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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 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
slide17

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)

slide36

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
slide38

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

Paroxysmalnocturnal hemoglobinuria (PNH)

slide39

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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