Lymphoid Leukaemias Flashcards
What is acute lymphoblastic leukaemia?
Malignancy of the lymphoid cell line affecting B or T lymphocytes causing uncontrolled proliferation of immature blast cells along with marrow failure and tissue infiltration.
What are the risk factors for ALL?
Radiation during pregnancy
Down’s syndrome
Young age
What are the 3 features that ALL cancers are classified by?
This is based on 3 systems
Morphological features under the microscope giving 3 types – L1, L2 and L3
Immunological surface markers used to classify into Precursor T-cell or B-cell
Cytogenetics chromosomal analysis often showing translocations
What are the clinical features of ALL?
Marrow failure – Anaemia, thrombocytopaenia and infection from leukopenia (excepting the malignant line)
Infiltration causing hepatomegaly, splenomegaly, lymphadenopathy, orchidomegaly
CNS involvement resulting in cranial nerve palsies and meningism
Common infections: chest, mouth, perianal and skin leading to bacterial septicaemia. Also, zoster, CMV, measles, candidiasis, and pneumocystis pneumoniae.
DIC
Leukostasis
How should suspected ALL be investigated?
FBC Blood film Cytogenetic analysis Immunophenotyping Bone marrow biopsy and aspiration CXR and CT to look for mediastinal and abdominal lymphadenopathy Lumbar puncture for CNS involvement PCR to detect minimal residual disease after treatment
How should a patient with ALL be managed upon presentation and outside of chemotherapy
Immediate support – blood/platelet transfusion, IV fluids, allopurinol (prevent tumour lysis syndrome) and insert central line
Treat any suspected infection swiftly using the neutropenic regimen
Walking exercises to relieve fatigue
How is a patient with ALL treated with chemotherapy?
Remission induction
Consolidation – high-medium dose regimen over several weeks
CNS prophylaxis (methotrexate or +/- CNS irradiation)
Maintenance for 2 years
Absolute treatment is a matched allogenic marrow transplantation once in 1st remission
Remission common in blood, CNS or testis so examine at follow up
What is the prognosis of children and adults with ALL?
Prognosis
Children – 70-90% cure rate
Adults – 40% cure rate
What are the indicators of poor prognosis in ALL
Older age Male Philadelphia chromosome CNS signs B-cell ALL
What is chronic lymphocytic leukaemia?
Progressive accumulation of a malignant clone of functionally incompetent B cells. Most common type of leukaemia. (very rarely T cell)
What are the risk factors for CLL?
Age > 60 White male Family history Trisomies Deletion e.g. del16p13
Describe the Rai staging of CLL
Rai Stage
Presentation
Survival
1 Lymphocytosis alone >13yrs
2 Lymphocytosis and lymphadenopathy 8yrs
3 Lymphocytosis and spleno or hepatomegaly 5yrs
4 Lymphocytosis and anaemia <110 2yrs
5 Lymphocytosis and platelets <100 1yrs
What are the clinical features of someone with CLL?
Often no presenting symptoms instead an incidental finding on routine FBC showing markedly raised Lymphocytes
Anaemia and infection prone (due to low IgG) (paraparesis)
B Symptoms: weight loss, sweats (rare in CLL)
Anorexia if severe
Enlarged rubbery non tender nodes
Splenomegaly and hepatomegaly
Smudge or smear cells
Late stage
Autoimmune haemolysis (warm type)
Marrow infiltration and failure causing low HB, low platelets and low neutrophils
How should someone with suspected CLL be investigated?
FBC
Blood film showing smear cells
Bone marrow biopsy and aspiration
CD23 and Beta2 Microglobulin correspond with disease bulk
How is CLL managed?
Only consider drugs if symptomatic e.g. significant B symptoms or problematic, lymphadenopthay or hepato-splenomegaly
Chemotherapy Fludarabine, rituximab and cyclophosphamide
Steroids if Autoimmune haemolysis
Radiotherapy to help lymphadenopathy and splenomegaly
Allogenic Stem cell transplantation in carefully selected patients
Supportive care – transfusions, IV IgG
Need vaccines against pneumococcus and haemophilus, also must be very wary of shingles. Otherwise monitor closely for progression to lymphoma.
