Hemolytic Anemia II

  • View
    223

  • Download
    0

Embed Size (px)

Text of Hemolytic Anemia II

  • 7/28/2019 Hemolytic Anemia II

    1/18

    Acquired Hemolytic Anemia

    Immune hemolytic anemiaNone-immune hemolytic anemia

  • 7/28/2019 Hemolytic Anemia II

    2/18

    Allo -immune hemolytic anemia

    Autoimmune hemolytic anemia

    Drug induced immune hemolytic anemia

    Immune hemolytic anemia

  • 7/28/2019 Hemolytic Anemia II

    3/18

    Allo-immune hemolytic anemia

    1. Incompatible ABO blood transfusion

    Donor Blood

    Goup A

    Recipient

    Group O

    Agglutination

    Hemolysis

    (cells contain A antigen) (cells contain neither A or B antigen)

    Plasma contains Anti-A and Anti-B

    (iso-antibody)

  • 7/28/2019 Hemolytic Anemia II

    4/18

    Clinical and Pathological effects

    Rigor : loin pain.hemoglobinuria

    jaudice (after 12 hours)

    SHOCK maybe DEATH

    Perhaps laterHemostatic failure, Renal failure maybe DEATH

    Allo-immune hemolytic anemia

    1. Incompatible ABO blood transfusion

  • 7/28/2019 Hemolytic Anemia II

    5/18

    2. Incompatibilities with in the Rh blood group system

    Incompatible blood transfusion

    DONOR

    (not previously transfused or pregnant)

    Rh-ve (i.e. rbcs do not contain Rh antigen)

    Plasma contains no Rh antibodies NO agglutination

    Rh +ve

    Later (months, years)Transfusion

    of Rh +ve blood

    to macrophage system for degradation

    in usual way but Rh+ve cells

    act as foreign antigens

    Formation of

    anti-Rh antibodies stimulated

    Agglutination

    and hemolysis

    RECIPIENT

    Allo-immune hemolytic anemia

    (rbcs contain Rh antigen)

  • 7/28/2019 Hemolytic Anemia II

    6/18

    Hemolytic Disease of the New born (HDN)

    First Pregnancy Rh+ve fetus in Rh-ve mother-no antibodies present.

    Healthy Baby

    Fetal circulation

    Placenta

    UterusMaternal

    circulation

    Damaged

    chorionic villus

    Rh+ve

    fetal rbcs

    into mother

    (Rh-ve)

    maternal

    blood sinus

    Trophoblast

    Gradual elimination

    by macrophage system

    Iso-immunisation

    Anti-Rh antibodies

    formed (IgG type)

    2. Incompatibilities with in the Rh blood group system

    Allo-immune hemolytic anemia

  • 7/28/2019 Hemolytic Anemia II

    7/18

    Hemolytic Disease of the New born (HDN)

    2. Incompatibilities with in the Rh blood group system

    Allo-immune hemolytic anemia

    Subsequent pregnancies

    Maternal anti-Rh antibodies IgG type

    Pass placental barrier

    Enter fetal circulation and destroy fetal red cells

    (agglutination and hemolysis)

    Placenta

    Uterus

  • 7/28/2019 Hemolytic Anemia II

    8/18

    Hemolytic Disease of the New born (HDN)

    2. Incompatibilities with in the Rh blood group system

    Allo-immune hemolytic anemia

    The effect are graded into 3 categories of severity:

    1. Congenital hemolytic anemia

    2. Icterus gravis neonatorum

    3. Hydrops fetalis

    severe anoxia in utero with cardiac failure and edema.

    severe anemia and jaudice,

    brain damage due to Kernicterus

    mild anemia and jaundice

  • 7/28/2019 Hemolytic Anemia II

    9/18

    Autoimmune Hemolytic Anemia (AIHA)

    1. Warm autoimmune hemolytic anemia (WAIHA)

    2. Cold autoimmune hemolytic anemia (cold AIHA)

  • 7/28/2019 Hemolytic Anemia II

    10/18

    Autoimmune hemolytic anemia :Warm antibody type (WAIHA)

    Reaction at normal temperature (37 C)

    = Antibody usually IgG type

    = Antigenic determinant

    Cell membrane

    modified

    Cell becomes

    microspherocytes

    With consequences similar tohereditary spherocytosis-

    early sequestration in spleen (RE)

  • 7/28/2019 Hemolytic Anemia II

    11/18

    Autoimmune hemolytic anemia : Cold antibody type

    Reaction at temperatures usually below 30 Cthey occur in peripheral circulation and in cold weather.

    Antibody

    combineswith RBC

    Reactions

    = Antigenic determinant

    Agglutination

    Clinically present as painfulhand and feet

    Amboceptor effect

    Ag/Ab activates complement

    Acute intravascular hemolysis

    usually of IgM type

  • 7/28/2019 Hemolytic Anemia II

    12/18

    Raynauds phenomenon

    manifested by marked pallorof the fingers, in the cold type

    Peripheral blood film showing

    a neutrophil/red cell rosette

  • 7/28/2019 Hemolytic Anemia II

    13/18

    Drug-Induced Immune Hemolytic Anemia

    1. Drug Adsorption Mechanism

    Penicillins, Cephalosporin and Streptomycins

    mechanism

    First the drug is nonspecifically adsorbed to the patients red cellSecond the drug must be able to elicit an antibody response

    + Drug (Ag) AntibodyB-Lymphocyte

    RBC

  • 7/28/2019 Hemolytic Anemia II

    14/18

    Drug-Induced Immune Hemolytic Anemia

    2. Immune Complex Mechanism : Innocent bystanderQuinidine and Phenacetin

    Drug (Ag) Antibody

    B-Lymphocyte

    (IgG and/or IgM)

    Extravascular

    hemolysisIntravascular

    hemolysis

    RE Complement

  • 7/28/2019 Hemolytic Anemia II

    15/18

    Drug-Induced Immune Hemolytic Anemia

    3. Methyldopa-Induced (Autoimmune) Mechanism

    methyldopa and related drugs (Aldomet, L-dopa):

    treatment of hypertension

    Drug (Ag) Antibody

    B-Lymphocyte

    Hemolysis

  • 7/28/2019 Hemolytic Anemia II

    16/18

    Direct Coombs test

    Indirect Coombs test

  • 7/28/2019 Hemolytic Anemia II

    17/18

    None-immune hemolytic anemia

    Red cell fragment syndrome

    Microangiopathic hemolytic anemia

    Macroangiopathic hemolytic anemia

    March hemoglobinuria

    Hypersplenism

    Paroxymal Nocturnal Hemoglobinuria

    the bone marrow produces red cells with defective cell

    membrane which are particular sensitive to lysis by complement

  • 7/28/2019 Hemolytic Anemia II

    18/18

    PNH : Acid lysis test. The affected red cells (on left) show

    marked complement-dependent lysis in acidified fresh serum

    at 37 C. Preheating the acidified serum inactivates complement,preventing lysis of the affected cells