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Haem14: Hemolytic anemia Congenital

Congenital hemolytic anemias.

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Haem14: Hemolytic anemia Congenital

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  1. Learn…ignorance will disappear of itself…! 1

  2. Shashidhar Venkatesh Murthy A/Prof & Head of Pathology College of Medicine & Dentistry ….. foundation of clinical medicine Clinical Pathology: RBC 1.4: Congenital Hemolytic Anemia CPC System Topic : Term2 Week1 - Haem 1/2. : Haematology - RBC Disorders. : 1: Anemia Intro 2: IDA, MBA & ACD 3: Acquired HA 4: Congenital HA. 5: Others.

  3. Pathogenetic 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 Syndromes  Increased loss / destruction:  Blood loss anemia – Acute / Chronic - bleeding.  Hemolytic anemia – Congenital / Acquired.  Acquired / External injury.  Immune AIHA (Warm/Cold) Mechanical, Drugs & Parasites  Congenital / Internal RBC defect  Defective Membrane: Hereditary Spherocytosis.  Defective Hemoglobin: Sickle & Thalassemia  Deficient Enzyme: G6PD deficiency anemia. •Cell Mem •Hb. •Enzymes 3 3

  4. Hereditary Spherocytosis: Spectrin deficiency* 1. Chronic hemolytic anemia (from birth or late) 2. Plenty of spherocytes. (more than in WAHA) 3. Massive splenomegaly. 4. Cholecystitis and cholelithiasis 5. Aplastic, megaloblastic or hemolytic crisis (Comb’s test negative) 4

  5. Hereditary Spherocytosis: Normal spleen size of a fist…! Splenectomy: Therapy for severe HS 5

  6. G6PD Deficiency: Oxidative hemolysis 1. G6PD deficiency. 2. Oxidative damage (old RBC) 3. Heinz bodies (globins) 4. Bite & blister cells (dry RBC*) 5. Episodic Hemolysis. 6. Splenomegaly, gall stones.. 6

  7. "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..

  8. Hemoglobin Disorders: Congenital Hb. disorders: Globin chain* • Qualitative: Hemoglobinopathy eg. Sickle • Quantitative: Thalassemia syndromes. Hb F - α2γ2- 75% Hb A - α2β2 25% Hb A α2β2- 97% Hb A2α2δ2 - 2.5% Hb F α2γ2- <1% HbA HbF Physiologic anemia – change from Hb F to Hb A. 8

  9. Hb Electrophoresis: New born Screening - Patient  Hb-S Hb-F Hb-A A B C D Control Hb Barts Sickle (β4) Normal αThal αThal+ Sickle. + 9

  10. • Heinz bodies. • Basophilic stippling. Thalassemia Pathologenesis  Normal Hb. - micro/hypo  Destruction - Hemolysis.  Marrow Hyperplasia.  Iron - Hemochromatosis Trait - Minor - Major 10

  11. Thalassemia: Minor / Trait  Quantitative - globin deficiency.  Defective globin chain synthesis.  α, β, , , … types.   normal Hb & Abnormal globin complexes.  α thal  α  Hb  excess β forms abnormal tetramers (Heinz bodies, basophilic stippling etc) Major / Disease  Minor: Normal / mild anemia. micro hypo, target cells.  Major: severe Haemolytic anemia (transfusion dependent) 11

  12. "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..

  13. Sickle Cell Anemia: Hemoglobinopathy. Acute Chest Syndrome 13

  14. Sickle Cell Anemia

  15. Sickle Cell Disease: Clinical Features: 1. Anemia 2. Jaundice 3. Gall stones 4. Leg Ulcers 5. Auto-splenectomy. 6. Crisis* Splenic atrophy – (Auto-infarction) 15

  16. The power of thought is not a compelling force. It is a building force, and it is only when used in the latter sense that desirable results can be produced. -- Christian D. Larson

  17. You are the stone.. Need help? contact me… 1. Office location: DB39-136 (Townsville) 2. Office Tel: 4781 4566 3. Email: venkatesh.shashidhar@jcu.edu.au 4. Emergency?: 0416933704 Need personal coaching? Email me for an appointment.

  18. The pessimist waits for better times, and expects to keep on waiting; the optimist goes to work with the best that is at hand now, and proceeds to create better times. -- Christian D. Larson

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