of 34/34
Hemolytic Anemias

1394330044 Hemolytic Anemia

  • View
    20

  • Download
    0

Embed Size (px)

DESCRIPTION

hemolytic anemia

Text of 1394330044 Hemolytic Anemia

  • Hemolytic Anemias

  • Three essential features

    Premature destruction of red cells and a shortened red cell life span

    Elevated erythropoietin levels and a compensatory increase in erythropoiesis

    Accumulation of hemoglobin degradation products

  • Pathogenesis and classification

    Acute and Chronic (clinical classification)

    Inherited or Acquired

    Sites of red cell destruction Intravascular

    Extravascular (tissue macrophages,mainly spleen)

    Nature of defect Intrinsic /intracorpuscular RBC abnormality

    (Usually inherited,exception PNH)

    Extrinsic abnormality /extracorpuscular

  • Etiologic and pathogenetic classification scheme

    Inherited hemolytic disorders

    Defects in the erythrocyte membrane (hereditary spherocytosis,elliptocytosis,stomatocytosis etc)

    Hereditary enzyme deficiency (disorders of HMP shunt,G6PD def, PK deficiency etc)

    Inherited disorders of Hb or synthesis (Unstable Hb disorders,sickle cell,thalassemia syndromes, other homozygous hemoglobinopathies)

  • Etiologic and pathogenetic classification scheme

    Acquired Hemolytic anemia (classified on the basis of extrinsic factor causing hemolysis)

    Immune causes

    Auto-immune acquired Hemolytic anemia

    Hemolytic disease of new born

    Incompatible blood transfusion

  • Non-immune causes

    Mechanical hemolytic anemia (cardiac,microangiopathic,march hemoglobinuria)

    Miscellaneous (infectious agents,physical agents,chemical agents,hypophosphatemia,liver disease etc)

    Paroxysmal Nocturnal Hemoglobinuria (acquired hemolytic but intrinsic red cell abn)

  • globin porphyrin

    bilirubin

    bilirubin

    Conjugated bilirubin

    urobilinogen

    Urobilinogen Urinary elimination

    stercobilinogen Fecal elimination

    Enterohepatic circulation

  • Sites of destruction of red cells:Extravascular and

    intravascular

    Extravascular : RE system

    Red cells become less deformable or are identified as Foreign

    Spleen is the major assasin

  • Significant intravascular destruction

    PNH

    Associated with certain infections

    Blackwater fever

    Clostridial sepsis

    Chemical agents

    Thermal injury

  • Clinical features of congenital hemolytic anemias

    Degree of anemia

    Jaundice

    Aplastic crisis

    Splenomegaly

    Cholelithiasis

    Leg ulcers

    Skeletal abnormalities

  • Splenomegaly in hereditary spherocytosis

  • Clinical features of acquired hemolytic anemias

    Onset mainly acute,may also be insidious

    After transfusion of incompatible blood or after exposure to drugs

    Aching pain back,abdomen,limbs

    Pallour

    Jaundice

    Trachycardia

  • Lab features of hemolysis

    Signs of increased red cell destruction

    Increased indirect s.bilirubin

    Increased serum LDH

    Decreased serum haptoglobin

    Decreased RBC survival

    Increased rate of CO production

  • Signs of intravascular hemolysis

    Hemoglobinemia

    Hemoglobinuria

    Hemosiderinuria

    Decreased hemopexin

    Methemalbuminemia

    Increased serum bilirubin

  • Signs of accelerated erythropoeisis

    Reticulocytosis

    Macrocytosis

    Polychromatophilia

    Basophilic stippling

    Erythroblastosis

    Abnormal red cell forms,autoagglutination

    Bone marrow:erythroid hyperplasia

  • Morphological abn in hemolytic anemia

    Cell Description Disorder

    Spherocyte Spheric HS,IHA,Burns

    Elliptocyte OVal HE,Megaloblastic a

    Stomatocyte Uniconcave,slit like pallour Her stomatocytosis,alcoholism

    Acanthocytes, irregular spicules Spur cell anemia

    Echinocytes Regular,even,excess spicules

    Uremia

    Sickle cell crescent SC Anemia

    Target cell Solid area in central pallor Thalasemia,HbC,liver disease

    Schistocyte Triangular,helmet shaped Microangiopathic anemia,HUS

    Bite cells,ghost cells G6PD

  • Specific tests

    Osmotic fragility

    Coombs test

    Heinz Body determination

    Hb electrophoresis/HPLC

  • 80 50 70 60 30 40 20 10

    25

    100

    75

    50

    % of NaCl

    % o

    f hem

    oly

    sis

  • Anti-Globulin (Coombs) Testing Direct antiglobulin testing

    Indirect antiglobulin testing

    Patients RBCs

    Patients serum

    Anti-C3d

    Anti-IgG

    +

    RBCs

    +

    Anti-IgG

    +

  • To be differentiated from

    Post haemorrhagic anemia

    Recovery from nutritional deficiency anemia

    Recovery from marrow failure

    Look for signs of hemolysis to establish a diagnosis of hemolytic anemia