Chronic lymphocytic leukemia (CLL) affects usually older adults and is more chronic, hence the name.
Symptoms
Usually the patient with CLL will not have any symptoms for some time. The first sign may be that a loved one or the patient notices a swollen lymph gland that is painless. This often prompts a doctor’s office visit. The doctor will confirm that lump and may even find an enlarged liver and an enlarged spleen. A blood screening test will point to the diagnoses of CLL. Later the patient will get lack of appetite and rapid weight loss, fatigue and shortness of breath. Later-on with progressive disease there will be a generalized lymph gland swelling (lymphadenopathy) and abdominal fullness from liver and spleen enlargement and abdominal lymphadenopathy. A late sign is a proneness to bacterial infections, viral infections and fungal infections because antibodies are missing (hypogammaglobulinemia) and there is a low white blood cell count (granulocytopenia). This all is due to the overabundance of abnormal lymphocytes. They are look-alikes, but have no function other than to destroy the other blood cell types thus producing the symptoms of this particular blood cancer, CLL.
Tests
Blood tests show a sustained absolute lymphocyte count of more than 5,000 per micro liter. At the same time there are more than 30% lymphocytes in the bone marrow. In some patients there is a low antibody count (hypogammaglobulinemia). The B-cell type CLL has B cell surface markers, which can be determined through immunotyping.
Staging
There is a popular staging system in North America, which has been very useful in comparing the results from one institution to the next. The Rai classification employs five stages from 0 to IV as follows (modified from Ref.1):
Chronic lymphocytic leukemia (CLL)
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Rai classification:
Stage 0 : absolute lymphocyte count in blood more than 10,000 per microliter and more than 30% lymphocytes in bone marrow
stage I : like in stage 0 and enlarged lymph glands
stage II : stage I and spleen and liver enlargement
stage III : stage II and anemia with hemoglobin of less than 11g per deciliter
stage IV : stage III and low platelet count (less than 100,000 per microliter) _____________________________________________________
Chronic lymphocytic leukemia treatment
Not every case of CLL has to be treated. Patients with CLL have a very good overall survival chance. B-cell CLL has an overall survival of 50% over 10 years without any treatment. However, there are tremendous differences in survival between the various Rai stages. For instance, patients in stage 0 to II of the Rai classification will live 5 to 20 years, with stage 0 being the better survival. Patients with stage II or IV will only live on average another 3 to 4 years. When bone marrow failure is evident( the worst among stage IV), survival may only be weeks or a few months. Unfortunately the weakness of the immune system that is associated with a more advanced stage of CLL leads to secondary cancers, which have a major impact on the overall survival.
Chemotherapy: When treatment is felt to be necessary, the proven combination has been and still is chlorambucil as well as corticosteroids. The hematologist has to individualize the therapy. Care has to be taken that over treatment does not kill the patient. Basically the goal of chemotherapy is to bring CLL into remission and hope that the relatively benign underlying course returns.
Corticosteroid therapy: When hemolytic anemia or thrombocytopenia (low platelet counts) are setting in as a complication of CLL, then it is time to treat this with corticosteroids. However, serious side effects from prolonged use have to be avoided.
Radiotherapy: Occasionally local radiation is given to areas of lymph gland swelling or liver and spleen enlargement. This is usually only used as a palliative care as serious long-term side effects are known to occur with radiotherapy.
References:
1. Cancer: Principles &Practice of Oncology. 4th edition. Edited by Vincent T. DeVita, Jr. et al. Lippincott, Philadelphia,PA, 1993. Chapter on Acute Leukemia.
2. Cancer: Principles&Practice of Oncology. 5th edition, volume 2. Edited by Vincent T. DeVita, Jr. et al. Lippincott-Raven Publ., Philadelphia,PA, 1997. Chapter on Acute Leukemia.
3. Conn’s Current Therapy 2004, 56th ed., Copyright © 2004 Elsevier
4. Ferri: Ferri’s Clinical Advisor: Instant Diagnosis and Treatment, 2004 ed., Copyright © 2004 Mosby, Inc
