1. Haematology Aaqid Akram MBChB (2013) Clinical Education Fellow

2. Objectives Understand the pathophysiology behind haematological disorders Understand how to recognise and initiate management for haematological disorders

5. Haemoglobin

6. Hereditary Anaemias Disorder of structure/synthesis of Hb Enzyme Deficiencies for energy Red cell membrane protein abnormalities

8. Sickle Cell Disease Homozygous – Sickle Cell Anaemia Heterozygous – Sickle Cell Carrier Red sickling during oxygenation Amino acid substitution in Beta globin chain Increased rigidity Increased aggregation in microcirculation Haemolytic anaemia + tissue infarction Sickle cell gene spread through mainly Africa

10. Treatment / Prevention Pregnant + at risk groups Painful Crisis Pulmonary Sequestration Stroke Aplastic Crisis Splenic Sequestration Hydroxyurea Sickle Cell + Beta Thalassaemia Annual ophtalmology

11. Thalassaemia Alpha / Beta Protected against malaria Similar distribution to sickle cell disease Homozygous (Major) Severe anaemia Variability in red cell size and shape Hypochromic Target cells Splenomegaly Iron Loading Heterozygous (Trait) Mild hypochromic microcytic anaemia Subclinical otherwise

12. Treatment / Prevention Pregnant + at risk groups Blood Transfusions Iron Overload Splenomegaly

13. Myeloproliferative disease Abnormal growth of blood cells in the bone marrow Polycythaemia Rubra Vera (red cells) Essential Thrombocythaemia (platelets) Idiopathic Myelofibrosis Tear drops / leukoerythroblasts Extramedullary haemopoeises (liver/spleen) BM transplant if poor prognosis Haemorrhage, infection, acute leukaemia

16. Chronic Myeloid Leukaemia (CML) Malignant myeloproliferative disorder Single abnormal haemopoetic stem cell Still function similar to normal blood cells Proliferation over months/years takes over normal blood cell production Philadelphia chromosome (22q-) [cytogenetic studies] Imatinib / Interferon alpha Allogenic / autologous stem cell transplantation

17. Acute Leukaemia

20. Same day specialist referral Chemotherapy induce remission initially Consolidate leukaemic cell population Supportive care Blood products Abx BM transplant

21. Myelodysplasia Impaired BM function May progress to AML (30%) Risk Factors Radiotherapy Alkylating agents (4-10 years) Benzene Smoking Petroleum Neurofibromatosis type 1

22. Treatment / Prevention Likely palliative Supportive Blood Transfusions Abx Granulocyte colony stimulating factor (GCSF) EPO Immunosuppression Chemotherapy Allogenic Stem Cell Transplant

23. Myeloma Tumour of monoclonal plasma cells Proliferation Accumulate in BM Anaemia BM Failure Bone Destruction Monoclonal antibody (paraprotein) overproduction Detectable in serum / urine (Bence Jones)

24. Hodgkin’s Lymphoma Malignant tumour of lymphatic system Histology – Reed Sternberg Cells B lymphocytes Staging determines treatment Enlarged lymph nodes Lymph node biopsy Ann Arbor staging Management Chemotherapy / Radiotherapy / Stem Cell Transplant

25. Non-Hodgkin’s Lymphomas x5 more common than Hodgkins Lymphoma Low grade Subclinical Painless + slowly progressive lymphadenopathy High grade Systemic symptoms Rapidly growing bulky lymphadenopathy B Cell / T Cell H. Pylori – MALT EBV – Burkitt’s Lymphoma Chemotherapy / Radiotherapy / Stem Cell Transplant

27. Chronic Lymphocytic Leukaemia (CLL) Abnormal B lymphocytes (monoclonal) Normal appearance Immature and non reactive Immunological compromise Subclinical (90%) Infections / Enlarged lymph nodes / Bleeding Smudge/Smear Cells Chemotherapy / Stem Cell Transplant / Splenectomy

28. So Basically…. Figure out which branch of haematopoeisis is affected Is it proliferative or dysplastic? All will have anaemia symptoms All will require supportive treatment Almost all will have management regimes including Chemotherapy / Radiotherapy / Stem Cell Transplant +/- Splenectomy If you need to give regular transfusions then don’t forget iron chelation therapy