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 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
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
Sickle cell disease and sickle cell trait
• painfull crisis with analgesics
• rigorous dental care necessary due to ↑
susceptibility to infection
The thalassaemias
• α-thalassaemias Asians, Africans and AfroCaribbean
• 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
Leukopenia and agranulocytosis
agranulocytosis
• clinical effects of severe neutropenia: fever
prostration, mucosal ulceration
• drug induced leukopenias
• tab 22.12
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
Haemorrhagic diseases
Haemorrhagic diseases
• haemorrhagic diseases = purpura (platelet
deffects) and clotting deffects
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
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)
Haemorrhagic diseases
• Laboratory investigations:
– tab 23.2
– essential is look for anaemia
– blood grouping
Haemorrhagic diseases
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
Haemorrhagic diseases
– typical site palate
– +/- excessive gingival bleeding or blood blister
– tab 23.3
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
Haemorrhagic diseases
• management:
– corticosteroids
– transfusions of platelets
– anti…???
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
Haemorrhagic diseases
localised oral purpura
• sometimes blood blister without haemostatic
defect
• choking sensation („angina bullosa
haemorrhagica“)
• rupture → ulcer
• systemic purpura should be excluded
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
Haemorrhagic diseases
B) Clotting disorders
• tab 23.5
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
Haemorrhagic diseases
• 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
Haemorrhagic diseases
– aspirin and related analgesics avoided!
– extractions in mild haemophilia with
antifibrinolytic drugs
Haemorrhagic diseases
Christmas disease (haemophilia B)
• fIX
• inherited
• more stable → replacement therapy in longer
intervals
• other the same as in haemophilia A
Haemorrhagic diseases
Acquired clotting defects
a) vitamin K deficiency
• causes: obstructive jaundice, malabsorption
• surgary delayed to haemostasis recover
• +/- vitamin K
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
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
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
Cervical lymphadenopathy
• dental and periodontal infections most
common cause
• lymphomas
• HIV infection
• tab 26.1
• investigation: recent viral illness –
lymphadenopathy resolves after some months
Cervical lymphadenopathy
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
Cervical lymphadenopathy
Syphilis
• lymph nodes enlarged, soft and rubbery
• primary or secondary stage
• Treponema pallidum in a direct smear or by
serological finding
• management: atb
Cervical lymphadenopathy
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
Cervical lymphadenopathy
• management: history, clinical features,
exclusion of other causes, disease is mild and
self limiting, +/- suppuration and sinus
formation
Cervical lymphadenopathy
Lyme disease
• transmitted by insects, deer ticks
• tab 26.5
• management: history + clinical picture
• confirmed serologically
• atb!
Cervical lymphadenopathy
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!
Cervical lymphadenopathy
AIDS
• soon after infection transient glandular fever
like-illness
• later +/- wide spread lymphadenopathy (GLS)
• → AIDS
Cervical lymphadenopathy
Toxoplasmosis
• intestinal parasite of many domestic animals
(cats)
• management: serologically, antimicrobial
treatment
Cervical lymphadenopathy
Mucocutaneous lymph node syndrome
(Kawasaki´s disease)
• tab 26.8
• management: clinical and ECG finding
• aspirin, γ-globulin
Cervical lymphadenopathy
Drug-associated lymphadenopathies
• occasional toxic effect of long term treatment
with the antiepileptic drug, phenytoin can
mimic lymphoma
• management: