Background

1. Symptoms
   • In the early stages, the condition is asymptomatic, the only feature being a peripheral lymphocytosis.
   • CLL usually progresses slowly with increasing lymphocytosis, lymphadenopathy, splenomegaly, hepatomegaly and bone marrow involvement with cytopenias. Systemic symptoms (fever, weight loss and night sweats) are rare.
   • Other complications of CLL include autoimmune haemolytic anaemia, immune thrombocytopenia and hypogammaglobulinaemia with recurrent infections and herpes zoster. CLL can also transform to high-grade lymphoma (Richter’s transformation).
   • Due to the increased risk of infections prompt antibiotic therapy should be offered.
2. Signs
   • The following are considered as one area of organ enlargement each: neck, axillae, groin, spleen, liver e.g. bilateral cervical nodes = one site
   • Lymphadenopathy in CLL is usually bilateral, soft, mobile and painless. Unilateral lymphadenopathy is unusual in CLL and may require further investigation.
3. Staging

   Stage	Definition	Prognosis (median survival – years)
   A	0-2 areas of organ enlargement	>12+ years
   B	3-5 areas of organ enlargement	7 years
   C	Hb <100 g/L or platelets <100x109/L (unless due to immune mechanisms)	2 years

Assessment of Peripheral Lymphocytosis

1. <7 x 10^9 /L with no lymphadenopathy or other concerning features

   • No further investigation
   • No follow-up needed

2. <7 x 10^9 /L with lymphadenopathy

   • Request flow cytometry

3. >7 x 10^9 /L

   • Request flow cytometry

4. Any lymphocyte count with haematologist recommendation for further investigation

   • Request flow cytometry

Management post-flow cytometry

1. If no monoclonal population, no follow-up needed

2. If other monoclonal B-cell lymphoproliferative disorders (e.g. hairy cells, mantle cells), discuss with haematologist

3. If raised monoclonal B-cells and < 0.5 x 10^9 /L monoclonal B-cells

   • Diagnosis = low count B-cell lymphocytosis
   • No follow up

4. If raised monoclonal B-cells and 0.5 - 4.9 x 10^9 /L monoclonal B-cells

   • Diagnosis = high count B-cell lymphocytosis
   • 1-2% progression per year to CLL
   • Annual lymph node examination and FBC
   • No need for referral

5. If raised monoclonal B-cells >5 x 10^9 /L monoclonal B-cells

   • CLL diagnosis

6. If CLL diagnosis, see signs, symptoms and staging in above background information

7. If CLL diagnosis, laboratory Investigations and age-appropriate screening for breast, prostate & colon cancer

   • Perform direct Coombs test (DCT) and immunoglobulins
   • Discuss with haematologist if DCT positive

8. If CLL diagnosis and asymptomatic Stage A, no need for referral

   • Discuss diagnosis and prognosis with patient
   • Ask about symptoms of fever, persistent night sweats, weight loss, history of recurrent infections
   • Ask about any signs to suggest skin malignancy
   • Examine for lymphadenopathy and hepatosplenomegaly
   • 6-monthly follow up for 1st year (then yearly if stable or slow, asymptomatic progression)
   • Check FBC
   • Annual flu vaccination recommended
   • Pneumococcal vaccination recommended 

Information

• Patient Resources
  • Leukaemia and blood cancer NZ (LBC) charity produce patient information booklets on “Chronic Lymphocytic Leukaemia” and factsheets on “active monitoring”. These can be downloaded from the website www.leukaemia.org.nz
  • Leukaemia and Blood Cancer NZ and the Bay of Plenty Cancer Society also run local patient support groups and may be able to offer one-one support as appropriate.

  
  Contact details:

  Leukaemia and Blood Cancer NZ
  0800 15 10 15
  www.leukaemia.org.nz

  Bay of Plenty Cancer Society
  111 Cameron Road, Tauranga
  07 571 2035
  www.waikato-bop.cancernz.org.nz

• Acknowledgements

  This pathway was developed in collaboration with:

  Title	Name
  GP Liaison & Bay Navigator Lead	Dr Chris Tofield
  Consultant Haematologist	Dr Marie Hughes

Disclaimer: These pathways, for the care and management of patients within Bay of Plenty, have been developed jointly by primary and secondary care clinicians. They provide guidance for General Practice teams to diagnose and manage patients suffering from a number of different conditions, and contain patient information resources. The pathways are maps of publicly-funded services accessed by referral from the community, and are strongly evidence based, but are not full clinical guidelines. As the pathways are suggested guidance only, while using them you must exercise your own clinical judgement and pertinent clinical data when treating your patient. This site is intended to be flexible and frequently updated. While every effort has been made to ensure accuracy, all information should be verified.