What are the main clinical features of Chronic lymphocytic leukaemia?

• often none: may be picked up by an incidental finding of lymphocytosis
• constitutional: anorexia, weight loss
• bleeding, infections
• lymphadenopathy more marked than chronic myeloid leukaemia

What is the management of Chronic lymphocytic leukaemia?

• patients who have no indications for treatment are monitored with regular blood counts
• fludarabine, cyclophosphamide and rituximab (FCR) has now emerged as the initial treatment of choice for the majority of patients
• ibrutinib may be used in patients who have failed a previous therapy

What are the common complications of Chronic lymphocytic leukaemia?

• anaemia
• hypogammaglobulinaemia leading to recurrent infections
• warm autoimmune haemolytic anaemia in 10-15% of patients
• transformation to high-grade lymphoma (Richter's transformation)

What is Richter's transformation?

occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin's lymphoma

What symptoms indicate Ritcher's transformation?

• lymph node swelling
• fever without infection
• weight loss
• night sweats
• nausea
• abdominal pain

What causes Chronic lymphocytic leukaemia?

a monoclonal proliferation of well-differentiated lymphocytes which are almost always B-cells

What is the most common form of leukaemia seen in adults?

Chronic lymphocytic leukaemia

What is seen in bloods in Chronic lymphocytic leukaemia?

• lymphocytosis
• anaemia: may occur either due to bone marrow replacement on autoimmune hemolytic anaemia (AIHA)
• thrombocytopenia: may occur either due to bone marrow replacement on immune thrombocytopenia (AIHA)

What is seen in blood film in Chronic lymphocytic leukaemia?

smudge cells (also known as smear cells)

What is the key investigation for Chronic lymphocytic leukaemia?

immunophenotyping

What are the indications of treatment of Chronic lymphocytic leukaemia?

• progressive marrow failure: the development or worsening of anaemia and/or thrombocytopenia
• massive (>10 cm) or progressive lymphadenopathy
• massive (>6 cm) or progressive splenomegaly
• progressive lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months
• systemic symptoms: weight loss > 10% in previous 6 months, fever >38ºC for > 2 weeks, extreme fatigue, night sweats
• autoimmune cytopaenias e.g. ITP

What are the Poor prognostic factors for Chronic lymphocytic leukaemia?

• male sex
• age > 70 years
• lymphocyte count > 50
• prolymphocytes comprising more than 10% of blood lymphocytes
• lymphocyte doubling time < 12 months
• raised LDH
• CD38 expression positive
• TP53 mutation

What are the Chromosomal changes seen in Chronic lymphocytic leukaemia?

• deletion of the long arm of chromosome 13 (del 13q) is the most common abnormality, being seen in around 50% of patients. It is associated with a good prognosis
• deletions of part of the short arm of chromosome 17 (del 17p) are seen in around 5-10% of patients and are associated with a poor prognosis