1. Introduction to Haematology! Elliot Catchpole PCMD Starting with anaemias!

2. Objectives To be able to define what an anaemia is To describe the symptoms of classical anaemia To identify the different aetiologies of anaemia and how to differentiate between them (which investigations to perform) To be able to select an appropriate course of management for anaemia based on the aetiology.

3. Lets begin... What is in blood?

4. CELLS Plasma Platelets PENPEN RBC WBC ROTEINS LECTROLYTES UTRIENTS

5. What is in blood? Red blood cells? RBC KIDNEY EPO RBC ERYTHROBLAST RETICULOCYTE RBC

6. ANAEMIA “A reduction in haemoglobin, either through a decreased RBC mass or increased plasma volume” Measuring RBC activity Haematocrit (or PCV)= % concentration of RBC’s in blood Males = 45 (13-18Hb) Females = 40 (11.5-16Hb)

7. MCV Mean Cell Volume = The size of each RBC Microcytic <76 Normocytic 76-96 MACROCYTIC >96 -IRON deficiency -Thalassaemias -Sideroblastic Chronic Disease Haemolysis B12 and Folate -Alcohol -Liver Disease Megaloblastic Non-Megaloblastic -G6PD Deficiency -Hereditary spherocytosis -Paroxysmal Nocturnal Haemoglobinurea -Autoimmune Haemolysis -Sickle Cell -G6PD Deficiency -Hereditary spherocytosis -Paroxysmal Nocturnal Haemoglobinurea -Autoimmune Haemolysis -Sickle Cell

8. Presentation All present the same! Severity based on HCT ANAEMIA >30 = Asymptomatic 25-30 = Fatigue 20 -25 = Dyspnoea, confusion <20 = MI Fatigue Pallor Light- headedness

9. Iron Deficiency CAUSES = 1) Bleeding 2) Diet (incl. Vit C) *PRESENTATION* = Spoon nails, angular stomatitis (chronic) *INVESTIGATIONS* = RDW TREATMENT = 1) Ferrous Sulphate (PO) 2) Blood Transfusion (IM Fe if cannot tolerate infusion) Serum FeLow Transferrin (TIBC)HIGH FerritinLow

10. Thalassaemia CAUSES: Genetics (Major=homozygote, minor/trait=heterozygote) *PRESENTATION* = (minor) MCV, minor symptoms INVESTIGATIONS – smear – ‘Target cells’ *Electrophoresis* TREATMENT - Trait = No active treatment Major = Lifelong transfusions Serum FeNormal Transferrin (TIBC)Normal FerritinNormal

12. Sideroblastic Anaemia CAUSES = Genetic, Alcohol, Lead, Medications, Vit B6 deficiency INVESTIGATIONS = *Prussian Blue stain* TREATMENT = Genetic/deficiency: Pyridoxine OTHER: Remove cause Serum FeHIGH Transferrin (TIBC)Normal, either FerritinHIGH

13. Chronic Disease Caused by many chronic diseases Treat the underlying cause Serum FeLow Transferrin (TIBC)Low FerritinHIGH

14. Questions A 54 year old women comes to the clinic due to fatigue. FBC shows a decreases HCT (30) and an MCV of 68. What is the most likely dfiagnosis when the following additional features are described? 1)Elevated Red-cell distribution of width and high platelets 2)Low Iron, low TIBC, low reticulocytes. A history of rheumatoid arthritis 3)A very low MCV with few symptoms and an elevated red-cell count. Normal Iron studies.

15. Questions A 23 year old woman has been feeling tired and lethargic for the past 18 months. She occasionally feels dizzy on standing and is generally weak. Otherwise, she is well. FBC showed: Hb – 9.5mg MCV – 69fl Which is the single most appropriate further investigation to confirm the diagnosis? a) Hb electrophoresis b) HBA2 level c) Serum Iron + Ferritin d) Thyroid function tests e) Vitamin B12 + Folate levels

16. Questions A pale 60 year old man presents with feelings of tiredness and light-headedness. MCV – 60fl HCT – 30 His Faecal occult blood test is negative 3 times. What is the next best step in the management of this patient? a) Start iron replacement therapy b) Electrophoresis c) Transfuse d) Colonoscopy e) Peripheral Blood smear

17. D) Colonoscopy There is such a high incidence of microcytic anaemia in colonic malignancy that excluding GI malignancy is very important in this age group. Faecal occult blood is not sensitive enough to exclude colon cancer.