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Hemolysis - Differential Diagnosis Framework 1) Environment - Fragmentation - MAHA: TTP, HUS, DIC, HELLP
Drug-associated, PCH, Evans Syndrome ... - Unconjugated Bilirubin ... Differential #Diagnosis #hematology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Macrophage Activation Syndrome ... Periodic fever syndromes ... ↑ AST ↑ ALT ↑ bilirubin ... Diagnosis #Management #Hematology ... #Rheumatology
Bilirubin Metabolism - Pathophysiology • Conjugated Bilirubin • Unconjugated Bilirubin By Dr. Khushboo Gala @LouLectures #Bilirubin #Metabolism #Pathophysiology
Bilirubin Metabolism ... • Conjugated Bilirubin ... • Unconjugated Bilirubin ... LouLectures #Bilirubin ... Pathophysiology #hepatology
Inherited Non-hemolytic Disorders of Hyperbilirubinemia == Disorders of Conjugation == Gilbert Syndrome: • 5-10% of the population
Conjugation == Gilbert Syndrome ... hyperbilirubinemia syndrome ... autosomal recessive syndrome ... Crigler-Najjar Syndromes ... #hepatology #diagnosis
Classification of Jaundice Unconjugated hyperbilirubinemia (predominantly indirect bilirubin) • Increased bilirubin production (eg, hemolytic anemias, hemolytic reactions,
predominantly indirect bilirubin ... • Increased bilirubin ... ) • Impaired bilirubin ... predominantly direct bilirubin ... Faulty excretion of bilirubin
Biochemistry and Metabolism of Bilirubin Hemolysis → Hemoglobin → Heme → Biliverdin → Unconjugated Bilirubin (a.k.a. "indirect
Metabolism of Bilirubin ... → Unconjugated Bilirubin ... "indirect bilirubin ... and "indirect" bilirubin ... pathophysiology #hepatology
Hypereosinophilia and Hypereosinophilic Syndrome • Secondary Hypereosinophilic Syndrome • Clinically Relevant HES Variants • When to
Hypereosinophilic Syndrome ... Hypereosinophilic Syndrome ... suspect underlying hematologic ... Hypereosinophilic #Syndrome ... #HES #Hematology
Laboratory Testing in Hemolytic Anemia Intravascular vs Extravascular Hemolysis Reticulocytes Unconjugated bilirubin LDH Peripheral smear Haptoglobin Urine hemosiderin Urine hemoglobin Carboxyhemoglobin #Hemolytic #Anemia #Laboratory #Testing #workup
Unconjugated bilirubin ... workup #diagnosis #hematology
Aplastic Anemia and Bone Marrow Failure Syndromes with Pancytopenia Fanconi Anemia Acquired Aplastic Anemia Dyskeratosis Congenita & Telemore Biology
Marrow Failure Syndromes ... Shwachman-Diamond Syndrome ... #Pancytopenia #Syndromes ... anemia #diagnosis #Hematology
Liver Disease in Pregnancy Intrahepatic Cholestasis of Pregnancy (ICP) • Onset/Trimester: 25-32 weeks • Typical clinical features:
fold elevation, Bilirubin ... necrosis HELLP Syndrome ... fold elevation, Bilirubin ... but variable ++, Bilirubin ... Disease #Pregnancy #hepatology
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