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Blood loss Hemolysis (intrinsic) Membrane: hereditary spherocytosis, elliptocytosis Hemoglobin: sickle cell, unstable hemoglobin Glycolysis: pyruvate kinase deficiency, etc Oxidation: glucose-6-phosphate dehydrogenase deficiency Hemolysis (extrinsic) Immune: warm antibody, cold antibody Microangiopathic: thrombotic thrombocytopenic purpura, hemolyticuremic syndrome, mechanical cardiac valve, paravalvular leak Infection: clostridial Hypersplenism
Decreased production
Hemoglobin synthesis: iron deficiency, thalassemia, anemia of chronic disease DNA synthesis: megaloblastic anemia Stem cell: aplastic anemia, myeloproliferative leukemia Bone marrow infiltration: carcinoma, lymphoma Pure red cell aplasia
Classification of anemias by mean cell volume. Microcytic Iron deficiency Thalassemia Anemia of chronic disease
Macrocytic Megaloblastic Vitamin B12 deficiency Folate deficiency Nonmegaloblastic Myelodysplasia, chemotherapy Liver disease Increased reticulocytosis Myxedema Normocytic Many causes
THE THALASSEMIAS
Essentials of Diagnosis Microcytosis out of proportion to the degree of anemia. Positive family history or lifelong personal history of microcytic anemia. Abnormal red blood cell morphology with microcytes, acanthocytes, and target cells. In -thalassemia, elevated levels of hemoglobin A2 or F.
persons from southeast Asia and China, and, less commonly, in blacks. Three genes are present: the patient is a hematologically normal (silent carrier). Two a-globin genes are present: a-thalassemia trait. These patients are clinically normal with a mild microcytic anemia. Only one a-globin chain is present: hemoglobin H disease. This is a chronic hemolytic anemia of variable severity (thalassemia minor or intermedia). Physical examination: pallor and splenomegaly. Although affected individuals do not usually require transfusions, they may do so during periods of hemolytic exacerbation caused by infection or other stresses. All four a-globin genes are deleted, the affected fetus is stillborn as a result of hydrops fetalis.
(Italian, Greek) and to a lesser extent Chinese, other Asians, and blacks. Homozygous for -thalassemia have thalassemia major. Affected children are normal at birth but after 6 months, develop severe anemia requiring transfusion. Numerous clinical problems ensue, including growth failure, bony deformities (abnormal facial structure, pathologic fractures), hepatosplenomegaly, and jaundice. Hemosiderosis results in a clinical picture similar to hemochromatosis, with heart failure, cirrhosis, and endocrinopathies. A milder form of -thalassemia (allowing a higher rate of globin gene synthesis) have thalassemia intermedia: chronic hemolytic anemia but do not require transfusions except under periods of stress. They survive into adult life but with hepatosplenomegaly and bony deformities. Patients heterozygous for -thalassemia have thalassemia minor and a clinically insignificant microcytic anemia.
HEMOLYTIC ANEMIAS
Intrinsic
Membrane defects: hereditary spherocytosis, hereditary elliptocytosis,
paroxysmal nocturnal hemoglobinuria Glycolytic defects: pyruvate kinase deficiency, severe hypophosphatemia Oxidation vulnerability: glucose-6-phosphate dehydrogenase deficiency, methemoglobinemia Hemoglobinopathies: sickle cell syndromes, unstable hemoglobins, methemoglobinemia
Extrinsic
Immune: autoimmune, lymphoproliferative disease, drug toxicity Microangiopathic: thrombotic thrombocytopenic purpura, hemolytic-uremic
syndrome, disseminated intravascular coagulation, valve hemolysis, metastatic adenocarcinoma, vasculitis Infection: plasmodium, clostridium, borrelia Hypersplenism Burns
Kesimpulan
Anamnesis dan fisik diagnostik merupakan kunci
utama untuk menegakan diagnosis Anemia Lakukan anamnesis dan fisik diagnostik sesuai dengan patofisiologi dari anemia yang terjadi Lakukan selalu reanamnesis untuk mencari data baru berdasar data yang sudah ada Lakukan allo dan auto anamnesis untuk mendapat data yang valid. Pakailah bahasa komunikasi yang dimengerti oleh pasien.