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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 What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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Page 1: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 2: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 3: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 4: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 5: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 6: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 7: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 8: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

The designated areas are:

NeckArmpitsGroinLiver

spleen.

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.

Research has shown that stage A CLL does not usually need treatment

chronic lymphocytic leukemia

Page 9: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

The designated areas are:

NeckArmpitsGroinLiver

spleen.

Staging

Stage B:

the person has a high white blood cell count and glands are enlarged in three or more areas.

chronic lymphocytic leukemia

Page 10: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

The designated areas are:

NeckArmpitsGroinLiver

spleen.

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.

chronic lymphocytic leukemia

Page 11: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 12: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

Treatment:

chemotherapy

•Initially orally

•Intravenously if orally shows no improvement

chronic lymphocytic leukemia

Page 13: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 14: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 15: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 16: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 17: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

Treatment:

High dose chemotherapy & stem cell transplants

In the experimental phases.

chronic lymphocytic leukemia

Page 18: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

Prognosis:

Who can say…….

These are elderly patients

chronic lymphocytic leukemia

Page 19: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

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

Page 20: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

Prognosis:

Stage B:

average survival of around seven or eight years

chronic lymphocytic leukemia

Page 21: Chronic lymphocytic leukemia What is C.L.L. a chronic disease one particular type of lymphocyte (B-cells) accumulates. not rapidly growing and proliferating

Prognosis:

Stage C:

year and-a-half to two years

chronic lymphocytic leukemia