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79 Cards in this Set
- Front
- Back
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what is CLL characterized by ? |
proliferation and accumulation of relatively mature appearing lymphocytes in the BM, blood,spleen, liver and lymph nodes.
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in most cases what cell undergoes a malignant transformation ?
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single clone of beta lymphocyte
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less than 5% of what cells show CLL ?
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t cell
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what is the etiology of CLL ?
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unknown
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exposure to ionizing radiation and cytotoxic drugs or chemicals result in CLL T/F
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F they don't actually
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is there any genetic predesposition in CLL ?
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there appear to be since it is the most frequent type of leukemia occurring in multiple family members but no pattern of inheritance have been reported
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what is the incidence of cll in the united states and europe ?
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it is the most common leukemia with to 30% of all cases
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what is the incidence in the orient of CLL ?
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rare less than 5%
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what is the age group of CLL ?
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over 50 years mean is 60
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what is the percentage of incidence in adults less than 40 yo ?
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less than 10
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is the disease frequent or reran children ?
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rare
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does CLL affect males or females more ?
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males 2:1
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how is the course of CLL ?
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variable
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what percentage of the pt with cll are asymptomatic ?
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25 %
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how is the diagnosis made in asymptomatic patients with CLL ?
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on accidental blood count or in the course of investigating large lymph nodes or splenomegaly lymphocytosis are detected
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the patients with CLL may remain asymptomatic for how long ?
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many years
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some patients with CLL may present with severe ...
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BM failure
anemia thrombocytopenia |
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CLL may have a rapid progression course with a survival time of ...
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less than 2 years
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what is the prognosis of most patients with CLL ?
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intermediate they may not require treatment for a couple years but eventually will
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what are the symptoms of CLL ?
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fatigue
fever frequent infections bleeding enlarged painless lymphnodes night sweats weight loss |
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WBC count in CLL ?
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increase above 15k /mm3
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RBC count and platelet count is ...
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decreased depending on the involvement of the BM
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small lymphocytes make up what percentage of the absolute lymphocytes ?
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70-99%
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what does the PB show ?
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uniform pop of cell
relatively mature, round compact nu, clumped chromatin, no nucleoli, little cytoplasm |
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the cells in the pb of CLL are similar to what case ?
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malignant lymphoma
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what type of lymphoma does CLL look like ?
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diffuse well-differentiated lymphocytic lymphoma
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what type of cells are seen in the the PB of CLL ?
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smudge or smear cells
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why do we see smudge or smear cells ?
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lymphocytes are fragile and break on spreading
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what type of cells are seen in some people in a percentage of 10% ?
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prolymphocytes
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how are the pro lymphocytes different than the typical CLL cells ?
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large
dispersed chromatin prominent nucleolus low nuclear to cytoplasm ratio |
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how is CLL most commonly diagnosed ?
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flow cytometry
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what can you say about the B cells involved in CLL ?
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they are differentiated with some but not all features of a mature B - cell
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the lymphocytes appear to be like in what movie ...
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frozen in differentiation and does not mature to the final stage of the B cell development
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CLL display a lot or a few of SIg ?
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relatively small amount
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SIg causes ...
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faint fluorescence
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immunoglobulin isotope display that most cells show which chain class ?
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single heavy chain class typically mu and some display both mu and gamma
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what light chains does cLL show ?
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kappa or lambda
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can CLL show both kappa and lambda ?
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no
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what does the fact that no kappa and lambda can be seen on the same cell say ?
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they suggest monoclonality of the neoplasm
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what surface receptors are seen on CLL ?
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B Ags
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What CDs are seen on CLL cells ?
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CD 19
CD 20 CD 25 |
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what CD do CLL cells coexpress that can be identified on a subpopulation of normal B cells ?
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CD5
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BM aspirate / biopsy may provide what kind of information ?
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valuable information for diagnosis
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what does the BM aspirate show ?
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increased percentage of small lymphocytes with round nuclei
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what other cell types does the bone marrow aspirate show ?
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many histiocytes and mast cells in spicules
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what tests are used to detect the prognosis of CLL ?
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FISH
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what abnormality shown with fish predicts a less aggressive course ?
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13q-
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what mutations show a more progressive disease ?
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17q-
11q- trisomy 12 |
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high levels of what with what CD are associative with more progressive disease ?
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beta-2-microglobulin
CD38 |
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Factoid : what year did Rai get the staging system of CLL ?
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1975 ken 2e3ed howe w el bouta am rekbet ma3o
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what are the stages of rai ?
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0-IV
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stage 0 what are the clinical features ?
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lyphocytosis in the blood and BM only
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Stage 1 what are the clinical features ?
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lymphocytosis + lymphadenopathy
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stage II what are the clinical features?
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lymphocytosis + hepatomegaly or splenomegaly or both + lymphadenopathy
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what are the stage III clinical features ?
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lymphocytes + anemia (Hb<11g/dl), lymph nodes, spleen, liver maybe enlarged
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stage IV what are the clinical features ?
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thrombocytopenia pt <100k
anemia organomegaly |
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Rai classification divides patients into 3 groups depending on the prognosis
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low risk
intermediate risk high risk group |
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on acidic electrophoresis what agar is used ? |
0
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how does the HbD move on acidic agar ? |
towards the cathode along with HbA and A2 |
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what is the mean survival of people at low risk group cll ?
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>10 y
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what are the stages and percentage and mean survival of group 2 ?
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stages I, II
50% of patients about 6 years |
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what stages are in the 3 group and what is the percentage of patients and what is the mean survival ?
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III, IV
25% of patients < 2 years |
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a small proportion of CLL changes into ...
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a more aggressive form
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are the transformation of CLL like CML to the blast phase ?
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no
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what is the percentage of acute transformations in CLL ?
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less than 1 %
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transformation into Richter's syndrome occurs in what percentage of pts ?
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5%
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what percentage of CLL transforms into prolymphocytic and MM ?
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15%
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CLL may cause infections such as ?
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frequent infections
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what are the most seen types of infections in CLL ?
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upper and lower rest tract infections
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what types of immunresponses are impaired in CLL ?
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both humoral and CMI
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what type of immune abnormalities are seen in CLL ?
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Hypogammaglobulinemia
T-cell abnormalities Auto immunity |
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why do we see hypogammaglobulinemia in CLL patients ?
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failure of the CLL clone to differentiate into normal plasma cells capable of secreting Abs
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most of the autoimmune bodies are targeted toward what cells ?
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mature blood cells RBCs WBCs and Platelets
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what percentage of patients with CLL have autoimmune hemolytic anemia ?
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15%
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are autoimmune thrombocytopenia and granulocytopenia as common ?
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no
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does CLL always require treatment ?
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no
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what mission is initiated in patients with CLL ?
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the watch and wait
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can CLL be cured ?
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no
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what drug can be used as a targeted drug therapy in CLL ?
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rituximab
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