presentation

1. ENZYME DEFICIENCY ANEMIAENZYME DEFICIENCY ANEMIA
MICROANGIOPATHICMICROANGIOPATHIC
HEMOLYTIC ANEMIAHEMOLYTIC ANEMIA
ABNORMALITIES OF CELLSABNORMALITIES OF CELLS
ROLL # O1ROLL # O1
ROLL # 02ROLL # 02
ROLL # 03ROLL # 03
ROLL # 05ROLL # 05

2. ENZYME DEFICIENCY ANEMIAENZYME DEFICIENCY ANEMIA
PYRUVATE KINASE DEFICIENCYPYRUVATE KINASE DEFICIENCY
InheritedInherited
metabolic disordermetabolic disorder
AutosomalAutosomal
recessiverecessive
PATHOPHYSIOLOGPATHOPHYSIOLOG
YY
ATPATP
2,3 BPG2,3 BPG
TREATMENTTREATMENT
SPLENECTOMYSPLENECTOMY
BLOODBLOOD
TRANSFUSIONTRANSFUSION
CLINICAL
FINDING
JAUNDICEJAUNDICE
SPLENOMEGALYSPLENOMEGALY
LAB FINDINGSLAB FINDINGS
POIKILOCYTOSISPOIKILOCYTOSIS

3. GLUCOSE 6 PHOSPHATE DEHYDROGENASEGLUCOSE 6 PHOSPHATE DEHYDROGENASE
DEFICENCY (G6PD)DEFICENCY (G6PD)
It is an inherited disease characterized by hemolytic anemiaIt is an inherited disease characterized by hemolytic anemia
caused by inability to detoxify oxidizing agentscaused by inability to detoxify oxidizing agents

4. GLUCOSE 6 PHOSPHATE DEHYDROGENASEGLUCOSE 6 PHOSPHATE DEHYDROGENASE
DEFICENCY (G6PD)DEFICENCY (G6PD)
ETIOLOGY:ETIOLOGY:
Neonatal jaundiceNeonatal jaundice
The jaundice which may be severe typically results form increasedThe jaundice which may be severe typically results form increased
production of unconjugated bilirubin.production of unconjugated bilirubin.
SplenomegalySplenomegaly
HemglobinuriaHemglobinuria
Drug induced haemolysis.Drug induced haemolysis.
CLINICAL FINDINGS:CLINICAL FINDINGS:
Weakness or FatigueWeakness or Fatigue
Urine that is dark yellow.Urine that is dark yellow.
Yellow skin colorYellow skin color

5. LABORATORY FINDINGSLABORATORY FINDINGS
 Reticulocyte preparation to count reticulocyte & to look Heniz BodiesReticulocyte preparation to count reticulocyte & to look Heniz Bodies
 Complete blood count , Hb , checking Bilirubin level .Complete blood count , Hb , checking Bilirubin level .
 Bite cellsBite cells
 PolychromasiaPolychromasia
 Bister cell (Half-Ghost Cells)Bister cell (Half-Ghost Cells)

6. TREATMENT
• Blood transfusion (severe anemia).
• Stop drug intake which increase the formation of free
radicals.
POLYCHROMASIAPOLYCHROMASIA

7. MICROANGIOPATHIC HEMOLYTICMICROANGIOPATHIC HEMOLYTIC
ANEMIA (MAHA)ANEMIA (MAHA)
 The hemolysis that occur due to destruction of the red blood cell in theThe hemolysis that occur due to destruction of the red blood cell in the
small blood vessels as they pass by the micro thrombi formed by fibrinsmall blood vessels as they pass by the micro thrombi formed by fibrin
deposition and platelet aggregation.deposition and platelet aggregation.
 ““HELMET CELLS”HELMET CELLS” oror “SCHISTOCYTES”.“SCHISTOCYTES”.
 Associated with peripheral thrombocytopenia.Associated with peripheral thrombocytopenia.
CAUSES:CAUSES:
 Thrombotic thrombocytopenic purpuraThrombotic thrombocytopenic purpura
 DICDIC
 Mechanical traumaMechanical trauma

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