Week 4 – Handout.docx

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Week 4 – Handout 1. Normal to decreased Hb, Increased RBC, Decreased MCV & MCH, moderate microcytosis, Mild target cells and elliptocytes, mild polychromasia and basophilic stippling. HbF slightly increased, HbA1 present. What is your provisional diagnosis?  Also called β-Thalassaemia trait  One of the two genes that produce β-globin chains is defective (heterozygous)  Asymptomatic  Often confused with iron d

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  Week 4  –  Handout 1. Normal to decreased Hb, Increased RBC, Decreased MCV & MCH, moderate microcytosis, Mild target cells and elliptocytes, mild polychromasia and basophilic stippling. HbF slightly increased, HbA1 present. What is your provisional diagnosis?     Also called β -Thalassaemia trait    One of the two genes that produce β -globin chains is defective (heterozygous)   Asymptomatic   Often confused with iron deficiency anaemia Hb Normal -Decreased RBC Increased(Marrow compensating for ineffective haemopoiesis) MCH & MCV Decreased Hypochromasia Mild Anisocytosis Moderate Microcytes Poikilocytosis MildTarget Cells +, mild elliptocytes Immature Forms Polychromasia, basophilic stippling (wrights stain)    Clinically well except when: Stressed, Infected or Pregnant 2. 65 y old female, transfusion dependant. Hb is 85 g/L. MCV and MCH is low. RBC count slightly low. Dimorphic blood film observed. Comment on the morphology. What are the characteristic features you find in this film? What is your provisional diagnosis? What would be your further tests to confirm your diagnosis? β -Thalassaemia major    Chronic haemolysis due to excess α chains that precipitate in the red cell forming Heinz bodies   Bone marrow expands deforming bones   Hepatomegaly & splenomegaly (extramedullary haemopoiesis)   Possible gallstones, gout & jaundice with Chronic haemolysis   Not usually detected until 6 months of age   If not treated in childhood, classic thal develops within years   ß Thal patients depend on transfusions, but excess iron from transfusions may lead to cardiac and hepatic problems   More iron is absorbed from the gut leading to Fe overload   Largely overcome by the use of desferroxamine   Cardiac failure can occur due to the constant high output of blood required to maintain an adequate perfusion of tissues Hb Low RCC Normal  – Increased(Marrow compensating for ineffective haemopoiesis) MCH & MCV Low Hypochromasia Markedly low Anisocytosis Marked (Macrocytes, Microcytes) Poikilocytosis Marked target cells,TearDrops, Schistocytes Acanthocytes, Howell Jolly Bodies, Target Cells (post splenectomy) Immature Forms Moderate polychromasia, nRBC(bone marrow response)  3. Fill in the genotype possibilities for Alpha thalassaemia: One gene: Two genes:  Alpha thal major/HbH disease: Hydrops foetalis: 4. Young African-American women presented at the GP for regular health check. Blood film morphology indicated moderate microcytosis, hypochromia, anisocytosis and target cells. HbS was 35% and HbA 62%. What is your most likely diagnosis?   5. 46-year-old male recently moved from the Middle East to Alaska. Fainted on a camping trip. On examination at the ED, patient was finding it difficult to breathe and had splenomegaly. Blood film morphology shows evidence of haemolysis. Antibody screening cells negative at 37C. What should the transfusion scientist ’s  next move be? 6. What is the characteristic morphological feature of RBC you see in this film. What is the provisional diagnosis? Explain its pathophysiology.
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