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37 Cards in this Set

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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?
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?
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