1 MBChB V: Imaging Haematology Session 2 MJ Coetzee

2 Table of contents Haematological malignancies –Leukaemias Acute Chronic –Myeloma –Lymphoma Thrombosis & haemostasis –Thrombocytopenia –Haemophilia –DIC

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4 Leukaemias: rough classification Myeloid (granulated cells) –Chronic (myeloproliferative conditions) e.g. Chronic myeloid leukaemia (CML) –Acute myeloid leukaemias arise suddenly, deadly e.g. acute promyelocytic leukaemia Lymphoid (lymphocyte line) –Chronic e.g. Chronic lymphocytic leukaemia Course: years –Acute lymphoblastic arise suddenly, deadly e.g. acute lymphoblastic leukaemias (ALL)

5 Lymphoid Myeloid

6 More mature cells: chronic leukaemias More immature cells: acute leukaemias

7 Myeloproliferative diseases

8 Organomegaly in a patient with a myeloproliferative disease

9 CML Chronic Myeloid Leukaemia Peripheral blood: neutrophils with left shift & eosinophils & basophils Increase in white cell layer in centrifuged blood: “leukaemia”

10 CML: Philadelphia chromosome t(9;22)

11 Target of Gleevec (drug against CML): works on signal transduction The normal tyrosine kinase transmits a signal from the surface to the nucleus in a controlled manner The abn. tyrosine kinase is an ONCOGENE that stimulates the nucleus to divide Intracelllular signal transducers

12 Polycythaemia vera polycythaemia = many cells in the blood; vera = Latin for true (unexplained) Patient of Sir William Osler in whom he first described PV

13 Many RBCs on a blood smear Cellular biopsy in PV

14 CT of oganomegaly Splenomegaly Bone marrow fibrosis Idiopathic myelofibrosis

15 Essential thrombocythaemia

16 Gangrene in essential Thrombocythaemia (abnormal platelet clumping)

17 Acute myeloid leukaemia  Different classifications  FAB (French-American-British)  WHO (world Health Organization)  Can represent different cell lines  Myeloid cells of stages of maturation  Promyelocytes  Erythroblasts  Platelets, etc.

18 Myeloblasts: peripheral blood

19 Myeloblasts with an Auer rod

20 AML Bone marrow packed with blasts Myeloblasts with granules (granules are typical of myeloid cells

21 Lymphoid leukaemias Chronic lymphocytic leukaemia Acute lymphoblastic leukaemia

22 CLL (chronic lymphocytic leukaemia): clinically Cervical lymph nodes Mediastinal lymph nodes

23 CLL bone marrow: infiltrates of mature lymphocytes

24 CLL: peripheral blood with many lymphocytes

25 CLL: laboratory CLL cells express typical antigens op their membranes. These are detected by means of flow cytometry

26 ALL: clinical picture Rapid course Lymphadenopathy Organomegaly Haemorrhages Infections

27 ALL: lymphoblasts

28 Burkitt's lymphoma/leukaemia blasts

29 Myeloma Vague complaints –Bone pain –Fractures –Infections –Malaise Please remember to determine paraproteins in blood and urine 1% of cancers > 10 % of haematologic cancers in the US Annual incidence ± 4 per 100,000 Increase in cases –?enhanced availability and use of medical facilities

30 Monoclonal band of abnormal Ig Normal Myeloma Normal

31 Myeloma Osteopenia Lytic lesions Pathological fractures

32 Myeloma: rouleaux on peripheral blood

33 Myeloma: plasma cells in bone marrow

34 Congested veins resulting from paraproteins, esp. IgM

35 Haemostasis Haemorrhagic diatheses –Hereditary haemorrhagic diatheses Haemophilia Von Willebrand disease –Acquired haemorrhagic diatheses ITP Thromboses –Venous –Arterial

36 Purpura (fine peticchiae)

37 Purpura (ecchymoses): larger bleeds

38 Haemarthrosis in haemophilia

39 Haemophilia: X-rays (ankylosis) Knee Elbow

40 Haemophilia Family tree

41 Haemophilia: repeated bleeds Dark hypertrophic synovium; osteoartritis; osteophytes

42 Dangerous bleeds Bleed in floor of the mouth stretches downward and obstructs the airways Volkman contracture or compartment syndrome of fore arm (hand useless)

43 ITP: bone marrow packed with megakaryocytes

44 TTP: few platelets red cell fragments (always exclude HIV)

45 Superficial thromboses: clinically Skin necrosis

46 DIC: clinical picture

47 DIC: histological picture