A 73-year-old woman develops shingles (herpes zoster infection) in the right T7 dermatome. She attends her GP who also notices that she has palpable lymph nodes in the right cervical region. The GP undertakes a full blood count, which shows the following:
Hb 131 g/dL
WBC 27.7 × 109/L
Lymphocytes 82%
Platelets 277 × 109/L
Blood film – predominance of mature lymphoid cells.
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(a) What is the likely diagnosis?
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Correct answer: This patient presents with a lymphocytosis of mature cells. The likely diagnosis is chronic lymphocytic leukaemia (CLL). This patient would have been Binet stage A. She has an infection and is immunosuppressed. She should be treated with antiviral treatment, e.g. aciclovir or valciclovir.
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(b) What further tests would you undertake?
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Correct answer: Flow cytometry of peripheral blood lymphocytes should be undertaken. In CLL, the malignant cells are positive for the CD19 and CD22 antigens and are also CD5 positive. The cells will be clonal, i.e. they will express only κ or λ light chains. Serum levels of immunoglobulins are typically depressed in CLL leading to generalized immunosuppression. The expression of ZAP70 should be assessed – ZAP70-positive and CD38-positive CLL has a poor prognosis. The degree of somatic mutation in the immunoglobulin heavy chain genes relates to prognosis – mutated genes generally indicate a favourable prognosis, whereas unmutated genes indicate a more primitive cell and are usually associated with a worse prognosis. Cytogenetic analysis should also be undertaken.
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(c) What treatment would you institute?
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Correct answer: This patient should be treated with aciclovir. Treatment of CLL Binet stage A is with observation only. There is no evidence that institution of chemotherapy at this stage will be of benefit. This patient should be closely followed.
See Chapter 39 for further details.
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(d) What complications can occur?
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Correct answer: Patients with CLL are immunocompromised, particularly in terms of humoral immunity. They may develop infection and also have an increased risk of developing new malignancies, e.g. gastrointestinal cancer. There is an increased risk of autoimmune haemolytic anaemia and thrombocytopenia in CLL.
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(e) What is the long-term prognosis of this condition?
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Correct answer: Median survival if Stage A CLL is over 10 years.
