haematology,
Chronic Lymphocytic Leukaemia (CLL)
Sep 09, 2020
Mnemonic
- LSAT (used for Rai scoring system as well as signs/symptoms)
Rai scoring system
- Stage 0: High WBC Stage 1: Lymphadenopathy Stage 2: Splenomegaly + Hepatomegaly Stage 3: Anemia Stage 4: Thrombocytopenia
Introduction
- Progressive accumulation of malignant clone of functionally incompetent B-lymphocytes (abnormal proliferation of abnormal cells)
- affects older patients (in 60s )
Signs and symptoms
- Constitutional symptoms but can have no signs
- Signs as per mnemonic above
Tests
- High lymphocytes
- Later: autoimmune haemolysis
- Marrow infiltration causes: anaemia, decreased neutrophils and platelets (as per mnemonic)
Complications
- Autoimmune haemolysis.
- ↑Infection due to low WCC (=↓IgG), bacterial, viral especially herpes zoster
- Marrow failure
Natural history
- ⅓ never progress (or even regress), ⅓ progress slowly, and ⅓ progress actively
- Death is often due to infection or transformation to aggressive lymphoma (Richter’s syndrome)
Management
- Chemotherapy if symptomatic
- Radiotherapy for lymphoadenopathy and splenomegaly
- Steroid for autoimmune haemolysis
- Stem cell transplantation in selected patients
- Supportive: transfusion