Hemolytic Anemia Dr Sommanikhone PHANGMANIXAY Pediatrician General Pediatrics

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Hemolytic Anemia ເລອດຈາງຈາກການແຕກຂອງເ ມດ ເລອດແດງ Dr. Sommanikhone PHANGMANIXAY Pediatrician General Pediatrics

Hemolytic Anemia ເລອດຈາງຈາກການແຕກຂອງເ ມດ ເລອດແດງ Dr. Sommanikhone PHANGMANIXAY Pediatrician General Pediatrics

Approach to normocytic anemia Is there increased red cell production? check reticulocyte count increased

Approach to normocytic anemia Is there increased red cell production? check reticulocyte count increased Is there evidence of hemolysis? yes hemolytic anemia normal or decreased Is there evidence of: - renal failure - endocrine failure - chronic inflammation anemia of renal failure anemia of endocrine failure anemia of chronic disease no recent bleed If not, then consider a primary marrow problem (MDS, MM, infiltration…) bone marrow investigation

An approach to hemolytic anemia Hemolytic anemia Immune • Autoimmune • Alloimmune Non-immune Congenital

An approach to hemolytic anemia Hemolytic anemia Immune • Autoimmune • Alloimmune Non-immune Congenital Acquired Defects of: • RBC membrane/ skeleton • Infections sepsis malaria (eg. Hereditary spherocytosis) • Drug-induced (other causes of immune hemolysis are rare) • Mechanical • RBC enzymes (eg. G 6 PD deficiency) • Hemoglobin prosthetic heart valve microangiopathic HA

ປດໃຈທເຮດໃຫເມດເລ ອດແດງແຕກ -Extravascular RBC cause: 6. ປະຕກລຍາທາງ Immune ເຊນ : isoimmunenization, autoimmune hemolytic anemia(AIHA)

ປດໃຈທເຮດໃຫເມດເລ ອດແດງແຕກ -Extravascular RBC cause: 6. ປະຕກລຍາທາງ Immune ເຊນ : isoimmunenization, autoimmune hemolytic anemia(AIHA) Systemic lupus erythematous (SLE), Rhumatoid arthritis, Wiskott-Aldrich syndrome, or Ulcerative colitis. 7. Medications: Penicilline, antimalaria medications, sulfa medications, or acetaminophen

An approach to hemolytic anemia Hemolytic anemia Immune • Autoimmune • Alloimmune Non-immune Congenital

An approach to hemolytic anemia Hemolytic anemia Immune • Autoimmune • Alloimmune Non-immune Congenital Acquired Defects of: • RBC membrane/ skeleton • Infections sepsis malaria (eg. Hereditary spherocytosis) • Drug-induced (other causes of immune hemolysis are rare) • Mechanical • RBC enzymes (eg. G 6 PD deficiency) • Hemoglobin prosthetic heart valve microangiopathic HA

G 6 PD deficiency § Laboratory diagnosis § bite cells § Heinz bodies §

G 6 PD deficiency § Laboratory diagnosis § bite cells § Heinz bodies § measure G 6 PD level § Treatment § supportive § avoid precipitants § counsel patient/family

Causes of intravascular hemolysis § Mechanical § prosthetic heart valve, tight AS § march

Causes of intravascular hemolysis § Mechanical § prosthetic heart valve, tight AS § march & bongo drummer’s hemoglobinuria § Microangiopathic § DIC, TTP, HUS § Immunological § acute hemolytic transfusion reaction, PNH § Infection § malaria § Clostridium welchii sepsis § Enzymopathy § severe G 6 PD deficiency

An approach to hemolytic anemia Hemolytic anemia Immune • Autoimmune • Alloimmune Non-immune Congenital

An approach to hemolytic anemia Hemolytic anemia Immune • Autoimmune • Alloimmune Non-immune Congenital Acquired Defects of: • RBC membrane/ skeleton • Infections sepsis malaria (eg. Hereditary spherocytosis) • Drug-induced (other causes of immune hemolysis are rare) • Mechanical • RBC enzymes (eg. G 6 PD deficiency) • Hemoglobin prosthetic heart valve microangiopathic HA

Normal membrane cytoskeleton Hereditary spherocytosis loss of membrane = loss of SA = loss

Normal membrane cytoskeleton Hereditary spherocytosis loss of membrane = loss of SA = loss of deformability = increased splenic clearance