HAEMATOPATHOLOGY

1. HAEMATOPATHOLOGY

2. Anaemias and leukaemias

3. Anaemia • types (etiology): • 1) iron deficiency • most common type • chronic menstrual blood loss, peptic ulcer, haemorrhoids • 2) pernicious anaemia • macrocytic anaemia • +/- neurological disease • folate insufficiency

4. Anaemia • 3) leukaemia • cause of normocytic anaemia • childhood! • 4) sickle cell trait • 5) thalasaemia

5. Anaemia • clinical features • tab 22.2, 3, 4 • mucosal disease • glossitis • recurrent aphthae • candidiosis and angular stomatitis

6. Anaemia • dangers of general anesthesia • any reduction of oxygenation → irreparable brain damage, myocardial infarction → gen. anesthesia should be provided in hospital • lowered resistence to infection • oral candidiosis • osteomyelitis

7. Sickle cell disease and sickle cell trait • people of African, Afro-Caribbean and Mediterranean or Middle Eastern origin • sickle cell diease = homozygotes • sickle cell trait = heterozygotes • abnormal Hb (HbS) with the risk of haemolysis, anaemia and other effects • in heterozygotes sufficient normal Hb (HbA) is formed to allow normal life

8. Sickle cell disease and sickle cell trait Sickle cell disease: • complications from polymerisation of deoxygenated HbS (less soluable than HbA) • → chronic haemolysis → chronic anaemia • exacerbation of sickling raises blood viscosity → blocking of capillaries and sickling crisis • tab 22.5 • + abnormal susceptibility to infections (Pneumococcal, Meningococcal) and osteomyelitis

9. Sickle cell disease and sickle cell trait dental aspects of sickle cell disease and s.c.trait: • Hb ≤ 10g/dl → v. s. homozygote • s.c. trait: gn anaesthesia with full oxygenation s.c. disease: • +/- oral mucosa pale or yellowish due to jaundice • +/- radiographics changes in skull and jaws • prompt atb treatment

10. Sickle cell disease and sickle cell trait • painfull crisis with analgesics • rigorous dental care necessary due to ↑ susceptibility to infection

11. The thalassaemias • α-thalassaemias Asians, Africans and Afro-Caribbean • ß-thalassaemias Mediterranean (Greeks) • diminished synthesis of globin chains → resulting relative excess of other chains → precipitation in ery → +/- haemolysis • severity of disease depends on the numbers of affected genes • minor = heterozygotes • major = heterozygotes

12. The thalassaemias thalassaemia minor: • mild, but persistent microcytic anaemia, otherwise asymptomatic • +/- splenomegaly

13. The thalassaemias thalassaemia major: • severe hypochromic, microcytic anaemia • great enlargement of liver and spleen • skeletal abnormalities (marrow expansion) • life saving transfusions, but iron depositions in tissues → haemosiderosis → dysfunction of glands and other organs → xerostomia

14. Leukaemia • leukaemic white blood cells production → supress of other cell lines of the marrow

15. Leukaemia acute leukaemia • ALL most common leukaemia of children • AML in adults • tab 22.7 • splenomegaly, hepatomegaly, +/- lymphadenopathy • mucosal pallor, abnormal gingival bleeding • tab 22.8

16. Leukaemia • management: • biopsy of gingival swelling • vigorous oral hygiene to controll the bacterial population before complications develop • extractions avoided, if necessary – blood transfusion, generous atb cover

17. Leukaemia • chronic leukaemia

18. Leukopenia and agranulocytosis leukopenia • WBC ≤ 5000³/l • different causes tab 22.10 • chance haematological finding x severe - immunodeficiency

19. Leukopenia and agranulocytosis agranulocytosis • clinical effects of severe neutropenia: fever prostration, mucosal ulceration • drug induced leukopenias • tab 22.12

20. Leukopenia and agranulocytosis aplastic anaemia • failure of production of all bone marrow cells (pancytopenia) • systemic and oral effects: purpura, anaemia, susceptibility of infection • cause: unknown, ai, drug induced • management: stop drugs, give atb and transfusions

21. Haemorrhagic diseases

22. Haemorrhagic diseases • haemorrhagic diseases = purpura (platelet deffects) and clotting deffects

23. Haemorrhagic diseases • Investigation of a history of excessive bleeding: • careful history essential tab. 23.1 • most of the haemorrhagical diseases are hereditary! • bleeding for up to 24hrs after an extraction usually due to local causes or a minor defect of haemostasis → more prolonged bleeding is significant

24. Haemorrhagic diseases • Clinical examination: • signs of anaemia and purpura • examination of the mouth → planning of the operation • haemophilia – all essential extractions carried out at a single operation with fVIII cover • radiographs (to prevent complications)

25. Haemorrhagic diseases • Laboratory investigations: • tab 23.2 • essential is look for anaemia • blood grouping

26. Haemorrhagic diseases A) Purpura • typical result of platelet disorders • bleeding time prolonged but clotting function normal (with exception of of vW disease)

27. Haemorrhagic diseases • general features of purpura: • purpura = bleeding into the skin or mucous membranes causing petechiae or ecchymoses or „spontaneous bruising“ • haemorrhage immediately follows the trauma and ultimately stops spontaneously as a result of normal coagulation • thrombocytopenia = platelets ≤ 100 000 mm³ • spontaneous bleeding uncommon until platelets ≤ 50 000 mm³

28. Haemorrhagic diseases • typical site palate • +/- excessive gingival bleeding or blood blister • tab 23.3

29. Haemorrhagic diseases ITP • IgG auto Ab • ↓ number of platelets • children or young adult women • first sign could be profuse gingival bleeding or postextraction haemorrhage • +/- spontaneous bleeding into the skin

30. Haemorrhagic diseases • management: • corticosteroids • transfusions of platelets • anti…???

31. Haemorrhagic diseases AIDS • ai thrombocytopenia can be early sign drug associated purpura • aspirin + others interfere with platelet function • others act as haptens → immune destruction of platelets or suppress marrow function • tab 23.4

32. Haemorrhagic diseases localised oral purpura • sometimes blood blister without haemostatic defect • choking sensation („angina bullosa haemorrhagica“) • rupture → ulcer • systemic purpura should be excluded

33. Haemorrhagic diseases von Willebrand´s disease • both by prolonged bleeding time and deficiency of fVIII • usually inherited, AD • deficiency of fVIII mild → purpura more common manifestation

34. Haemorrhagic diseases B) Clotting disorders • tab 23.5

35. Haemorrhagic diseases Haemophilia A • most common, severe • fVIII deficiency • 6/100 000 • severe haemophilia typically effects in childhood – bleeding into muscles or joints after minor injuries • mild haemophilia (fVIII ≥ 25%) – no symptoms until an injury, surgery or dental extraction

36. Haemorrhagic diseases • severe and prolonged bleeding can also follow local anaesthetic injections! (inferior dental blocks!)

37. Haemorrhagic diseases • clinical features: • positive family history • 30% patients negative history! • bleeding starts after a short delay (normal platelet and vascular responses) → persistent bleeding, can continue for weeks • haemarthroses • intracranial haemorrhage! • deep tissue bleeding → obstruction of airways! • HBV, HCV+! • +/- formation of anti fVIII Ab

38. Haemorrhagic diseases • principles of management: • radiographs (local status, prevention of complications) • admission to hospital • replacement therapy • as much surgical work as possible in one session 23.6 • for dental extraction fVIII level 50-75% • postoperatively: atb, risk of bleeding greatest 4-10 days postoperatively

39. Haemorrhagic diseases • aspirin and related analgesics avoided! • extractions in mild haemophilia with antifibrinolytic drugs

40. Haemorrhagic diseases Christmas disease (haemophilia B) • fIX • inherited • more stable → replacement therapy in longer intervals • other the same as in haemophilia A

41. Haemorrhagic diseases Acquired clotting defects a) vitamin K deficiency • causes: obstructive jaundice, malabsorption • surgary delayed to haemostasis recover • +/- vitamin K

42. Haemorrhagic diseases b) anticoagulant treatment • coumarin (warfarin) • dental extraction save with INR 2-3 • few teeth extracted in one session, trauma should be minimal, sockets can be sutured • anticoagulation should not be stopped • for large surgery → stopped with agreement of physician • short term: heparin (acts only about 6hrs) → surgery delayed for 12-24hrs

43. Haemorrhagic diseases c) liver disease • obstructive jaundice • extensive liver damage (viral hepatitis, alcoholism) • haemorrhage can be severe and difficult to control • → vitamin K • antifibrinolytic agents • fresh plasma infusion

44. Lymphomas

45. Lymphomas • any type of lymphocytes, most frequently B cells • all malignant • Hodgkin + non Hodgkin lymphomas (NHL) • relatively frequently involve cervical lymph nodes x rare in the mouth

46. Lymphomas A) NHL • adults predominantly affected • nondescript, soft, painless swelling +/- ulcerated • histologically: • + invasion of adjacent tissues • + if traumatised – inflammatory cells can obscure the lymphomatous nature of the tu

47. Lymphomas • management: • biopsy! • staging!

48. Lymphomas • Burkitt´s lymphoma • nasopharyngeal (T cell) lymphoma – mlg midline granuloma • MALT! • + local manifestation of gn disease

49. Cervical lymphadenopathy

50. Cervical lymphadenopathy • dental and periodontal infections most common cause • lymphomas • HIV infection • tab 26.1 • investigation: recent viral illness – lymphadenopathy resolves after some months