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Clinical Haematology > Leukaemia, Lymphomas and Myelomas > Flashcards

Leukaemia, Lymphomas and Myelomas Flashcards

1
Q

leukaemia, lymphoma and myeloma are though to arise by what?

A

genetic modification in a single cell in marrow or peripheral tissue:
Onco genes
Tumour suppressor genes

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2
Q

Leukaemia

A

Uncontrolled growth occurs in bone marrow
raised circulating with cell count and bone marrow failure
most common childhood cancer but affects 3x more adults

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3
Q

What are the causes of leukaemia?

A 
Majority = unknown
but:
previous chemo with alkylating agent
Radiation exposure
Benzene/formaldehyde exposure
Genetic conditions
Myelodysplasia
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4
Q

What is AML?

A

Acute Myeloid Leukaemia: elevated blast cells and promyelocytes

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5
Q

What is ALL?

A

Acute lymphoblastic leukaemia: elevated lymphoblasts and prolymphocytes

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6
Q

What is CML?

A

chronic Myeloid leukaemia: elevated neutrophils and myelocytes

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7
Q

What is CLL?

A

Chronic Lymphoid leukaemia: 70% of WBCs appear as small lymphocytes

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8
Q

What is a myeloblast?

A

immature precursor of cells that can become any of myeloid cells

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9
Q

What is a lymphoblast?

A

immature precursor of cell that can become any of the lymphoid cells

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10
Q

Describe in basics AML

A

-uncontrolled proliferation of myeloid stem cells, large no. of immature WBCs, particularly GRANULOCYTES and MONOCYTES
Affects all ages - median age is 60
categorised M0-M1

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11
Q

Symptoms of AML

A 
Acute, often critically ill
Malaise, fever, sweats
Symptoms of Anaemia
Neutropenia 
Thrombocytopenia
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12
Q

Why do these symptoms of AML occur?

A

increased production of cells, lots of metabolism occurring causes increase temp (fever), thus sweats. Increased use of energy causes Malaise. AML os omcreased ,yeoloid cell line, so the other bone marrow cells are pushed out, thus anaemia symptoms. Reduced production of neutrophils causes neutropenia. Reduced production of platelets causes Thrombocytopenia.

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13
Q

Blood tests for AML:

A

> 20% of WBCs are blast cells in blood film
High leukocytosis, low leukopenia or normal WBC count
Anaemia (NORMOCYTIC,NORMOCHROMIC)
Thrombocytopenia
Blast cells in peripheral blood film
WBCs are MPO positive (myeloperoxidase)

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14
Q

What is the prognosis of AML?

A

Chemotherapy leads to complete remission rates of 80-90% in younger patients, but cure rates lower ~45%. Older patients don’t tolerate chemo as well

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15
Q

Describe in basics what is ALL?

A 
uncontrolled proliferation of lymphoid stem cell, resulting in large no. of abnormal lymphoblasts in the bone marrow
80% childhood leukaemia
incidence 1.1:100,000
85% B cell, 15% T cell
Categorised L1-L3
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16
Q

What are the symptoms of ALL?

A 
Starts abruptly
Bone and joint Pain
symptoms of bone marrow failure
Generalised lymphadenopathy
Hepatosplenomgealy
Testicular involvement
CNS manifestations
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17
Q

What are the cause of the symptoms for ALL?

A

Site of ALL is at the bone marrow, resulting in bone pain, causing increased risk of fractures. Bone marrow failure occurs as a result of overcrowding of the bone marrow. Generalised lymphadenopathy is a result go enlarged lymph node due to lymphocytes migrating to lymph nodes to filter lymph. Enlarged spleen and liver causes hepatosplenomegaly.

18
Q

What are the blood tests for ALL?

A

Over production of lymphoid blast cells results in overcrowding of the bone marrow causing:
>20% of WBCs blast cells in blood film
High leukocytosis, low leukopenia or normal WBC count, can be >200x10^9/L
Anaemia: NN
Neutropenia
Thrombocytopenia
WBCs are MPO negative

19
Q

The prognosis for ALL is…

A

majority of children are curable to a standard, adult a cure is less frequent, stem cell transplant often needed.

20
Q

Describe basically what CML is?

A

Uncontrolled proliferation of myeloid stem cells resulting in a large no. of circulating leukocytes particularly neutrophils
Peak onset 40-50yrs of age
Males>females
incidence is 0.8:100,000
associated with the presence of philadelphia chromosomes (oncogene ABL is translocated from chromosome 9 to face with BCR gene on chromosome 22, resulting BCR-ACL gene encodes a protein with high tyrosine kinase activity)

21
Q

What are the symptoms of CML?

A 
Fatigue
weigh Loss
Sweaty
Splenomegaly
Sensation of fullness
Hepatomegaly
Gout
Bruising
Leukostasis
22
Q

Why does gout occur in CML?

A

Rapid cell turnover causes release of purines, purines broken down to uric acid, increasing rate levels. Gout cased by deposition of uric acid in joints

23
Q

Blood tests associated with CML

A

over production of myeloid cells results in over crowding in bone marrow causing:
high leukocytosis WBC count, usually >50x10^9/L
neutrocytosis
increase in complete spectrum of myeloid cells seen in peripheral blood. levels of neutrophils & mylecytes exceed this of blast cells and promyelocytes
increase in basophils
Anaemia (NN)
Platelet count most frequently increased (thrombocytosis) tho can be reduced (thrombocytopenia) or normal.
Neutrophil alkaline phosphatase score in low
increased LDH and rate
FISH for philadelphia chromosome

24
Q

Prognosis of CML

A

indolent chronic pase, followed by period of acceleration and a final generally fatal acute leukaemia phase
Stem cell transplant is the only known cure but is associated with significant mortality
The tyrosine kinase inhibitor IMTINIB is the preferred initial therapy

25
Q

Describe briefly CLL

A 
uncontrolled clonal proliferation of B lymphocytes
disease of elderly  (typically >50yrs)
Males>females
incidence 2.8:100,000
Almost all are B cells
considerable overlap with lymphomas
26
Q

What are the symptoms of CLL?

A 
Often asymptomatic
Symmetrical and painless
may have symptoms of anaemia 
immunosuppression resulting in bacterial infection in early stages and viral fungal/infection later
weight loss
indolent
27
Q

Blood test for CLL

A

over production of lymphocytes results in overcrowding of bone marrow resulting in:
Lymphocytosis (increased lymphocyte count) >5x10^9/L sometimes up to 300
70-99% of WBC appear as small round lymphocytes
smear cells
immunotyping of the lymphocytes almost always shows them to be B cells
Anaemia NN
Thrombocytopenia

28
Q

Prognosis of CLL

A

initially indolent, chemo is often not immediately needed in early CLL
often die of other causes first in elderly
more aggressive in advanced stages

29
Q

What is a lymphoma?

A

Cancer of the lymphoid tissue (spleen, lymph nodes, tonsil, adenoid, thymus). Results in swollen lymph tissue filled with either B or T cells

30
Q

What percentage of lymphomas are hodgkins are what percentage are non-hodgkin?

A

40% hodgkins

60% non-hodgkins

31
Q

What is hodgkins lymphoma?

A

malignant B cells that accumulate in the lymph node, malignant cells form only a minority of the tumour, compromising inflammatory response

32
Q

What are the peak ages of occurrence of hodgkins lymphoma?

A

15-35yrs
over 50yrs
(Males>females)
2.6:100,000

33
Q

What are the symptoms of hodgkins lymphoma?

A 
asymmetrical and painless. 
lymphadenopathy usually in cervical regions
starts in neck spread via lymph chain
splenomegaly
fever
weight loss
night sweats
pruritus (inflammatory cells releasing chemicals causing intactness) 
fatigue
34
Q

What is the prognosis of hodgkins lymphoma?

A

good prognosis - 90% cure rate
largely determined by stage of disease
chemo leads to high cure rates even in advanced disease
significant risk of secondary malignant as late side effect of treatment
chemo can cause another leukaemia later on in life

35
Q

What is non-hodgkin lymphoma?

A

large group of lymphoid tumour with variable presentation and history. (B cells most common)
5th out common cancer in western world
incidence 14.2:100,000
any age, median =50yrs

36
Q

What are the signs and symptoms of NHL?

A

asymmetrical, painless lymphadenopathy
less fever, night sweats and weight loss than HL
Sore throat
symptoms of anaemia, thrombocytopenia or neutropenia with infections
acute abdominal symptoms sue to spell/liver
enlargement and involvement of GI tract

37
Q

Blood tests for NHL

A

NN anaemia (maybe autoimmune haemolytic anaemia
Advanced bone marrow involvement gets: neutropenia and thrombocytopenia
lymph node biopsy
Cytogenetics

38
Q

Prognosis of NHL

A

indolent, NHL response well to chemo but cure is elusive.
aggressive NHL may be cured with chemo and RIUXIMAB
increasing use of stem cell transplant

39
Q

What is multiple myeloma?

A

cancer of clonal plasma cells in bone marrow (b-cells)
Abnormal secreting B cells that secret a single homogenous Ig called a PARAPROTEIN. 100s of same Ab
Rare before age 40, peaks oner 70yrs
incidence 4.8:100,000
Males>females

40
Q

What are the signs symptoms of Multiple Myeloma?

A

bone pain
symptoms of anaemia, thrombocytopenia and leukopenia
renal failure, hypercalcaemia
amyloidosis

41
Q

What blood test for multiple myeloma?

A 
plasma cells naturally home to bone marrow, result in overcrowding in bone marrow causing:
increased plasma cells in bone marrow
presence of PARAPROTEIN in serum/urine
Pancytopenia
Anaemia (NN or Macrocytic)
42
Q

What is the prognosis of Multiple Myeloma?

A

Chemo followed by stem cell transplant.
cure is elusive
good palliative care is crucial

Clinical Haematology (10 decks)

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