ACUTE LYMPHOCYTIC LEUKAEMIA/LYMPHOMA Flashcards
ALL results from the clonal proliferation of cells from the earliest stages of lymphoid maturation (i.e. B or T blast cells).
________ of ALLs are B-lineage and _______ are T-lineage.
Can also be classified as a _______________.
It is the most common childhood malignancy and has its peak incidence in childhood. 85% of children are cured but the prognosis is worse with increasing age.
TdT is positive in ALL and absent in myeloid blast and mature lymphocytes.
- TdT is a cellular marker for ______________
- ALL is associated with _______________
85%;
15%
non-Hodgkin lymphoma
pre-T and pre-B cells
Down syndrome after the age of 5.
What is the difference between lymphocytic leukaemia and lymphoma?
Lymphocytic Leukaemia
- Disease starts from lymphocyte in BM
- Can spread to LN and Spleen
- Mature or Immature depends on Acute / Chronic
Lymphoma
- Disease starts from Lymphocyte in Lymphatics
- Then spreads to Spleen / Liver
- Can spread to BM (in stage IV)
- Usually mature B / T cells
What are the symptoms of ALL?
Anemia: Weakness, tiredness, malaise
Thrombocytopenia: easy bruising and bleeding
Neutropenia: Otitis media, pharyngitis, pneumonia, fever
Tissue Infiltration (more common in ALL than AML)
- Enlarged lymph nodes
- Thymic mass in a teenager (T-ALL): especially in lymphoblastic lymphoma
Bone pain
- CNS involvement leading to ICP (headache and vomiting)
- Testes
- Hepatosplenomegaly
Leukostasis: dyspnoea, headache, TIA, Stroke, confusion, visual problems
Release of cellular contents (is a possible complication): Tumor Lysis Syndrome
What are the signs of ALL?
- Pallor
- Petechial haemorrhage
- Lymphadenopathy
- Hepatosplenomegaly
- Bone tenderness
- Effects of Tumor Lysis Syndrome causing hyperuricemia (Acute Kidney Failure, Gout)
- Fever
- Testicular enlargement
What are the investigations performed for ALL?
Peripheral Blood Film: Anaemia, neutropenia, thrombocytopenia, circulating blast cells
Bone Marrow Aspirate: Lymphoblast infiltration >20%.
Immunophenotyping
- All ALLs are TdT positive.
- B-cell leukaemia blast cells have a phenotype defined by the presence of CD 10, CD19 and CD20.
- T-cell lymphoblast express markers from CD2 to CD8. CD10 NOT expressed.
Cytogenetic analysis: to confirm subgroups to determine management and prognosis
Blood group, LFTs, creatinine, electrolytes, calcium, phosphate, uric acid, coagulation screen
What is the prognosis of ALL?
For ALL, prognosis is very dependent on cytogenetic subgroups, particularly for B linage ALL.
Good prognosis: Hyperploidy (increase in number of chromosomes), t(12;21) -> more common in children, t(1;19)
Poor prognosis: Hypoploidy, Philadelphia chromosome t(9;22), t(4;11)
What is the management of ALL?
Supportive care (similar to AML)
- Central venous catheter
- Red blood cell and platelet transfusions
- Broad spectrum antibiotics for fever
- Prophylaxis for Pneumocystis
- Leukapheresis – Extreme leukocytosis (WBC > 200): leukapheresis
If Cx by TLS
- Hyperuricemia: hydration, allopurinol (if pre-Tx) / Rasburicase (after TLS onset)
- Hyperphosphatemia; aluminum hydroxide, calcium acetate / carbonate
- Hyperkalemia: fluids, diuretics
- Sometimes Haemodialysis if AKI, or severe Electrolyte derangements
Definitive Mx: Systemic Chemotherapy
- Induction cycle to get the patient into remission (blast count in BM has reduced sufficiently)
- Intensification/consolidation cycles
- Early intrathecal chemotherapy for ALL (to treat occult CNS disease)
- Requires prophylaxis to scrotum
- Prolonged continuation/ maintenance phase
- Two to three years of therapy
Definitive Mx: Targeted Therapy
- Ph-positive need special treatment with imatinib (targets genetic abnormality associated): Tyrosine Kinase Receptor Inhibitor
Treatment must be tailored to the prognosis
Definitive Mx: Allogenic hematopoietic stem cell transplant: consider if poor prognosis
