Haemathopathology.ppt

HAEMATOPATHOLOGY

Anaemias and leukaemias

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

Anaemia

• 3) leukaemia– cause of normocytic anaemia– childhood!

• 4) sickle cell trait• 5) thalasaemia

Anaemia

• clinical features• tab 22.2, 3, 4

• mucosal disease• glossitis• recurrent aphthae• candidiosis and angular stomatitis

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

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

Sickle cell disease and sickle cell traitSickle 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


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


• painfull crisis with analgesics• rigorous dental care necessary due to ↑

susceptibility to infection

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

The thalassaemias

thalassaemia minor:• mild, but persistent microcytic anaemia,

otherwise asymptomatic• +/- splenomegaly

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

Leukaemia

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

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

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

Leukaemia

• chronic leukaemia

Leukopenia and agranulocytosis

leukopenia • WBC ≤ 5000³/l• different causes tab 22.10• chance haematological finding x severe -

immunodeficiency


agranulocytosis• clinical effects of severe neutropenia: fever

prostration, mucosal ulceration

• drug induced leukopenias• tab 22.12


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

Haemorrhagic diseases


• haemorrhagic diseases = purpura (platelet deffects) and clotting deffects


• 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


• 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)


• Laboratory investigations:– tab 23.2– essential is look for anaemia– blood grouping


A) Purpura• typical result of platelet disorders• bleeding time prolonged but clotting function

normal (with exception of of vW disease)

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


– typical site palate– +/- excessive gingival bleeding or blood blister – tab 23.3


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


• management: – corticosteroids– transfusions of platelets– anti…???


AIDS• ai thrombocytopenia can be early signdrug associated purpura• aspirin + others interfere with platelet

function • others act as haptens → immune destruction

of platelets or suppress marrow function• tab 23.4


localised oral purpura • sometimes blood blister without haemostatic

defect • choking sensation („angina bullosa

haemorrhagica“)• rupture → ulcer• systemic purpura should be excluded


von Willebrand´s disease• both by prolonged bleeding time and

deficiency of fVIII• usually inherited, AD• deficiency of fVIII mild → purpura more

common manifestation


B) Clotting disorders• tab 23.5


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


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

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

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


– aspirin and related analgesics avoided!– extractions in mild haemophilia with

antifibrinolytic drugs


Christmas disease (haemophilia B)• fIX• inherited• more stable → replacement therapy in longer

intervals• other the same as in haemophilia A


Acquired clotting defectsa) vitamin K deficiency• causes: obstructive jaundice, malabsorption • surgary delayed to haemostasis recover• +/- vitamin K

Haemorrhagic diseasesb) 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


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

Lymphomas

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

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

Lymphomas

• management:– biopsy!– staging!

Lymphomas

• Burkitt´s lymphoma• nasopharyngeal (T cell) lymphoma – mlg

midline granuloma• MALT!• + local manifestation of gn disease

Cervical lymphadenopathy


• dental and periodontal infections most common cause

• lymphomas• HIV infection• tab 26.1• investigation: recent viral illness –

lymphadenopathy resolves after some months


TBC• Mcb tuberculosis + atypical Mcb• clinical features:• pathology: granulomas, Mcb → Mcb culture

or DNA tests• management: suspicion of TBC – affected

nodes should be excised intact


Syphilis• lymph nodes enlarged, soft and rubbery• primary or secondary stage• Treponema pallidum in a direct smear or by

serological finding• management: atb


Cat scratch disease• tab 26.4• pathology: destruction of lymph node

architecture, necrosis and lymphocytic infiltration, formation of histiocytic granulomas and central suppuration

• WS staining• x deep mycoses


• management: history, clinical features, exclusion of other causes, disease is mild and self limiting, +/- suppuration and sinus formation


Lyme disease• transmitted by insects, deer ticks• tab 26.5• management: history + clinical picture• confirmed serologically• atb!


Infectious mononucleosis• self-limiting lymphoproliferative disease• tab 26.6• +/- more persistent lymphadenopathy which

may mimic a lymphoma• management: peripheral blood picture (atypical

lymphocytes), Paul-Bunnell test, anti EBV Ab, ampicillin or amoxicilin should be avoided!


AIDS• soon after infection transient glandular fever

like-illness• later +/- wide spread lymphadenopathy (GLS)• → AIDS


Toxoplasmosis• intestinal parasite of many domestic animals

(cats)• management: serologically, antimicrobial

treatment


Mucocutaneous lymph node syndrome (Kawasaki´s disease)

• tab 26.8• management: clinical and ECG finding• aspirin, γ-globulin


Drug-associated lymphadenopathies• occasional toxic effect of long term treatment

with the antiepileptic drug, phenytoin can mimic lymphoma

• management:

Haemathopathology.ppt

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