10. Haematology II: Leukaemia & Lymphomas Flashcards
LOs
1.
what is leukaemia?
2.
what does it result in?
1
- Malignant neoplasms of haemopoietic stem cells
2
- Result in diffuse replacement of bone marrow and normal blood precursor cells by neoplastic cells
- Bone marrow failure leads to anaemia, neutropenia and thrombocytopenia
- Leukaemic cells spill over into blood and may infiltrate organs
how leukaemias classified?
classified based on cell type involved and its maturity:
- cell type
- maturity
what are the classes of leukaemia split into?
- Cell type
~ Myeloid cell line
~ Lymphoid cell line - Maturity
~ Acute > 50% myeloblasts or lymphoblasts in
bone marrow at clinical presentation
~ Chronic cells are more differentiated
myeloid cell line?
lymphoid cell line?
how are leukaemias diagnosed?
- BLOOD FILM
~ Suggests diagnosis
~ Due to presence of Abnormal WCC (white cells)
~ Presence of blasts - BONE MARROW
~ usually confirms diagnosis
~ Hypercellular
~ Numerous blast cells
what can blood film test and bone marrow aspirate tell us?
- Important prognostic indicators
~ Leukaemia type
~ Cell phenotype
~ Chromosomal abnormalities
aetiology of leukaemias
- Ionizing radiation
- Chemicals including benzene and alkylating agents
- Viruses (e.g.. HTLV – human T-cell leukaemic virus)
- Genetic factors (e.g.. Down’s syndrome)
- Acquired haematological disorders such as aplastic anaemia
what can bone marrow infiltration result in?
- can result in failure of other important cell lines
- this can result in:
~ anaemias
~ impacted immune system
~ impact coagulation and platelet function - these over all can thus then cause:
- pallor
- malaise
- fever + infection
- bleeding
- bruising / petechiae
- why may tissue infiltration occur?
- what can tissue infiltration result in/ cause?
1
- neoplastic cells may spill out of the bone marrow into the peripheral blood
- this can lead to infiltration of other organs
2
- Lymphadenopathy
- Hepatosplenomegaly
- Central nervous system
- Bone and joint pain (ALL)
- Testicular swelling (ALL)
- Gingival hypertrophy (AML) (important for dentistry)
names of acute and chronic leukaemias?
ACUTE (immature cell lines) (could be myeloid or lymphoid depending on type of cell)
- Myeloid (AML = acute myeloid leukaemia)
- Lymphoid (ALL = acute lymphoblastic leukaemia)
CHRONIC (typically more mature cells)
- Myeloid (CML = chronic myeloid leukaemia)
- Lymphoid (CLL = chronic lymphocytic leukaemia)
ALL - Acute lymphoblastic leukaemia
1
age of peak incidence
2
management
1
Children - peak incidence 4-5 years
2
- Remission induced with non-myelosuppressive chemotherapy
- 2-12 years >60% cure rate with chemotherapy
- Adults 20% cure rate
- Combination chemotherapy to induce remission
- CNS treatment performed prophylactically
- Maintenance therapy for up to 2 years increases disease-free survival
AML - Acute myeloid leukaemia
1
incidence at what age most likely?
2
management?
1
- Most common leukaemia in adults
- Increasing incidence with age
- X400 higher incidence in children with Down’s syndrome
2 Management
- >80% cure rate with intensive chemotherapy in young patients
- 15% resistant disease
- 4-5 courses of intensive chemotherapy (each lasting 5-10 days)
- No maintenance therapy
- Autologous and allogenic stem cell transplant (if fail chemotherapy)
CLL - Chronic lymphocytic leukaemia
1
common age?
Male : female incidence?
2
clinical features
1
- 25 % of all leukaemias
- Elderly (>60years)
- M:F ~ 2:1
2
- Constitutional symptoms
- Lymphadenopathy
- Splenomegaly
- Recurrent infections
- Abnormal FBC (anaemia, thrombocytopenia)
- Hypogammaglobinaemia
CLL - Chronic lymphocytic leukaemia
treatment?
- Asymptomatic patients do not require treatment (monitoring only)
- 30% of patients with early stage disease die of unrelated cause
- Chemotherapy typically effective
- Median survival 10-12 years
- Mortality usually due to infection or bone marrow failure
- Bone marrow transplantation occasionally attempted in younger patients with poor prognostic disease
CML - Chronic myeloid leukaemia
what is philadelphia chromosome?
what did it lead to?
- > 90% have ‘Philadelphia chromosome’
- ‘Balanced translocation’ between 9 and 22
- Resultant oncogene with tyrosine kinase activity
- Lead to first targeted therapy for leukaemia – imatinib (tyrosine kinase inhibitor)
Hodgkin’s disease lymphomas
1
peak incidence age?
2
annual incidence (M + F)?
3
aetiology?
1
- Peak incidence in third decade
- Possible bimodal age with second peak adults> 60yrs)
2
Annual incidence
Male - 3 per 100,000
Female - 1.8 per 100,000
3
Aetiology unknown
EBV (infectious mononucleosis) suggested
how is Hodgkin’s disease lymphomas staged?
- staged based on Ann Arbor system
- I Single LN region
- II Two LN regions
- III Groups on both sides of
diaphragm - IV Widespread disease outside
lymphatic tissues - B symptoms
~ Weight loss
~ Night sweats
~ Unexplained fever
DENTAL RELEVANCE OF LEUKAEMIAS + LYMPHOMAS
Leukaemic infiltration?
- in some patients leukaemic cells spill out of bone marrow and deposit in various tissues
- this can include gingiva + bone
- Typically gingival however also bone infiltration
- 3.6% of dentate patients
-c18.5% in AML
- Friable and haemorrhagic
- Increased tooth mobility
DENTAL RELEVANCE OF LEUKAEMIAS + LYMPHOMAS
1
Intraoral lymphomas
2
management
- can develop intraorally
- Typically non-Hodgkin lymphomas (NHL) associated with HIV
- Most commonly affected sites are the fauces and gingivae
- Typically present as rapidly enlarging masses with bone destruction
2
- Chemotherapy
- Radiotherapy
Haematopoietic stem cell transplant (HSCT)
Graft vs host disease
- Serious complication of allogenic HSCT
- Divided into acute and chronic GvHD based on clinical findings
- Oral cGvHD most of most common presentations
- Characterised by lichenoid inflammation - particularly on the tongue and buccal mucosa
- Salivary glands may also be involved with hypofunction (major glands) and recurrent mucoceles (minor glands)
MANAGEMENT
- Lichenoid treated with topical steroids
- Xerostomia treated in same way as any other cause
