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Hemolytic Anemias

Hemolytic Anemias. Defined as those anemias result from an increased in the rate of red cell destruction. The red cell destruction is usually removed extravascular by macrophages of reticuloendothelial system (RE) [BM, liver & spleen]. Acute destruction or chronic destruction

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Hemolytic Anemias

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  1. Hemolytic Anemias • Defined as those anemias result from an increased in the rate of red cell destruction. • The red cell destruction is usually removed extravascular by macrophages of reticuloendothelial system (RE) [BM, liver & spleen]. • Acute destruction or chronic destruction • Extravascular or Intravascular hemolysis • Hereditary or Acquired hemolytic anemia SJAOUNI, KAU

  2. SJAOUNI, KAU

  3. G6PD, glucose-6-phosphate dehydrogenase; Hb, hemoglobin. SJAOUNI, KAU

  4. Diagnosis • History • Clinical features • Laboratory findings • CBC (Hb & MCV), Plt, (WBC & diff) • Retic • Blood film • Specific tests SJAOUNI, KAU

  5. CBC WBC 8.3 10^3/L NE % 64.5 % LY % 27.6 % MO % 5.1 % EO % 2.6 % BA % 0.2 % NRBC %0.0 % NE # 5.3 10^3/L LY # 2.3 10^3/L MO # 0.4 10^3/L EO # 0.2 10^3/L BA # 0.0 10^3/L NRBC #0.0 10^3/L RBC 4.94 10^6/L HGB 14.3 g / dL HCT 40.8 % MCV 82.6 fL MCH 28.9 pg MCHC 35.0 g / dL RDW 12.8 % PLT 268 10^3/L MPV 7.5 fL RET % RET # IRF MRV SJAOUNI, KAU

  6. Laboratory findings in Hemolytic Anemia • Features of increased red cell destruction: • S. bilirubin,  urine urobilinogen breakdown, fecal stercobilinogen, s. haptoglobins absent. • Feature of  red cell productions • Reticulocytosis • BM erythroid hyperplasia • Damaged red cell • Morphology • Osmotic fragility & autohemolysis • Red cell survival SJAOUNI, KAU

  7. Autoimmune Hemolytic Anemia (AIHAs) Caused by antibody production by the body against its own red cells. • Characterized by a positive direct antiglobulin test (DAT) called Coombs test • Divided into warm and cold according Abs react at 37oC or 4oC. SJAOUNI, KAU

  8. SJAOUNI, KAU

  9. Cold Autoimmune Hemolytic Anemia In these syndromes the antibodies whether: • Monoclonal: Idiopathic cold agglutination lymphomas. • Polyclonal as infection: Infectious mononucleosis, Mycoplasma pneumonia • Patient may have a chronic hemolytic anemia aggravated by cold. Sometimes associated with intravascular hemolysis. Laboratory: • Similar like warm AIHA • Less spherocyte, more red cell agglutination • DAT reveals complement (C3d) SJAOUNI, KAU

  10. G6PD, glucose-6-phosphate dehydrogenase. SJAOUNI, KAU

  11. Causes of hereditary hemolytic anemia. SJAOUNI, KAU

  12. INHERITANCE ■ AUTOSOMAL ■ SEX-LINKED SJAOUNI, KAU

  13. Sex-linked Inheritance SJAOUNI, KAU

  14. Glucose-6-Phosphate Dehydrogenase (G6PD)Deficiency • Sex-linked hereditary hemolytic anemia • Acute intravascular hemolysis due to oxidant stress. • Episodes of acute hemolytic anemia • Most patients are asymptomatic • 4 Clinical syndromes: • oxidative stress-induced hemolysis by certain drugs, infection and other illness. • Favism (fresh and uncooked beans are more dangerous than dried cooked ones. • Neonatal jaundice • Chronic hemolytic anemia SJAOUNI, KAU

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