Downloaded 16 times
Uploaded byAskin Kaplan
Hemolytic anemia akk
This document provides an overview of hemolytic anemia, including definitions, classifications, causes, signs and symptoms, and diagnostic tests. It discusses extravascular and intravascular hemolysis, inherited and acquired hemolytic anemias, specific conditions like glucose-6-phosphate dehydrogenase deficiency, autoimmune hemolytic anemia, microangiopathic hemolytic anemia, and paroxysmal nocturnal hemoglobinuria. Physical exam findings of anemia and hemolysis are outlined. Diagnostic tests for hemolytic anemia including blood counts, chemistries, peripheral smear, Coombs test, and hemoglobin electrophoresis are also summarized.
More Related Content
Similar to Hemolytic anemia akk
Hemolytic anemia akk
- 1. AşkınK.KaplanM.D. Hemolytic Anemia Asst. Prof.Aşkın K. Kaplan M.D. Maltepe University Maltepe Faculty of Medicine
- 2. AşkınK.KaplanM.D. RED BLOOD CELLLIFE CYCLE
- 3.
- 4.
- 5. AşkınK.KaplanM.D. Anemia- Clinical History •Symptoms acute vs chronic • Duration of symptoms • Previous blood counts • Previous diagnosis of anemia • History of treatment with iron, folate, or cobalamin • Familial history of anemia • Gastrointestinal system symptoms • Nutrition status • Medications
- 6. AşkınK.KaplanM.D. Anemia- Clinical History •Consumption of alcohol • Menstruation and pregnancy • Jaundice and dark urine • Loss of weight • Fever, night sweats • Abdominal discomfort • Stomatitis, glossitis • Paresthesia, weakness
- 7. AşkınK.KaplanM.D. Anemia- Physical Examination •The major aim on physical examination is – to find signs of organ or multisystem involvement – to assess the severity of the patient's condition. • Pallor • Jaundice • The presence or absence of lymphadenopathy, hepatomegaly, splenomegaly, and bone tenderness, leg ulcers, coilonichia • Signs of other hematologic abnormalities – petechiae due to thrombocytopenia – ecchymoses, and other signs of bleeding due to abnormalities of coagulation. – signs and symptoms of recurrent infections secondary to neutropenia or immune deficiency states. • The presence of occult blood
- 8. AşkınK.KaplanM.D. Source: Overview ofthe Anemias, Pathophysiology of Blood Disorders, 2e Citation: Aster JC, Bunn H. Pathophysiology of Blood Disorders, 2e; 2017 Available at: http://accessmedicine.mhmedical.com/content.aspx?bookid=1900§ionid=137394689 Accessed: March 02, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved
- 9. AşkınK.KaplanM.D. Classification of Anemia •Kinetic approach – Decreased RBC production – Increased RBC destruction (hemolysis) – Blood loss; bleeding • Morphologic – According to RBC size (MCV) • Macrocytic anemia • Microcytic anemia • Normocytic anemia – According to reticulocyte count • Hyporegenerative • Regenerative
- 10. AşkınK.KaplanM.D. Kinetic Approach Increased RBCDestruction- Hemolysis • Extravascular hemolysis – Intrinsic red blood cell defects – Extrinsic red blood cell defects • Intravascular hemolysis OR • Inherited hemolytic anemias • Acquired hemolytic anemias
- 11.
- 12. AşkınK.KaplanM.D. Increased RBC Destruction ExtravascularRBC Destruction Intrinsic RBC Defects • Hemoglobinopathies – Thalassemias – Sickle cell anemia – Unstable Hb variants • RBC membrane disorders – Hereditary spherocytosis – Hereditary elliptocytosis • RBC enzyme deficiencies – G6PD deficiency – Pyruvate kinase deficiency Extrinsic RBC Defects • Autoimmune hemolytic anemia (warm or cold reacting, drugs) • Liver disease • Hypersplenism • Infections (malaria, bartonella) • Oxidant agents (drugs) • Other agents (lead, copper, snake and spider bites) • Large granular lymphocyte leukemia • Intravenous immune globulin infusion
- 13. AşkınK.KaplanM.D. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency •G-6-PD: reduces NADP/oxidizes glucose-6- phosphate – Detoxifies free radicals and peroxides
- 14. AşkınK.KaplanM.D. Glucose-6-Phosphate Dehydrogenase Deficiency • Sex-linkeddisorder • 8% of African-American males • Hemolytic anemia usually occurs in the presence of stress (infection or drugs) – African form - mild hemolysis usually with drugs, infection, fever – Mediterranean form - more severe • Unique sensitivity to fava beans – Chronic non-spherocytic hemolytic anemia
- 15. AşkınK.KaplanM.D. Increased RBC Destruction IntravascularRBC Destruction • Microangiopathy – Aortic stenosis, prosthetic valve leak • Transfusion reactions – ABO incompatibility • Infection – Sepsis, severe malaria • Paroxysmal cold hemoglobinuria • Cold agglutinin disease • Paroxysmal nocturnal hemoglobinuria • Following infusion of Rho(D) immune globulin • Snake bites • Exposure to compounds with high oxidant potential – Copper in Wilson’s disease
- 16. AşkınK.KaplanM.D. Increased RBC Destruction HereditaryHemolytic Diseases • Red cell membrane disorders – Hereditary spherocytosis – Hereditary elliptocytosis – Hereditary pyropoikilocytosis – Hereditary stomatocytosis • Enzyme deficiencies – Hexose monophosphate shunt enzyme deficiencies • G6PD deficiency, glutathione synthetase deficiency – Glycolytic enzyme deficiencies • Pyruvate kinase deficiency, hexokinase deficiency • Globin chain abnormalities – Deficient globin synthesis; thalassemia – Structurally abnormal globins; sickle cell disease, unstable hemoglobins
- 17. AşkınK.KaplanM.D. Acquired Hemolytic Diseases •Deficiency of phosphatidylinositol- linked glycoproteins – Paroxysmal nocturnal hemoglobinuria (PNH) • Antibody mediated destruction; Coombs positive AIHA – Warm Ab, Cold Ab, paroxysmal cold hemoglobinuria • Mechanical trauma – Microangiopathic hemolytic anemias – Cardiac traumatic hemolysis – Repetitive physical trauma • Infections of red blood cells; Malaria • Toxic or chemical injury – Sepsis, snake venom, lead poisoning • Membrane lipid abnormalities; abetalipoproteinemia • Sequestration; hypersplenism
- 18. AşkınK.KaplanM.D. Hemolytic Anemia withExtravascular Hemolysis • Extravascular (reticuloendothelial system) – Hereditary • Hemoglobinopathies (sickle cell anemia) • Enzymopathies (G6PD deficiency) • Membrane defects (hereditary spherocytosis) – Acquired • Immune mediated – Autoimmune hemolytic anemia • Non-immune mediated – Spur cell hemolytic anemia – Paroxysmal nocturnal hemoglobinuria (PNH)
- 19. AşkınK.KaplanM.D. Acquired Hemolytic Diseases •Immune mediated hemolytic anemia • Non-immune mediated
- 20. AşkınK.KaplanM.D. Autoimmune Hemolytic Anemia •Warm antibodies (IgG-mediated) – Primary 45% – Secondary 40% • Lymphoproliferative disease • Connective tissue disease • Infectious disease – Drug-induced 15% • Laboratory testing – Normocytic/macrocytic anemia – Peripheral smear: spherocytosis
- 21. AşkınK.KaplanM.D. Anti-Globulin (Coombs) Testing Directantiglobulin testing Indirect antiglobulin testing Patients RBCs Patients serum Anti-C3d Anti-IgG + RBCs + Anti-IgG +
- 22.
- 23. AşkınK.KaplanM.D. Acquired Hemolytic Anemia withIntravascular Hemolysis • Mechanical damage (microangiopathy) • Chemical damage • Infection • ABO incompatibility
- 24. AşkınK.KaplanM.D. Non-immune hemolytic anemia MicroangiopathicHemolytic Anemia • Thrombotic thrombocytopenic purpura (TTP) • Hemolytic uremic syndrome (HUS) • Disseminated intravascular coagulation (DIC) • Vasculitis • Malignant hypertension • Metastatic neoplasm with vascular invasion • Preeclampsia/HELLP syndrome of pregnancy
- 25.
- 26. AşkınK.KaplanM.D. Hemolytic Anemia- Diagnostictests • Reticulocyte count is elevated • Peripheral blood smear is helpful • Blood chemistries – Increase in unconjugated bilirubin – Increase in lactate dehydrogenase – Decrease in haptoglobin • Spesific tests – Coombs test; immune hemolytic anemia – Paroxysmal nocturnal hemoglobinuria screening – Hemoglobin electrophoresis – Glucose-6-phosphate dehydrogenase levels – Intravascular hemolysis;hemoglobinuria, hemosiderinuria
- 27.
- 28. AşkınK.KaplanM.D. Reticulocytosis • Reticulocyte countis elevated – Anemia + reticulocytosis ≥ 5% strongly suggests the presence of hemolysis – Other causes of reticulocytosis • During the first 2 weeks of replacement therapy with vitamin B12 or folic acid • Acute hypoxia • Infiltrative bone marrow disorders
- 29. AşkınK.KaplanM.D. Autoimmune Hemolytic Anemia •Autoantibodies reacts with erythrocytes. – Warm antibody AIHA (≥ 370 C), Ig G antibodies – Cold antibody AIHA (< 370 C), Ig M and compleman antibodies – Paroxysmal cold hemoglobinuria, Donath Landsteiner autoantibody
- 30. AşkınK.KaplanM.D. Microangiopathic Hemolytic Anemia •Disseminated intravascular coagulation (DIC) • Thrombotic thrombocytopenic purpura (TTP) • Hemolytic uremic syndrome (HUS) Schistocyte
- 31. AşkınK.KaplanM.D. Morphologic Approach Red BloodCell Size • Microcytic; MCV < 80 fl • Normocytic; MCV 80- 96 fl • Macrocytic; MCV > 100 fl
- 32. AşkınK.KaplanM.D. Morphologic Classification ofAnemia Microcytic • Reduced iron availability • Iron deficiency anemia • Copper deficiency • Anemia of chronic disease • Reduced globin production • Thalassemia • Hemoglobinopathies • Reduced heme synthesis • Sideroblastic anemia • Lead poisining Normocytic • Acute post hemorrhagic anemia • Aplastic anemia • Pure red cell aplasia • Bone marrow infiltration • Bone marrow suppression • Hemolytic anemia • Systemic diseases Macrocytic • Megaloblastic • Cobalamin deficiency • Folic acid deficiency • Drug induced (hydroxyurea, methotrexate) • Non-megaloblastic • Hemolytic anemia • Liver disease • Hypothyroidism • Myelodysplastic syndrome
- 33.
- 34. AşkınK.KaplanM.D. Hemolytic Anemia General examinationJaundice, pallor Physical examination Spleen may be enlarged Liver may be enlarged Gallstones Discoloration of the urine Skeletal changes in congenital forms Reticulocytes Increased Bilirubin Increased (unconjugated) LDH Increased Haptoglobulin Decreased Hemoglobinuria Hemosiderinuria Increased in intravascular hemolysis Aşkın K. KAPLAN M.D.
- 35. AşkınK.KaplanM.D. Symptoms and Signs-PNH • Hemolysis – Intravascular hemolysis of variable severity • Hypercoagulable state – increase in venous thrombosis in the hepatic, other intra-abdominal, and peripheral veins • Impaired hematopoiesis • Predisposition to infections Aşkın K. KAPLAN M.D. PNH:Paroxysmal nocturnal hemoglobinuria
- 36. AşkınK.KaplanM.D. Microangiopathic Hemolytic Anemia TTP/HUS • Microangiopathic hemolytic anemia • Thrombocytopenia • Renal function abnormality • Neurologic abnormalities, usually fluctuating • Fever • Normal PT, aPTT, fibrinogen • Markedly increased LDH • Direct Coomb’s test negative • Increased serum indirect bilirubin Easy to diagnose and treat if you think of it Untreated TTP is deadly
- 37.
- 38.
- 39. AşkınK.KaplanM.D. Algorithm for theevaluation of hemolytic anemia Algorithm for the evaluation of hemolytic anemia. (CBC = complete blood count; LDH = lactate dehydrogenase; DAT = direct antiglobulin test; G6PD = glucose-6-phosphate dehydrogenase; PT/PTT = prothrombin time/partial thromboplastin time; TTP = thrombotic thrombocytopenic purpura; HUS = hemolytic uremic syndrome; DIC = disseminated intravascular coagulation) https://www.aafp.org/afp/2004/0601/p2599.pdf
- 40.
- 41.