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23 Cards in this Set

  • Front
  • Back
what are the essentials of diagnosis in CLL?
lymphocytosis >5000/mcl
coexpression of CD19 and CD5 on lymphocytes
what is the median age in CLL?
70 years
what are other epidemiologic findings in CLL?
most common leukemia in adults with 30%

90% of cases occurring after age 55
what are common classifications system of CLL?
Rai classification system (USA)

or Binet (Europe)
what is the grading in the Rai classification of CLL?
stage 0 lymphocytosis only
stage 1 lymphocytosis and lymphadenopathy
stage 2 organomegaly
stage 3 anemia
stage 4 thrombocytopenia
what are the definitions of risk groups in CLL?
Rai stage 0/1 low-risk
Rai stage 2 intermediate risk
Rai stage 3/4 high-risk
what are particular hematologic phenomena of CLL?
10% with autoimmune hemolytic anemia
2% with autoimmune thrombocytopenia

5% with transformation of isolated lymph node transformation into an aggressive large cell lymphoma (Richter syndrome)
what are the characteristic laboratory findings in CLL?
lymphocytosis usually greater than 20'000/mcL

hematocrit and platelets usually normal at presentation
what is the morphology of the bone marrow in CLL?
variable infiltration with small (mature) lymphocytes
what are the immunophenotypic findings in CLL?
coexpression of the B lymphocyte lineage marker CD19 with the T lymphocyte marker CD5
in what condition is coexpression of CD19 and CD5 also seen?
mantle cell lymphoma
how is CLL distinguished from mantle cell lymphoma?
cytogenetic studies

CLL with expression of CD23

low expression of surface immunoglobulin and CD20

abscence of overexpression of cyclin D1
what is a frequent immunologic complication of CLL?
hypogammaglobulinemia in 50%

small amount of IgM paraprotein in some (DD Waldenström macroglobulinemia)
what is the differential diagnosis to CLL?
viral infection and pertussis (Bordetella pertussis)

other lymphoproliferative disorders with splenomegaly and lymphocytosis, such as mantle cell lymphoma, follicular lymphoma, splenic marginal cell lymphoma, hairy cell leukemia, Waldenström macroglobulinemia (lymphoplasmacytic lymphoma)
keywords of CLL?
progressive accumulation of functionally incompetent but mature lymphocytes

chronic course: months to decades!
frequency of lymphadenopathy and splenomegaly in CLL?
50% with lymphadenopathy

25% with splenomegaly
what is the grading of CLL in the Binet classification system
A less than 3 regions affected, Hb>100 g/l, Tc>100 G/l

B 3 or more regions affected, Hb>100 g/l, Tc>100 G/l

C Hb<100 g/l or Tc <100 G/l

definition of areas: spleen, liver, lymph nodes (axillary, inguinal, cervical)
prognosis in CLL?
very variable, depending on cytogenetics
what are the treatment principles in CLL?
palliative,

first-line treatments are FCR (fludarabine/cyclophosphamide with rituximab) or bendamustine (=Ribomustin)

another second-line therapy is a monclonal antibody: anti-CD52 alemtuzumab = MabCampath
what are other particular findings in CLL?
increased risk for secondary neoplasms, by the majority solid, such as melanoma, bronchial carcinoma and gastrointestinal neoplasms
what is the incidence of CLL?
3-4/100'00

>20/100'000 in people >70 years
what are the characteristics of genetic changes in CLL in contrast to most hematological malignancies?
most with chromosomal deletions or loss of heterozygosity rather than translocations
what is the cell of origin in CLL?
naive B cell or memory B cell