Lymphoid Malignancy Flashcards
What is acute lymphoblastic leukaemia?
Haematological malignancy in lymphoid progenitor/non-differentiated lymphoid cells, resulting in increased production of lymphoblasts
In what age group is acute lymphoblastic leukaemia more common?
Most common childhood cancer
What is the prognosis of ALL in children?
Good, 90% 5 year survival
What is the prognosis of ALL in adults?
Poor, 30–35% 5 year survival
What are risk factors for poor prognosis ALL?
>Age
>WCC
Slow/poor response treatment
Cytogenetics: T(9;22) and t(4;11)
How does ALL present?
Thrombocytopenic bleeding
- Bruising
- Menorrhagia
- Epistaxis
Lethargy/malaise
Anaemia
Dyspnoea
Arthralgia
Recurrent infection: Neutropenia
CNS infiltration
Testicular disease
Weight loss
What investigations are used in ALL diagnosis?
Bone Marrow Biopsy
FBC, within 48 houts in young people with suspicious symptoms
- Increased WCC
Clotting
Blood film
What biopsy sign is present in ALL?
90% B lymphoblasts
How is ALL managed?
Supportive
- Treat infection
- Manage bleeding
Chemotherapy
Stem cell transplantation
Give complications of ALL
Haemorrhage
Thrombosis
Tumour lysis syndrome
What is tumour lysis syndrome?
Cell break down secondary to chemotherapy, resulting in the release of intracellular compounds such as potassium, phosphate and uric acid, resulting in renal failure
What drug is used to prevent tumour lysis syndrome?
Allopurinol
What is chronic lymphocytic leukaemia?
Malignancy characterised by monoclonal proliferation of well-differentiated/mature lymphocytes, resulting in functionally incompetent lymphocytes
What is the most common leukaemia worldwide?
Chronic lymphocytic leukaemia
What age group is CLL more common?
60+
What are the poor prognostic factors for CLL?
Advanced disease (Binet B or C)
Atypical lymphocyte morphology
Rapid lymphocyte doubling time (<12 months)
CD38+ expression
Loss/mutation p53; del 11q23 (ATM gene)
Unmutated IgVH gene status
How does CLL present?
Often asymptomatic at presentation
Recurrent infection
Weight loss
Bleeding
Sweating
Malaise
Abdominal discomfort
Lymphadenopathy, more marked than CML
Splenomegaly
Hepatomegaly
Petechiae
Pallor
What investigations are used in CLL?
Immunophenotyping
Bone marrow biopsy
Lymph node biopsy
FBC
- Warm autoimmune haemolytic anaemia
- Lymphocytosis
Blood film
- Smudge B cells/smear cells
How does Richter’s transformation present?
Lymph node swelling
Fever without infection
Weight loss
Night sweats
Nausea
Abdominal pain
What biopsy sign is seen in CLL?
>30% lymphocytes
What blood film sign is seen in CLL?
Smudge B cells/smear cells
What are the indications for treatment of CLL?
Progressive bone marrow failure
Massive lymphadenopathy
Progressive splenomegaly
Lymphocyte doubling time <6 months or >50% increase over 2 months
Systemic symptoms
Autoimmune cytopenias
How is CLL managed?
Often nothing/watch and wait
Supportive
- Blood transfusions
- Antibiotics
Cytotoxic chemotherapy
Monoclonal antibodies
Novel agents