Hemolytic anemias
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HEMOLYTIC ANEMIAS. increase red cell destruction = reduced red- cell life span. HEMOLYTIC ANEMIAS. A red blood cell survives 90 to 120 days in the circulation ; about 1% of human red blood cells break down each day

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Hemolytic anemias

HEMOLYTIC ANEMIAS

increase red celldestruction = reduced red-cell life span


Hemolytic anemias1
HEMOLYTIC ANEMIAS

  • A red blood cell survives 90 to 120 days in the circulation;about 1% of human red blood cells break down each day

  • The spleen is the main organ which removes old and damaged RBCs from the circulation


Mechanisms of hemolysis
Mechanisms of hemolysis

  • Extravascular

    • red cellsdestructionoccurs in reticuloendothelial system

  • Intravascular

    • red cellsdestructionoccurs in vascularspace


  • Signs of hemolytic anemia s
    Signs of hemolytic anemias

    • Symptoms of anemia – pallor, fatique, rapid pulse

    • Jaundice

    • Splenomegaly


    Diagnosis of hemolytic anemias
    Diagnosis of hemolyticanemias

    • Anemia

    • Reticulocytosis

    • Indirecthyperbilirubinemia

    • Increasedlevel of lactate dehydrogenase (LDH)

    • Absenceorreduced of free serum haptoglobin


    Intravascular hemolysis
    INtravascularhemolysis

    • laboratorysigns of intravascularhemolysis: - tests for hemolysis

      and aditionally: - hemoglobinemia - hemoglobinuria - hemosiderynuria


    Hemolytic anemias2
    hemolyticanemias

    Compensated hemolysis – increase erythropoesis compensates increase destruction of erythrocytes

    Decompensated hemolysis - erythropoesis can not compensates increae destruction – patient needs therapy


    Complications of increased chronic hemolysis
    Complications of increased, chronichemolysis

    • Folinic acid deficiency

    • Gallstones

    • Thrombosis

    • Hemolytic crisis

      - rapid destruction of large numbers of red blood cells


    Classification of hemolytic anemias
    Classification of hemolyticanemias

    • Hereditary

      • Membranedefect (spherocytosis, elliptocytosis)

      • Metabolicdefect (Glucoze-6-Phosphate-Dehydrogenaze (G6PD) deficiency, Pyruvatekinase (PK) deficiency)

      • Hemoglobinopathies (thalassemias, sicklecell anemia )

    • Acquired

      • Immunehemolyticanemias

      • Nonimmunehemolyticanemias


    Hereditary membrane defects
    Hereditary membrane defects

    • Spherocytosis

      • The most commondefect of red cellmembrane protein (1/2000 birth)

      • Inheritance - autosomaldominant

      • Deficient of membrane protein causeschange of shape (round, no central pallor)

      • Clinicalfeatures: jaundice, gallstones, splenomegaly, constitutional skeleton changes (ietowercranium, gothicpalate)

      • Laboratoryfeatures: anemia, hiperbilirubinemia, retikulocytosis,  LDH - bloodsmear - microspherocytes - abnormalosmoticfragilitytest

      • Treatment - splenectomy



    Hereditary membrane defects1
    Hereditarymembranedefects

    • Elliptocytosis


    Hereditary metabolic defect
    Hereditary metabolic defect

    • Glucoze-6-Phosphate-Dehydrogenaze (G6PD) deficiency

      • Hemolysisisinduce by infections, drugs

      • Hemolysisisintravascular

    • Pyruvatekinase (PK) deficiency


    Hereditary hemoglobinopathies
    HereditaryHemoglobinopathies

    • Thalassemias

      • Alfa thalassemia

      • Beta thalasemia: major, minor (trait), intermedia

      • Delta/Beta thalassemia

      • Hereditarypersistentce of fetal hemoglobin

    • Sicklecell anemia


    SICKLE CELL ANEMIADefinition: chronichemolytic anemia characterized by sickle-shaped red cellscaused by homozygousinheritance of Hemoglobin S


    Sickle cell anemia incidence
    SICKLE CELL ANEMIA - incidence

    - Occurs mainly in people of African, Caribbean, Mediterranean descent

    - Homozygous - about 0,3% of Arficans Americans in the USA (have sickle cell anemia)

    Hetezygotes-8-13% of Africans Americans

    (are not anemic, but the sickling trait = sicklemia can be demonstrated in vitro)


    Sickle cell anemia pathogenesis
    SICKLE CELL ANEMIA-pathogenesis

    - Hemolysis - because sickle RBCs are too fragile to withstand the mechanical trauma of circulation

    - Occlusion in microvascular circulation caused by distorted, inflexible RBCs adhering to vascular endothelium


    Sickle cell anemia clinical features
    SICKLE CELL ANEMIA - clinicalfeatures

    IN HOMOZYGOTES

    Onset in the first or second year of live

    Period episodes of acute vascular occlussion (painful crisis)

    Consequences of vaso-occlusion of the microcirculations (tissue ischemia and infarction) - infarction of spleen, brain, marrow, kidney, lung, aseptic necrosis, central nervous system and ophtalmic vascular lesions

    Events which impair tissue oxygenation can precipitate crisis (f.e. pneumonia)


    Sickle cell anemia therapy
    SICKLE CELL ANEMIA-therapy

    Preventive measures:

    prevention or remedy of: infections (penicillin prophylaxis and pneumococcal vaccination), fever, dehydratation,acidosis, hypoxemia, cold exposure

    Blood transfusions for very severe anemia

    New approaches to therapy;

    1. Activation of Hb F synthesis - 5-azacytidine

    2. Antisickling agents acting on hemoglobin or membrane

    3. Bone marrow transplantation


    2 acquired
    2. Acquired

    A. Immunehemolyticanemias 1. Autoimmunehemolytic anemia - caused by warm-reactiveantibodies - caused by cold-reactiveantibodies 2. Alloimmunehemolytic anemia (transfusion of incompatibleblood)

    B. Nonimmunehemolyticanemias 1. Chemicals 2. Bacterialinfections, parasiticinfections (malaria) 3. Hemolysisdue to physical trauma (e.g. microangiopathichemolytic anemia)

    4. Hypersplenism

    5. Paroxysmalnocturnal hemoglobinuria (PNH)


    Autoimmune hemolytic anemia aiha
    Autoimmunehemolytic anemia - AIHA

    • caused by warm-reactiveantibodies (70%)

      - infections, connectivetissuedisorders, drugs

    • caused by cold-reactiveantibodies (30%)

      – in temp. < 37 (4°)

      - infections, CLL, NHL

      Laboratoryfindings: directCoombs test(directantiglobulin test)

      Treatment: underlyingdisease, steroids, immunosupresive

      Avoid RBC transfusions


    2 acquired1
    2. Acquired

    • Immunehemolyticanemias 1. Autoimmunehemolytic anemia - caused by warm-reactiveantibodies - caused by cold-reactiveantibodies 2. Alloimmunehemolytic anemiaB. Nonimmunehemolyticanemias 1. Chemicals 2. Bacterialinfections, parasiticinfections (malaria) 3. Hemolysisdue to physical trauma (e.g. microangiopathichemolytic anemia) 4. Hypersplenism

      5. Paroxysmalnocturnal hemoglobinuria (PNH)


    Alloimmune hemolytic anemia
    Alloimmunehemolytic anemia

    • Transfusion of incompatibleblood

    • Serologicincompatibility

    • Aftertransplantation of bonemarrowororgans


    2 acquired2
    2. Acquired

    • Immunehemolyticanemias 1. Autoimmunehemolytic anemia - caused by warm-reactiveantibodies - caused by cold-reactiveantibodies 2. Alloimmunehemolytic anemiaB. Nonimmunehemolyticanemias 1. Chemicals 2. Bacterialinfections, parasiticinfections (malaria) 3. Hemolysisdue to physical trauma - microangiopathichemolytic anemia 4. Hypersplenism

      5. Paroxysmalnocturnal hemoglobinuria (PNH)


    Classification o f microangiopathic hemolytic anemia
    Classificationof microangiopathichemolytic anemia

    • Thromboticthrombocytopenic purpura (TTP)

    • Hemolyticuremicsyndrome (HUS)


    Microangiopathic hemolytic anemia
    Microangiopathichemolytic anemia

    • Intravascular hemolysis caused by fragmentation of normal red cells passing through abnormal arterioles

    • Arterioles are changed by deposition of platelets and fibrin

    • Microvascular lesion cause organ demage (kidney, CNS)


    Microangiopathic hemolytic anaemia
    Microangiopathichemolyticanaemia

    Underlying disease

    • Invasive carcinoma

    • Complication of pregnancy

    • Serious infection

    • Drugs


    Microangiopathic hemolytic anaemia1
    Microangiopathichemolyticanaemia

    • Symptoms:

      • Related to the primary disease

      • Related to organs demage

    • Laboratory findings

    • Blood film: schistocytes


    2 acquired3
    2. Acquired

    • Immunehemolyticanemias 1. Autoimmunehemolytic anemia - caused by warm-reactiveantibodies - caused by cold-reactiveantibodies 2. Transfusion of incompatiblebloodB. Nonimmunehemolyticanemias 1. Chemicals 2. Bacterialinfections, parasiticinfections (malaria) 3. Hemolysisdue to physical trauma - hemolytic - uremicsyndrome (HUS) - thromboticthrombocytopenic purpura (TTP) - prostheticheartvalves 4. Hypersplenism

      5. Paroxysmalnocturnal hemoglobinuria (PNH)


    Hypersplenism
    Hypersplenism

    • State of hyperactivity of the spleen

    • Causes of hypersplenism

      • Infection – bacterial, viruses, fungi

      • Inflammatory diseases -

      • Neoplasm

      • Storage disorders (Gaucher disease)

      • Other – amyloidosis, sarcoidosis


    2 acquired4
    2. Acquired

    • Immunehemolyticanemias 1. Autoimmunehemolytic anemia - caused by warm-reactiveantibodies - caused by cold-reactiveantibodies 2. Transfusion of incompatiblebloodB. Nonimmunehemolyticanemias 1. Chemicals 2. Bacterialinfections, parasiticinfections (malaria) 3. Hemolysisdue to physical trauma - hemolytic - uremicsyndrome (HUS) - thromboticthrombocytopenic purpura (TTP) - prostheticheartvalves 4. Hypersplenism

      5. Paroxysmalnocturnal hemoglobinuria (PNH)


    Pathogenesis- anacquiredclonaldisease, arising from a somaticmutation in a single abnormalstemcell- deficiency of the GPI (glycosyl-phosphatidyl-inositol) anchor on the surface of hematopoieticcells- red cellsaremoresensitive to the lyticeffect of complement- intravascularhemolysis

    Paroxysmal nocturnal hemoglobinuria (PNH)


    Paroxysmal nocturnal hemoglobinuria pnh
    Paroxysmalnocturnal hemoglobinuria (PNH)

    Symptoms

    • Irregularly hemoglobinuria occurs with darkbrownurine in the morning

    • Hemolysisisreleased by infection, surgeryorotherevents

    • Increasedrisk of thrombosis

    • Renalfailure

    • Neurologicmanifestation - headaches


    Laboratoryfeatures- hemoglobinuria - hemosiderinuria - pancytopenia - chronicurinary iron loss - serum iron concentrationdecreased - positiveHam’s test (acidhemolysis test) - specificimmunophenotype of erytrocytes (CD59, CD55)

    Paroxysmalnocturnal hemoglobinuria (PNH)


    Treatment- washed RBC transfusion - iron therapy - allogenicbonemarrowtransplantationMonoclonalantibodyEculizumab(trade name Soliris)

    Paroxysmalnocturnal hemoglobinuria (PNH)


    What do you have to remember
    What do youhave to remember

    Anemia +

    • Retikulocytosis

    •  bilirubin (unconjugated),  LDH

    •  haptoglobin

    • Haemoglobin and haemosiderin in urine


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