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chronic lymphocytic leukemia. What is C.L.L. ? a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating ( unlike many tumours ) accumulation of these malignant B cells causes enlarged liver, spleen, lymph nodes
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chronic lymphocytic leukemia • What is C.L.L. ? • a chronic disease • one particular type of lymphocyte (B-cells) accumulates. • not rapidly growing and proliferating (unlike many tumours) • accumulation of these malignant B cells causes • enlarged liver, spleen, lymph nodes • abnormalities in the immune system • Ab deficiencies • Auto-immune comditions
chronic lymphocytic leukemia • Why is an increase in lymphocyte numbers a problem ? • Lymphocyte numbers keep on increasing with the passage of time • Functionally whatever they are required to perform, they don't • Lymphocytes are the key effector cells of immunity. • Despite high lymphocyte numbers, patient is markedly immunocompromised.
chronic lymphocytic leukemia • Why is an increase in lymphocyte numbers a problem ? • And…. • Accumulation of lymphocytes starts with the enlarged lymph nodes, and eventually it infiltrates the bone marrow • Bone marrow that normally has approximately 15 - 20% of all the nucleated cells as lymphocytes may have 90-100 percent of cells as lymphocytes. • Therefore there is increasingly less room for • red corpuscles • platelets • other white cells
chronic lymphocytic leukemia • Why is an increase in lymphocyte numbers a problem ? • Normal cells start to go down in number to dangerous levels causing: • Anaemia • Bleeding • Infection
chronic lymphocytic leukemia Classification of C.L.L. In the past there were a variety of diseases that had been folded into the CLL label on morphological grounds. For instance, mantle cell leukemia But there are different subsets of patients with CLL that have distinctive prognostic factors based on some of the genetic mutations that occur As demonstrated by flow cytometry subsets of patients have to be managed clinically very differently
chronic lymphocytic leukemia • Symptoms: • Disease of the elderly • frequent infections • tiredness • bleeding and bruising • swollen lymph nodes (glands) • swollen abdomen • sweating or fever at night • weight loss In the lab Lymphocytosis >15 (15 wot?) Smear cells
chronic lymphocytic leukemia Staging CLL is a chronic disease. This means that it progresses slowly, usually over many years. In its early stage, CLL may not have any symptoms at all. Staging systems are used to assess the extent of the leukaemia
chronic lymphocytic leukemia • Staging • Stage A: • the person has a high white blood cell count, and either: • no enlarged lymph nodes, or • nodes in only two of five designated areas are enlarged. The designated areas are: Neck Armpits Groin Liver spleen. Research has shown that stage A CLL does not usually need treatment
chronic lymphocytic leukemia Staging Stage B: the person has a high white blood cell count and glands are enlarged in three or more areas. The designated areas are: Neck Armpits Groin Liver spleen.
chronic lymphocytic leukemia • Staging • Stage C: • the person has enlarged glands in three areas or more, • and also has • low red blood cell count, • low platelet count • both. The designated areas are: Neck Armpits Groin Liver spleen.
chronic lymphocytic leukemia • Treatment: • People with stage A chronic lymphocytic leukaemia do not have treatment if their illness is: • not causing any symptoms • progressing only very slowly • There is no advantage to having treatment if CLL is at an early stage • Treatment is only started if and when the symptoms become troublesome
chronic lymphocytic leukemia • Treatment: • chemotherapy • Initially orally • Intravenously if orally shows no improvement
chronic lymphocytic leukemia • Treatment: • Steroid therapy • Often prescribed along with chemotherapy. • As well as treating the CLL, they can help to reduce chemotherapy related sickness and tiredness.
chronic lymphocytic leukemia • Treatment: • Steroid therapy • Has side effects • swollen ankles from fluid retention • raised blood pressure • reduction in size and strength of muscles • tiredness • increased risk of infection • raised level of sugar in the blood • sugar in urine • weight gain • It is unusual for people with CLL to take steroids for long enough to cause all these side effects
chronic lymphocytic leukemia Treatment: Radiotherapy is sometimes used to treat bulky enlarged lymph nodes, or enlarged spleen. Alternatively, an enlarged spleen may be removed surgically
chronic lymphocytic leukemia Treatment: Monoclonal antibodies such as alemtuzumab and rituximab may be used to treat CLL. These can recognise CLL cells and destroy them, while having little effect on normal cells.
chronic lymphocytic leukemia Treatment: High dose chemotherapy & stem cell transplants In the experimental phases.
chronic lymphocytic leukemia Prognosis: Who can say……. These are elderly patients
chronic lymphocytic leukemia Prognosis: stage A: survival is equal to that of a normal age matched population. they will most likely die of something totally unrelated to CLL whether it's heart disease, a car accident, old age, and not from their disease.
chronic lymphocytic leukemia • Prognosis: • Stage B: • average survival of around seven or eight years
chronic lymphocytic leukemia Prognosis: Stage C: year and-a-half to two years