Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
37 Cards in this Set
- Front
- Back
what are the essentials of diagnosis in acute leukemia?
|
short duration of symptoms, including
fever, fatigue and bleeding cytopenia or pancytopenia more than 20% blasts in bone marrow blasts in peripheral blood in 90% |
|
what are the most common pathogens in infections due to neutropenia?
|
gram-negative bacteria (E. coli, Klebsiella, Pseudomonas) or fungi (Candida, Aspergillus)
|
|
what are common presentations of infection in acute leukemia?
|
cellulitis, pneumonia and perirectal infections
|
|
what are the histochemical findings in ALL?
|
neither peroxidase- nor butyrate esterase-positive
|
|
what are the immune-phenotypic findings in B-ALL?
|
ALL cells of B lineage will express CD19 and most cases will express CD10, known as cALLa (common ALL antigen)
|
|
what are the immune-phenotypic findings in T-ALL?
|
ALL cells of T lineage will usually not express mature T-cell markers, such as CD 3,4 or 8, but will express a combination of CD 2, 5 and 7 and do not express surface immunoglobulin
mnemonic: T for Two, plus 5 equals 7 |
|
what are the immune-phenotypic findings in ALL irrespective of lineage?
|
almost all ALL cells will express terminal deoxynucleotidyl transferase (TdT), except Burkitt type of ALL
|
|
what are the immune-phenotypic findings in the Burkitt type of ALL?
|
"lymphoma" phenotype, expressing CD19, CD20 and surface immunoglobulin, but not TdT
|
|
what are manifestation of ALL distinguishing it from AML?
|
affection of central nervous system in 10% of patients
presence of lymphadenopathy or hepatosplenomegaly suggests ALL |
|
what is the classification of ALL based on?
|
immunological classification, eg determination of immunologic phenotype
|
|
what is the technique used for determination of the immunologic phenotype?
|
flow cytometry
|
|
what is the method used to determine the cytogenetics?
|
FISH (fluorescence-in-situ-hybridization)
|
|
what is the differential diagnosis to ALL?
|
atypical lymphocytosis of mononucleosis and pertussis
other lymphoproliferative disorders, such as CLL and hairy cell leukemia |
|
what are the classification and the immunophenotypes of B-ALL?
|
immunological classification in
pro-B-ALL CD19+, CD79a common ALL CD19+, CD10+ (cALLa), cyIgM-, surfaceIg- pre-B-ALL CD19+, CD10+, cylgM+, surfaceIg- mature B-ALL TdT-, cyIg+, surfacelg+ All ALL are TdT and/or CD79a positive, except mature B-ALL which is negative for TdT cyIg = cytoplasmic immunoglobulin mIg = membrane immunoglobulin |
|
what are the distributions in frequency among B-ALL?
|
common B-ALL 60%
pro-B-ALL 15% pre-B-ALL 10% mature B-ALL 5% |
|
frequency of B-ALL vs T-ALL
|
80% B-ALL
20% T-ALL |
|
what are the diagnostic steps in evaluation of a leukemia?
|
morphology and histochemistry with myeloperoxidase, butyrate esterase
determination of immunophenotype with flow cytometry cytogenetic evaluation with FISH |
|
what are cytogenetic findings conferring a favorable diagnosis in ALL?
|
hyperploidy (with more than 50 chromosomes), but seldom seen in adults
|
|
what are unfavorable cytogenetics in ALL?
|
Philadelphia chromosome t(9;22)(q34;q11), 190 kD
and t(4;11) mnemonic: ALL = All Luck Lost in Philadelphia --> 4 = unlucky number plus seven= soon in heaven equals 11 |
|
what are the therapy principles in ALL?
|
induction therapy, consolidation therapy with the addition of central nervous system prophylaxis and maintenance therapy
|
|
what does the induction therapy in ALL typically consist of?
|
4-drug regimen chemotherapy including an anthracycline (daunorubicin or idarubicin), vincristin, prednisone and cyclophosphamid
mnemonic: CVP plus "antibiotic" or 5-drug regimen with L-asparaginase in addition |
|
what does the consolidation therapy of ALL consist of?
|
combination chemotherapy with c-ARA and a topoisomerase II inhibitor (antracycline or podophyllotoxine, eg etoposid)
mnemonic: C onsolidation for A (Ara) ll (topoisomerase II) |
|
what does the CNS prophylaxis of ALL consist of?
|
intrathecal chemotherapy with high-dose ara-C and methotrexate and whole brain irradiation
|
|
what does the maintenance therapy of ALL consist of?
|
6-mercaptopurin and methotrexate and prednisone
mnemonic: M&M; or M aintenance --> M ercaptopurine, M ethothrexate |
|
what is the distribution of ALL in children vs adults?
|
80% in children
20% in adults |
|
which immunophenotypic markers are expressed in most B-ALL?
|
CD79a+, TdT+,
|
|
in which subtype of B-ALL is TdT not expressed?
|
mature B-ALL
|
|
in which subtype of B-ALL is cALLa not expressed?
|
cALLa = CD10
is not expressed in pro B-ALL and mature B-ALL |
|
in which disorders is cALLa commonly expressed?
|
ALL, Burkitt's lymphoma, follicular lymphoma and some hematopoietic tumors
|
|
what does cALLA stand for?
|
common ALL antigen is a common name for CD10 which is a cell surface enzyme
|
|
what percentage of ALL is diagnosed under age 20?
|
60%
|
|
at what age is the peak incidence of ALL?
|
2-5 years
|
|
in what percentage can the Ph chromosome be found in ALL of adults and children?
|
adults 20% with 185 kD
children 5%, 90% with 190 kD, 10% with 210 kD |
|
what is the incidence of ALL?
|
1.5/100'000
|
|
what is the median age in ALL?
|
13 years
|
|
at what age is the peak incidence for B-ALL?
|
3 years
|
|
at what age is the peak incidence for T-ALL?
|
adolescence, when thymus reaches its maximal size
|