Chronic Lymphocytic Leukaemia Flashcards
what is the definition of CLL?
Mature B lymphocyte cancer in the blood
what is the epidemiology of CLL?
Most common leukaemia
Later in life, mainly in elderly
what is the aetiology of CLL?
Mutations, trisomies and deletions influence risk
Pneumonia may be a triggering event
what are the risk factors for CLL?
age over 60 years
male sex
white ethnicity
family history of CLL
what is the pathophysiology of CLL?
Essentially leukaemia is the presence of rapidly proliferating immature blast blood cells (can be precursors of RBCs, platelets or white cells) in the bone marrow that are non functional i.e. defective
• This poses two problems:
- Firstly, the leukaemia cells are dividing rapidly but serve no function so is wasting energy making useless cells and thus there is less energy available to make useful functional cells
- Secondly, due to rapid replication these cells take up a lot of space within the bone marrow meaning there is little space and also food for other cells to grow
• Because of this, the bone marrow is not able to make as many normal functioning cells resulting in fewer functioning blood cells in the blood resulting in the symptoms of leukaemia
• Eventually when there is no longer space in the bone marrow, leukaemia cells will be present in the blood too
CLL is a leukaemia of B lymphocytes
what are the key presentations of CLL?
shortness of breath and fatigue
Lymphadenopathy - enlarged rubbery, non tender
splenomegaly
hepatomegaly
what are the signs of CLL?
Enlarged spleen and liver
Enlarged lymph nodes
what are the symptoms of CLL?
Often no symptoms, anaemia symptoms, anorexia, weight loss, sweats
what are the first line investigations for CLL?
FbC - Normal or low Hb, WBC count high with very high lymphocytes
blood film - smudge cells may be seen in vitro
what are the gold standard investigations for CLL?
fluorescent in situ hybridisation (FISH)
molecular genetic analysis
direct antiglobulin test (DAT)
immunoglobulin levels
what are the differential diagnoses for CLL?
Primary B-cell leukaemias and B-cell lymphomas presenting with leukaemia, and T-cell derived conditions
how is CLL managed?
- Blood transfusions
- HUMAN IV IMMUNOGLOBULINS
- Chemotherapy or Radiotherapy
- Try stem cell transplant
how is CLL monitored?
Observation
Chemo repeat
Ibrutinib
Stem cell transplant
what are the complications of CLL?
- Autoimmune haemolysis
- Increased infection risk due hypogammaglobulinemia (low IgG); bacterial and viral especially herpes zoster
- Marrow failure
what is the prognosis for CLL?
- 1/3 will never progress
- 1/3 progress slowly
- 1/3 progress actively