Haem14: Hemolytic anemia Congenital

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Text of Haem14: Hemolytic anemia Congenital

JCU Shashi

1Learnignorance will disappear of itself!

1Clinical Pathology: RBC 1.4: Congenital Hemolytic AnemiaCPC: Term2 Week1 - Haem 1/2.System: Haematology - RBC Disorders.Topic: 1: Anemia Intro 2: IDA, MBA & ACD 3: Acquired HA 4: Congenital HA. 5: Others... foundation of clinical medicine Shashidhar Venkatesh MurthyA/Prof & Head of PathologyCollege of Medicine & Dentistry2Pathogenetic Classification of Anemia:Decreased Production:Nutrient Deficiency. Iron def (IDA) / Megaloblastic (MBA)Hemopoietic cell defect:Anemia of chronic disorders (ACD)Aplastic anemia (AA).Dysplastic anemia. Myelodysplastic SyndromesIncreased loss / destruction:Blood loss anemia Acute / Chronic - bleeding. Hemolytic anemia Congenital / Acquired.Acquired / External injury.Immune AIHA (Warm/Cold) Mechanical, Drugs & ParasitesCongenital / Internal RBC defectDefective Membrane: Hereditary Spherocytosis.Defective Hemoglobin: Sickle & Thalassemia Deficient Enzyme: G6PD deficiency anemia.33Cell MemHb.Enzymes3Hereditary Spherocytosis: Spectrin deficiency*4

Chronic hemolytic anemia (from birth or late)Plenty of spherocytes. (more than in WAHA)Massive splenomegaly.Cholecystitis and cholelithiasisAplastic, megaloblastic or hemolytic crisis(Combs test negative)4Hereditary Spherocytosis:5

Normal spleen size of a fist!

SplenomegalySplenectomy: Therapy for severe HS5

G6PD Deficiency: Oxidative hemolysisG6PD deficiency.Oxidative damage (old RBC)Heinz bodies (globins)Bite & blister cells (dry RBC*)Episodic Hemolysis.Splenomegaly, gall stones..6

6"Thinking should become your capital asset, no matter whatever ups and downs come across in your life.Dr. APJ Abdul Kalam, Former President of India..

7Hemoglobin Disorders:8

Physiologic anemia change from Hb F to Hb A.Hb A 22 - 97%Hb A2 22 - 2.5%Hb F 22 -