6,Hemolytic Anemia

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    1stintroduction1stintroduction

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    Definition

    Hemolytic anemias (HA) result

    from a shortened erythrocytelife span ,increased or faster

    destruction with insufficient

    compensation from increasederythrocyte production.

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    HA classification

    according to etiological

    factor

    Intracorpuscular defects

    Extracorpuscular defects

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    Intracorpuscular defectsInheritedRBC

    membrane defects(hereditary spherocytosis, HS)

    enzyme defects ( glucose-6-phosphate dehydrogenase G-6-PD,

    pyruvic ketolase, PK )hemoglobinopathies,

    Sickle cell, thalassemias

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    Acquired:PNH ( paroxysmal nocturnal hemoglobinuria )

    PNH is caused by a defect in the formation

    of protein anchor called GPI ( glycosylphosphatidyl inositol)Theloss of these cell-surface proteins enhancedsensitivity to complement-mediated cell

    destruction. This destruction is intermittent(paroxysmal). The disease can affect peopleof any age.

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    Extracorpuscular defectsInherited

    Abetalipoproteinemia

    A disorder of lipid metabolism inherited as an autosomal

    recessive trait characterized by the near absence of

    APOLIPOPROTEINS B and apoB-containing lipoproteins in

    plasma. Microsomal triglyceride transfer protein is deficient or

    absent in enterocytes. Clinical and laboratory findings includeacanthocytosis, hypocholesterolemia,

    peripheral neuropathy, posterior column degeneration, ataxia,

    and steatorrhea. Intellectual abilities may also be impaired.

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    Acquired

    autoimmune disease

    Trauma and microangiopathic: HUS(hemolytic-uremic syndrome),

    DIC,TTP

    Infectious agents: protozoa, bacteriachemical damage: drugs, venoms

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    HA classification

    according to hematolysis occurred place

    (1)Intravascular hemolysis

    Lysis occurs within the circulation ,RBC membraneinjury (trauma, autoimmune, infection)

    (2)Extravascular Hemolysis (most

    common)Lysis occurs in the tissues (Splenic and hepatic

    clearance of defective RBCs)

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    causes of hemolysis :

    immune reaction, chemistry,physics and biology factoreffect on RBC.such as

    autoimmune HemolyticAnemia(AIHA

    chemical intoxicant or medicine

    after cardiac valve replacementinfection result in hemolysis anemia

    hypersplenism

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    .definite lab test for HAstep of diagnose HA :

    First, must be sure whether HA exist

    Then, analyze the reasonsThere are specific tests which identify the

    specific types of hemolytic anemia. They areperformed after hemolysis has been established.

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    display evidences of

    hematoclasis increase

    display evidences ofRBC

    compensatory hyperplasia

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    2nd

    Screen tests ofhemolytic anemia

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    Extravascular Hemolysis

    Ingested by RE cell (spleen

    & liver)

    Intravascular Hemolysis

    Hgb liberated in

    blood vessel

    Hgb + haptoglobin

    Hgb + albumin

    Hgb excreted

    in urine

    Heme Globin

    Re-utilizedIron Protoporphyrin

    Re-utilized bilirubin

    q Serumhaptoglobin

    + hemalbumin

    & plasma Hgb

    +hemoglobinuria

    & hemosidenuria

    Hemolysis - RBC destruction

    RE: reticulo-endothelium

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    1.related test about RBC life shorten

    and hematoclasis increase

    (1) RBC life test: is the most direct evidence for

    diagnosis. Direct measurement of the red cell lifespan by isotopic tagging techniques shows a

    decreased life span.

    Half life of health adult RBC : 25-35 days

    Half life of HA:

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    (2) RBC morphology change:

    we can see spherocytosis ,sickle cell,

    acanthocytosis , anisocytosis,polychromatic erythrocyte, stippling red

    cell, Howell-Jollys Bodies, Cabots

    ring Bodies, nucleated red cell

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    (3) Elevated absolute reticulocyte

    count

    (4) Low red blood cell count and

    hemoglobin

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    (5) plasma lactic dehydrogenase

    (PLDH) test:

    RBC destroy LDH in cell release to

    plasma elevated serum LDH .

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    2.measurement of free-hemoglobin

    in plasma

    Referenc value: 1g/L), BG

    incompatibility transfusion, PNH extravascular hemolysis is normal

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    3.test of haptoglobin in serumReference value:0.7-1.5g/L

    Clinical meaning:

    decrease:intravasular hemolysis ( acute serious

    IVH---0 ) , extravasular hemolysis and

    liver disease increase :

    infection, trauma, malignant tumor

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    4.test of methemoglobin in plasma

    Normal :

    negative

    Positive :

    serious intravascular hemolysis

    ( Ferrihemoglobin )

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    5.hematoglo

    binuria test

    Positive results: only occur in the

    early days of acute IVH attack

    6.Rous test (hemosiderinuria test)Positive results: chronic IVH and

    lasting several weeks,such as PNH.In

    the early stage of hemolysis, this testis temporarily negative.

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    7.bilirubin metabolism abnormal

    elevated unconjugated bilirubin

    (UCB) in serum

    jaundice

    Increased urine and fecal

    urobilinogen

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    3rd

    Tests ofMembrane defects

    3rd

    Tests ofMembrane defects

    1. erythrocyte osmotic fragility test:

    Red blood cell sensitivity to change inosmotic pressure. When exposed to ahypotonic concentration of sodium in a

    solution, red cells take in more water, swelluntil the capacity of the cell membrane isexceeded, and burst

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    reference range :

    begin hemolysis: 0.42-0.46%4.2- 4.6g/L sodium chloride

    complete hemolysis: 0.28-0.34%

    4.2- 4.6g/L sodium chloride

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    clinical meanings

    to contrast with health adult above0.04g/LNaCL is (+)

    Fragility increase:

    Congenital spherocytosis ,

    Immune hemolytic Anemia

    Fragility decrease:thalassemia, IDA, cirrhosis

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    2.Incubated osmotic fragility test:

    3.autohemotysis and correction test

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    4th

    Tests ofEnzyme deficiencies

    4th

    Tests ofEnzyme deficiencies

    Hemolytic anemia caused by rbc Enzyme

    deficiencies also are called Erythrocyteenzymopathy. It refers to enzyme esp that

    takes part in RBC metablism (mainly

    glycometabolism ) have genetic deficiencies,

    then result in changed activity, causehemolysis diseases.

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    1. G6PDDeficiency

    glucose-6-phosphate dehydrogenase Indications for testing

    Acute or chronic non-spherocytic

    Hemolytic Anemia Asymptomatic children with

    significant risk factors (such asFavism )

    Nonphysiologic Neonatal Jaundicewith risk factors

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    Associated risk factors

    (1). African, Mediterranean, orAsian ethnicity

    (2). Family History of Jaundice,

    Anemia or Splenomegaly

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    2.methemoglobin reduction test

    3.cyanide-Ascorbate test

    4.nitro-tetrazolium test

    5.pyruvate kinase ultravioletspectrometry

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    5th

    Test o

    fhem

    oglobinopathi

    es

    5th

    Test o

    fhem

    oglobinopathi

    es

    hemoglolin electrophoresis

    reference value :

    adults hemoglolin include HbA

    (E2 F2 ),HbA

    2(E2 H2 ), and HbF(E2 K2)A 96-98% A2 1-3%F 1%

    Clinical significance:

    HbA2 increase: mitis -Mediterraneananemiasome megaloblastic anemia

    HbA2 decrease :IDA, sideroblastic anemia

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    6th

    Test ofauto immune hemolyticanemia (AIAH)

    6th

    Test ofauto immune hemolyticanemia (AIAH)

    Antihuman globulin test (Coombs Test )

    The Direct Coombs Test

    The Indirect Coombs Test

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    The Direct Coombs Test

    Looks for immunoglobulin &/or complement of

    surface of red blood cell (normally neither found

    on RBC surface)

    Coombs reagent - combination of anti-humanimmunoglobulin & anti-human complement

    Mixed with patients red cells; if immunoglobulin

    or complement are on surface, Coombs reagent

    will link cells together and cause agglutination of

    RBCs

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    The DirectC

    oombs Test