Chronic Lymphocytic Leukaemia Flashcards
It is the most common leukaemia in western countries. True or false?
True (world wide = ALL)
Who does it typically affect?
Elderly Caucasian males
Older than 60
Describe the pathophysiology
A progressive accumulation of a malignant clone of functionally incompetent B cells
Morphological mature cells that are incompetent
What causes it?
Acquired mutations, trisomies and deletions
How does CLL present?
Often asymptomatic and discovered incidentally
Marrow failure and immunosuppression- anaemia, frequent infections
Painless lymphadenopathy
Hepatomegaly/ splenomegaly
B symptoms: fever, chills, night sweats, weight loss
What is seen on FBC?
Raised WCC (persistent lymphocytosis) If advanced: low Hb and thrombocytopenia
What can be seen on peripheral blood film?
Smudge cells - mature lymphocytes that rupture easily
What would bone marrow biopsy show?
More than 30% small, mature lymphocytes
But not necessary to do BM biopsy for diagnosis
What does immunophenotyping show?
Mainly CD19/20 and CD5+ B cells
What complications can occur?
Warm autoimmune haemolytic anaemia
Hypogammaglobulinaemia - leading to recurrent infections (bacterial or viral - especially herpes zoster)
Transformation to high grade lymphoma = Richter’s transformation
What is Richter syndrome?
When CLL transforms into an aggressive, large B cell lymphoma
EBV May pay part in transformation
How does Richter syndrome present?
Weight loss Fever Night sweats Muscle wasting Increasing hepatosplenomegaly Lymphadenopathy
What test may be positive if there is haemolysis?
Coombs’ test
What staging system is used?
The Rai system
Describe the Rai staging system
Stage 0 - low risk - lymphocytosis alone - median survival >13 yrs
Stage l - intermediate risk - lymphocytosis and lymphadenopathy- 8 years
Stage II - intermediate risk - lymphocytosis, lymphadenopathy, hepato or splenomegaly - 5 years
Stage III - high risk - lymphocytosis, anaemia, lymph nodes, liver and spleen may or may not be enlarged - 2 years
Stage IV - high risk - lymphocytosis, thrombocytopenia, enlarged lymph nodes, liver or spleen, may have anaemia
