1Learn…ignorance will disappear of itself…!
….. foundation of clinical medicine
Shashidhar Venkatesh MurthyA/Prof & Head of PathologyCollege of Medicine & Dentistry
Clinical Pathology: RBC 1.4: Congenital Hemolytic Anemia
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.
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3•Cell Mem•Hb.•Enzymes
Hereditary Spherocytosis: Spectrin deficiency*
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1. Chronic hemolytic anemia (from birth or late)2. Plenty of spherocytes. (more than in WAHA)3. Massive splenomegaly.4. Cholecystitis and cholelithiasis5. Aplastic, megaloblastic or hemolytic crisis
(Comb’s test negative)
Hereditary Spherocytosis:
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Normal spleen size of a fist…! Splenomegaly
Splenectomy: Therapy for severe HS
G6PD Deficiency: Oxidative hemolysis1. G6PD deficiency.2. Oxidative damage (old RBC)3. Heinz bodies (globins)4. Bite & blister cells (dry RBC*)5. Episodic Hemolysis.6. Splenomegaly, gall stones..
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"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..
Hemoglobin Disorders:
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Physiologic anemia – change from Hb F to Hb A.
Hb A α2β2 - 97%Hb A2 α2δ2 - 2.5%Hb F α2γ2 - <1%
HbF HbA
Hb F - α2γ2- 75% Hb A - α2β2 25%
Congenital Hb. disorders: Globin chain*• Qualitative: Hemoglobinopathy eg. Sickle• Quantitative: Thalassemia syndromes.
Hb Electrophoresis:
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-Patient
Hb-SHb-FHb-A
Hb Barts(β4)
+
ControlA B C D
αThalNormal
αThal+Sickle.
Sickle
New born Screening
Thalassemia Pathologenesis
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• Heinz bodies.• Basophilic stippling.
Normal Hb. - micro/hypo Destruction - Hemolysis. Marrow Hyperplasia. Iron - Hemochromatosis
Trait - Minor - Major
Thalassemia:
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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)
Minor: Normal / mild anemia. micro hypo, target cells.
Major: severe Haemolytic anemia (transfusion dependent)
Minor / Trait
Major / Disease
"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..
Sickle Cell Anemia: Hemoglobinopathy.
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Acute Chest Syndrome
Sickle Cell Anemia
Sickle Cell Disease:Clinical Features:
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1. Anemia2. Jaundice3. Gall stones4. Leg Ulcers5. Auto-splenectomy.6. Crisis*
Splenic atrophy – (Auto-infarction)
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
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