Chronic Leukemia Flashcards
CHRONIC LEUKAEMIAS
(Rapidly or Slowly?) progressing malignancy that starts in blood- forming tissue and causes the production and accumulation of abnormal blood cells.
Slowly
CHRONIC LEUKAEMIAS
Accumulated blood cells are ________ cells . cells in (early or late?) stages of myeloid or lymphoid cell production
matured white
Late
CHRONIC LEUKAEMIAS
There are two main types: _________ and ________ , both of which primarily affect (children or adults?) .
chronic lymphocytic leukaemia (CLL) and chronic myeloid leukaemia (CML)
Adults
CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL)
____clonal disorder characterized by a progressive accumulation of __________ lymphocytes
- Characterized by chronic persistent lympho_____.
Mono
functionally incompetent
cytosis
CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL)
In the case of CLL, B-cell CLL derives from the _____________ B cell.
They express CD___, CD__ and CD___ and have (elevated or reduced ?) levels of membrane immunoglobulins (IgM, IgD), CD79b & FMC7.
antigen- experienced
19; 5; 23
Reduced
CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL)
CLL cells are monoclonal because they _____________________
express only one form of light chain (lambda or kappa) on their cell surface.
In CLL
All normal stages of lymphoid development are postulated to have a malignant counterpart.
T/F
T
CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL)
B cell CLL is characterized by a progressive accumulation within the marrow and blood of long-lived mature leukaemic lymphocytes due to:
stimulation of ______ by ______
due to reduced ______
not because of _______
growth by external signals
apoptosis
rapid production
CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL)
B cell CLL is characterized by a progressive accumulation within the marrow and blood of long-lived mature leukaemic lymphocytes due to:
•stimulation of growth by external signals e.g _____,_____ and ligands binding to ________, and
•due to reduced apoptosis felt to be largely mediated by overexpression of ___, a protein known to inhibit cellular apoptosis.
cytokines, chemokines; B cell receptors
bcl-2
T-CLL is a common disease.
T/F
F
T-CLL is a rare disease, therefore Much less is known about
_____ is the most common form of leukaemia in the western hemisphere
It makes up ___-__% of all leukaemias.
B-CLL
25-30
The peak incidence of CLL is in the ___ decade of life.
The overall incidence is 20 per 100,000 in persons >___ years of age.
6th
70
The male to female ratio of CLL is __:___
2:1.
95% of CLL is of ___ cell origin.
___-CLL is a rare disease.
B
T
CLL - Epidemiology
Overall median survival of patients is about ___-___ years.
10 to 20
CLL - Epidemiology
Individual prognosis can be quite variable – _____ in some patients and life expectancy is not shortened, _____ in others and can progress rapidly and survival from diagnosis may be __-__ years.
indolent
aggressive
2-3
CLL - Epidemiology
Mortality often due to:
•overwhelming _____ with marked ____ and ——-
•Overwhelming ______
•_______
•transformation to an aggressive ______, so called a _______
tumour burden; adenopathy and splenomegaly
infection
bleeding
Lymphoma; Richter’s transformation.
CLL – Clinical features
Incidental finding on _____, where there is isolated _____ and the patient is asymptomatic.
If complaints are present, they are usually:____,_______, night sweats, fever and ________
CBC; lymphocytosis
weakness, easy fatigue
weight loss.
CLL – Clinical features
• There may be frequent bacterial and viral infections because of ________.
•Patients with CLL may report ________ to _________ or _______
hypogammaglobulinemia
an exaggerated response
mosquito or other insect bites.
CLL – Clinical features
On physical examination, (symmetrical or asymmetrical?) peripheral ______ is most frequent abnormal finding involving the neck, axillae and inguinal regions.
Nodes are usually (painful or painless?) .
symmetrical; lymphadenopathy
Painless
CLL – Clinical features
_________ and ______ are variably present.
__________ and __________ are associated with CLL and can result in severe anaemia and thrombocytopenia.
Splenomegaly and hepatomegaly
Autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenic purpura (ITP)
CLL – Clinical features
_____ occurs 10-25% of the time, while ___ is seen in 2% of patients.
AIHA; ITP
CLL – Clinical features
________,______,_______, and _______ result from the immune dysregulation which occurs in CLL.
AIHA, ITP, hypogammaglobulinemia and an exaggerated response to mosquito bites
Which occurs more in CLL patients
AIHA or ITP
AIHA
Lymph gland involvement in CLL
•may be _____ or ____, but generally it is ____________
•________ or ______ but not ______
subtle or spectacular ; only within nodes
unsightly or uncomfortable; invasive
CLL – Laboratory findings
Lymphocytosis: diagnosis of CLL requires evidence of lymphocytosis, at least ____ × 109/L, and lymphocytic infiltration in the ______ of at least ___%.
10
bone marrow
40
CLL – Laboratory findings
With immunological methods, particularly the detection of monoclonal B-cell populations by _______, it is possible to diagnose the disease with lymphocyte counts below this threshold. (Absolute clonal B cell lymphocyte count >___ × 109/L)
light-chain restriction
5
CLL – Laboratory findings
30% of CLL patients have _______ or _______ secondary to an immune process or marrow infiltration.
anaemia or thrombocytopenia
CLL – Laboratory findings
Morphologically, the lymphocytes in blood films are (small or large?) and show scanty cytoplasm and a characteristic pattern of ________, nucleolus is ______
Small
nuclear chromatin clumping
inconspicuous
Azurophil granules are seen on all normal T cells
T/F
F
Azurophil granules are seen only in a minority of normal T cells.
CLL – Laboratory findings
The presence of ____/____ cells, literally _____, destroyed cells on a regular blood smear, is consistently seen with CLL
smudge/smear
flattened
CLL cells morphologicallu indistinguishable from normal lymphocytes.
T/F
T
CLL – Laboratory findings
•Peripheral film usually shows ____cytic _____chromic anaemia in (early or later?) stages.
•If AIHA is present, _____ and _____ can be seen on the blood smear.
•Bone marrow: cellularity is ___eased and 30-99% of nucleated cells are (mature or immature?) lymphocytes.
normo; normo; later
spherocytes and polychromasia
incr; mature
CLL in blood counts of 50x109/l (or more) are often with symptoms
T/F
F
As cells are small, non-functional and largely “inert”, CLL in blood counts of 50x109/l (or more) are often without symptoms
CLL – Laboratory findings
Immunophenotyping: CLL cells co-express CD___ and CD__.
19 ; 5
CLL – Laboratory findings
CD19 is a __ cell marker, and CD5 is a __-cell marker.
B
T
The expression of a T cell marker on a B cell is an example of _______
lineage infidelity
lineage infidelity is seen in only CLL
T/F
F
lineage infidelity that is seen not only in CLL but in AML and ALL as well.
CLL – Laboratory findings Immunophenotyping:
CLL cells are also positive for CD___, negative for CD____ (or dim+), are either ________ or _______ restricted and have a dim level of expression of the immunoglobulin heavy chains IgM and IgD.
23
79b
kappa or lambda light chain
Which is more normally, Bcell or Tcell
Tcell
The normal T-cell/B-cell ratio is reversed in CLL.
T/F
T
Normally, T lymphocytes constitute approximately ____% of the total lymphoid population and B lymphocytes, ____% of the lymphoid cells.
80
20
In B-cell CLL, B cells account for nearly ____% of all lymphocytes.
90
Cytogenetics and molecular diagnostics are needed to make the diagnosis of CLL.
T/F
F
They are not
CLL – Laboratory findings
Cytogenetics and molecular diagnostics are useful in _____________.
determining the survival of patients
CLL – Laboratory findings
Cytogenetics and molecular diagnostics:
In particular,___________, corresponding to a mutation in the _____ gene, is associated with a very short survival, as are ___________.
17p deletions; p53
11q deletions
CLL – Laboratory findings
Cytogenetics and molecular diagnostics:
Other markers of poor prognosis include the presence of the ____ mutation (with or without chromosome deletions), and an _________ gene.
p53
unmutated Vh
Vh corresponds to the _____ portion of the immunoglobulin _____ chain.
variable
heavy
CLL – Diagnostic criteria
•>__ x 109/L absolute blood lymphocytosis and cells morphologically _____ in appearance, sustained over at least a _____ period.
•___% lymphocytes in a _________ bone marrow.
•A monoclonal B-cell phenotype simultaneously showing CD__ positivity (dual positivity CD__, CD__-__ and CD___).
5; mature; 4 week
30; normocellular or hypercellular
5; 5; 19-20; 23