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    Immune Hemolytic Anemia

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    titis
    The document discusses immune hemolytic anemia, including its classification into autoimmune hemolytic anemia types like warm AIHA and cold AIHA, as well as alloimmune hemolytic anemia and drug-induced hemolytic anemia. The pathogenesis, clinical features, laboratory findings, management, and treatment of refractory cases are described for different types of immune hemolytic anemia.

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    Immune Hemolytic Anemia

    Uploaded by

    titis
    119 views17 pages
    The document discusses immune hemolytic anemia, including its classification into autoimmune hemolytic anemia types like warm AIHA and cold AIHA, as well as alloimmune hemolytic anemia and drug-induced hemolytic anemia. The pathogenesis, clinical features, laboratory findings, management, and treatment of refractory cases are described for different types of immune hemolytic anemia.

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    imune hemolitic anemia

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    14. Immune Hemolytic Anemia

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    The document discusses immune hemolytic anemia, including its classification into autoimmune hemolytic anemia types like warm AIHA and cold AIHA, as well as alloimmune hemolytic anemia and drug-induced hemolytic anemia. The pathogenesis, clinical features, laboratory findings, management, and treatment of refractory cases are described for different types of immune hemolytic anemia.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    119 views17 pages

    Immune Hemolytic Anemia

    Uploaded by

    titis
    The document discusses immune hemolytic anemia, including its classification into autoimmune hemolytic anemia types like warm AIHA and cold AIHA, as well as alloimmune hemolytic anemia and drug-induced hemolytic anemia. The pathogenesis, clinical features, laboratory findings, management, and treatment of refractory cases are described for different types of immune hemolytic anemia.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 17

    Red Cell Disorders:

    Immune Hemolytic Anemia

    Susanto Nugroho

    Faculty of Medicine
    University of Brawijaya

    Clinical Competencies
    After this session:
    Students can describe:
    - the pathogenesis of immune hemolytic anemia
    - the classification of immune hemolytic anemia
    - the principal management of immune hemolytic
    anemia
    Students can diagnose immune hemolytic anemia
    based on clinical features and laboratory findings

    Classification
    1. Autoimmune hemolytic anemia (AIHA)
    a. Warm AIHA
    b. Cold AIHA
    c. Mixed-type AIHA
    2. Alloimmune hemolytic anemia
    3. Drug-induced hemolytic anemia

    Pathogenesis
    Autoimmune Hemolytic Anemia (AIHA)
    AIHA are caused by antibody production by the
    body against its own red cells: characterized by a
    positive DAT or the Coombs test : cold, warm and
    mixed-types.
    Cold AIHA
    The autoantibody attaches to red cells (IgM)
    mainly in the peripheral circulation where the
    blood temperature is cooled (00C - 40C):
    are highly efficient at fixing complement
    intravascular & extravascular hemolysis

    Pathogenesis.....
    Warm AIHA
    Warm hemolysis refers to IgG autoantibodies,
    which maximally bind RBC at body
    temperature (37C).
    When warm autoantibodies attach to RBC surface
    antigens, these IgG-coated red blood cells are
    partially ingested by the macrophages of the
    spleen, leaving microspherocytes, the
    characteristic cells of AIHA. These
    spherocytes, which have decreased
    deformability compared with normal red blood
    cells, are trapped in the splenic sinusoids and
    removed from circulation.

    Pathogenesis.....
    Mixed AIHA
    Some patients with warm AIHA also possess a cold
    agglutinin. While the majority of these cold
    agglutinin are not clinically significant,
    occasionally they have a sufficient thermal
    amplitude (greater than 300C) or high titer
    (greater than 1:1,000 at0-400C) to indicate
    cold agglutinin syndrome (CAS)
    Mixed-type AIHA can be either idiopathic or
    secondary to lymphoproliferative disorders or
    SLE

    Pathogenesis.....
    Alloimmune Hemolytic Anemia
    The most severe alloimmune hemolysis is an
    acute transfusion reaction caused by ABOincompatible RBCs, e.g. transfusion of A donor
    into O recipient (who has circulating anti-A IgM
    antibodies) leads to complement fixation and
    a brisk intravascular hemolysis: may occur
    fever, chills, dyspnea, hypotension, shock.
    Delayed hemolytic transfusion reactions (310 days after a transfusion): caused by low
    titer antibodies to minor RBC antigens.

    Pathogenesis.....
    Drug-Induce Hemolytic Anemia
    Three mechanisms of action:
    1. drug-absorption (hapten-induced)
    2. immune complex
    3. autoantibody
    These IgG- and IgM-mediated disorders produce a
    positive DAT and are clinically and
    serologically indistinct from AIHA.

    Cold AIHA
    Etiologic classification:
    1. Idiopathic
    2. Secondary:
    - Infections: M. pneumonia, infectious
    mononucleosis
    - Lymphoma
    - Paroxysmal cold hemoglobinuria (rare,
    sometimes
    associated with infections, e.g. syphilis)
    Clinical features
    Mild jaundice & splenomegaly may be present
    May develop acrocyanosis (purplish skin
    discoloration) at the tip of the nose, ears,
    fingers, and toes caused by the agglutination of

    Cold AIHA..
    Laboratory findings
    Similar to those of warm AIHA, except that
    spherocytosis is less marked, red cells
    agglutinate in the cold and the DAT reveals
    complement (C3d) only on the RBC surface
    Management
    Treating the underlying cause
    Splenectomy does not usually help unless
    massive splenomegaly is present, and steroids
    are not helpful

    Warm AIHA
    Etiologic classification
    1. Idiopathic
    2. Secondary:
    - SLE, other autoimmune disease
    - CLL, lymphomas
    - Drugs, e.g. methyldopa, fludarabine
    Clinical features
    Occur at any age in either sex and varying
    severity
    The spleen in often enlarged
    Tend to remit and relapse
    May occur alone or in association with other
    diseases e.g. ITP, SLE or arise as a result of
    methyldopa therapy

    Warm AIHA..
    Laboratory findings
    Peripheral blood: typical of an extravascular
    hemolytic anemia with spherocytosis prominent
    The DAT (+): antibodies are best detected at
    370C
    Management
    Remove the underlying cause (e.g.
    methyldopa)
    Corticosteroids
    Splenectomy
    Immunosupression : azathioprine, CPH,
    cyclosporine
    Folic acid
    Blood transfusion

    Treatment of Refractory AIHA


    1. Intravenous immune globulin (IVIG)
    2. Danazol
    3. Newer immunosuppressives : mycophenolate
    mofetil
    4. Monoclonal antibodies : Rituximab (Rituxan),
    and Alemtuzumab (Campath-1H)

    Complications
    Thromboembolism
    The most common cause of death was
    pulmonary
    embolism and 27% patients suffered from an
    episode of venous thromboembolism
    AIHA in patients with SLE: the risk of
    thrombosis to be increased more than 4-fold
    Lymphoproliferative disorders
    Associated immune disorder (e.g., rheumatoid
    arthritis, temporal arteritis, Crohns disease,
    lupus, thyroiditis, Sjgrens syndrome) and
    chronic lymphocytic leukemia

    Modul Task
    Case:
    Mr. P, 35 year-old
    History taking: pale 2 week; jaundice; no bleed;
    history of fever 2 months ago; acrocyanosis at the
    tip of the nose, ears, fingers, and toes.
    Physical examination: conjunctiva palpebra
    anemic; sclera icteric; spleen enlargement
    (Suffner I)
    Laboratory results: Hb 6.5 g/dl; WBCs
    5640/mm3; platelet 284,000mm3; MCV, MCH and
    MCHC normal; peripheral blood smear:
    spherocytosis; DAT is positive.

    Questions:
    1.What is THE MOST PROBABLE DIAGNOSIS of this
    patient?
    2.Describe in brief CLASSIFICATION &
    PATHOGENESIS of this disease!
    3.How TO DIAGNOSE each type (warm and cold
    type) of this disease based on the clinical
    features and laboratory findings?
    4.How TO TREAT each type (warm and cold type)
    of this disease?
    5.How TO TREAT the refractory cases of this
    disease?
    6.What are COMPLICATIONS of this disease?
    Please.discuss this case in your group!!

    THANK YOU

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