD
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Leucocyte Disorders
Tulus Widiyanto, MD
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Myeloproliferation
Tulus Widiyanto, MD
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Acute Myelogenic Leukemia � A group of diseases
characterized by infiltration of the blood, bone marrow, and other tissues by neoplastic cells of the hematopoietic system.
� Etiology; Heredity, radiation, drugs, chemical exposure
� Symptoms resulting from bone marrow failure, symptoms resulting from organ infiltration with leukemic cells, or both.
Tulus Widiyanto, MD
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Acute Myelogenic Leukemia Workup & Treatment
Workup � Anemia and
thrombocytopenia � presence of greater than
20% blasts in the marrow
Tatalaksana � Anthracycline
(idarubicin or daunorubicin) or anthracenedione (mitoxantrone), combined with cytarabine
� Blood transfusion � Antibiotics � Growth factor (G-CSF)
Tulus Widiyanto, MD
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Acute Lymphocytic Leukemia � A malignant (clonal) disease of
the bone marrow in which early lymphoid precursors proliferate and replace the normal hematopoietic cells of the marrow.
� Symptoms; direct infiltration of the marrow, decrease of marrow elements.
� Palpable lymphadenopathy � Splenomegaly
Tulus Widiyanto, MD
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Acute Lymphocytic Leukemia Workup & Treatment
Workup � Anemia,
thrombocytopenia, neutropenia
� Presence of greater than 20% lymphoblastblasts in the marrow
� myeloperoxidase (MPO) (or Sudan black) and terminal deoxynucleotidyl transferase (TdT)
Tatalaksana � vincristine, prednisone,
anthracycline, and cyclophosphamide.
� Transplantation; Allogenic, stem cell
Tulus Widiyanto, MD
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Trombocyte Disorders
Tulus Widiyanto, MD
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Hemofilia Definition & Pathophysiology
Definition Pathophysiology � Hemofilia A:
Deficiency FVIII � Hemofilia B;
Deficiency FIX � History of
hemorrhage disproportionate to trauma, spontaneous hemorrhage, bleeding disorders in the family, concomitant illness
Tulus Widiyanto, MD
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HEMOFILIA Workup & Treatment
Workup
� Complete blood cell count
� Coagulation studies (aPTT)
� Factor VIII, IX, von willebrand assay
Treatment
� Cryoprecipitates infusion
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Immune Trombocytopenic Purpura
Definition Pathophysiology � Clinical syndrome in which
a decreased number of circulating platelets (thrombocytopenia) manifests as a bleeding tendency.
� Splenic sequestration and phagocytosis by mononuclear macrophages
Tulus Widiyanto, MD
� Acute & Chronic ITP
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Immune Trombocytopenic Purpura
Workup Treatment � Isolated thrombocytopenia � Assays for platelet antigen–
specific antibodies � ANA test
Tulus Widiyanto, MD
� ITP has no cure, and relapses may occur years after seemingly successful medical or surgical management
� Prevent remission; Cotricosteroids; Immunoglobulins