Canterbury DHB

Management of Early Stage B-CLL - Information for general practitioners

Management of patients with early stage B-cell chronic lymphocytic leukemia (B-CLL) in the community

Your patient has been diagnosed with early stage B-cell chronic lymphocytic leukaemia. This is a low grade B-lymphoproliferative disorder and the most common type of adult leukaemia. The diagnosis has been established on the peripheral blood counts and immunophenotyping of peripheral blood lymphocytes. Your patient has been referred to you for follow-up in the community because they have early stage disease (known as Binet stage A or Rai stage 0) and a low risk of disease progression in the next 5 years. Early stage disease has a median survival of greater than 12 years and often no treatment is required.

In the early stages, the condition is asymptomatic, the only clinical feature being a peripheral lymphocytosis. CLL usually progresses slowly with increasing lymphocytosis, bone marrow involvement with cytopenias, lymphadenopathy, hepatomegaly and splenomegaly. Systemic symptoms (fever, weight loss, sweating) are rare. Other complications of CLL include autoimmune haemolytic anaemia, immune thrombocytopenia, hypogammaglobulinaemia with recurrent respiratory tract infections, herpes zoster, and transformation to high grade disease (Richter’s syndrome).

Communication with patient

Anxiety generated by the word ‘leukaemia’ can nearly always be allayed by an explanation of the benign nature of the early stage of the disease and discussion that treatment of early stage disease is not required. Written information is available in the Leukaemia & Blood Foundation booklet (attached) and via their website (www.leukaemia.org.nz).

Management of early stage CLL patients in the community

6-monthly follow-up for 1st year (yearly if stable or slow, asymptomatic progression)

• ask for history of infections and constitutional symptoms
• check weight, examine for lymphadenopathy and hepatosplenomegaly
• offer flu vaccination at onset of each winter
• check CBC

If Hb falls, consider autoimmune haemolytic anaemia (AIHA). Check for evidence of red cell destruction (raised reticulocyte count, bilirubin and LDH; reduced haptoglobin) and auto-immune cause (Coomb’s test; also known as direct antiglobulin test).

Refer to Haematology department if

• Progressive lymphadenopathy or hepatosplenomegaly.
• Progressive fall in Hb or platelets.
• Lymphocyte count doubles in less than 6 months*.
• Weight loss >10% in previous 6 months, extreme fatigue or sweats (exclude other causes such as infection)

*The absolute lymphocyte count can rise >200 x 10⁹/litre and is not by itself a reason to start treatment.

Screening for 2nd malignancy

Patients with B-CLL have an increased risk of 2nd malignancies. Early stage patients should receive age-appropriate screening for breast, prostate and colon cancer and where appropriate counselling regarding smoking cessation. Non-melanomatous skin cancers can progress rapidly.

References and further reading

Oscier, D., C. Dearden, et al. (2012). "Guidelines on the diagnosis, investigation and management of chronic lymphocytic leukaemia." Br J Haematol 159(5): 541-564.

Leukaemia and Blood Foundation (www.leukaemia.org.nz)

UK CLL forum (www.ukcllforum.org)

Topic Code: 26784