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49 Cards in this Set
- Front
- Back
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what are the essentials of diagnosis in acute leukemia?
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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% |
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what are the most common pathogens in infections due to neutropenia?
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gram-negative bacteria (E. coli, Klebsiella, Pseudomonas) or fungi (Candida, Aspergillus)
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what are common presentations of infection in acute leukemia?
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cellulitis, pneumonia and perirectal infections
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what are the histochemical findings in ALL?
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neither peroxidase- nor butyrate esterase-positive
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what are the immune-phenotypic findings in B-ALL?
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ALL cells of B lineage will express CD19 and most cases will express CD10, known as cALLa (common ALL antigen)
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what are the immune-phenotypic findings in T-ALL?
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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 |
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what are the immune-phenotypic findings in ALL irrespective of lineage?
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almost all ALL cells will express terminal deoxynucleotidyl transferase (TdT), except Burkitt type of ALL
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what are the immune-phenotypic findings in the Burkitt type of ALL?
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"lymphoma" phenotype, expressing CD19, CD20 and surface immunoglobulin, but not TdT
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what are manifestation of ALL distinguishing it from AML?
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affection of central nervous system in 10% of patients
presence of lymphadenopathy or hepatosplenomegaly suggests ALL |
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what is the classification of ALL based on?
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immunological classification, eg determination of immunologic phenotype
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what is the technique used for determination of the immunologic phenotype?
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flow cytometry
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what is the method used to determine the cytogenetics?
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FISH (fluorescence-in-situ-hybridization)
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what is the differential diagnosis to ALL?
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atypical lymphocytosis of mononucleosis and pertussis
other lymphoproliferative disorders, such as CLL and hairy cell leukemia |
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what are the classification and the immunophenotypes of B-ALL?
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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 |
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what are the distributions in frequency among B-ALL?
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common B-ALL 60%
pro-B-ALL 15% pre-B-ALL 10% mature B-ALL 5% |
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frequency of B-ALL vs T-ALL
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80% B-ALL
20% T-ALL |
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what are the diagnostic steps in evaluation of a leukemia?
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morphology and histochemistry with myeloperoxidase, butyrate esterase
determination of immunophenotype with flow cytometry cytogenetic evaluation with FISH |
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what are cytogenetic findings conferring a favorable diagnosis in ALL?
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hyperploidy (with more than 50 chromosomes), but seldom seen in adults
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what are unfavorable cytogenetics in ALL?
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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 |
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what are the therapy principles in ALL?
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induction therapy, consolidation therapy with the addition of central nervous system prophylaxis and maintenance therapy
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what does the induction therapy in ALL typically consist of?
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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 |
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what does the consolidation therapy of ALL consist of?
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combination chemotherapy with c-ARA and a topoisomerase II inhibitor (antracycline or podophyllotoxine, eg etoposid)
mnemonic: C onsolidation for A (Ara) ll (topoisomerase II) |
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what does the CNS prophylaxis of ALL consist of?
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intrathecal chemotherapy with high-dose ara-C and methotrexate and whole brain irradiation
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what does the maintenance therapy of ALL consist of?
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6-mercaptopurin and methotrexate and prednisone
mnemonic: M&M; or M aintenance --> M ercaptopurine, M ethothrexate |
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what is the distribution of ALL in children vs adults?
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80% in children
20% in adults |
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which immunophenotypic markers are expressed in most B-ALL?
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CD79a+, TdT+,
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in which subtype of B-ALL is TdT not expressed?
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mature B-ALL
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in which subtype of B-ALL is cALLa not expressed?
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cALLa = CD10
is not expressed in pro B-ALL and mature B-ALL |
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in which disorders is cALLa commonly expressed?
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ALL, Burkitt's lymphoma, follicular lymphoma and some hematopoietic tumors
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what does cALLA stand for?
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common ALL antigen is a common name for CD10 which is a cell surface enzyme
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what percentage of ALL is diagnosed under age 20?
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60%
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at what age is the peak incidence of ALL?
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2-5 years
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in what percentage can the Ph chromosome be found in ALL of adults and children?
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adults 20% with 185 kD
children 5%, 90% with 190 kD, 10% with 210 kD |
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what is the incidence of ALL?
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1.5/100'000
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what is the median age in ALL?
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13 years
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at what age is the peak incidence for B-ALL?
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3 years
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at what age is the peak incidence for T-ALL?
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adolescence, when thymus reaches its maximal size
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What are the presenting symptoms of ALL?
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What are the presenting symptoms of ALL?
Symptoms result from bone marrow failure and pancytopenia. * Pallor & Fatigue * Bleeding * Infection LYMPHADENOPATHY and SPLENOMEGALY may also be present. |
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What is the presenting white count in ALL?
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What is the presenting white count in ALL?
The presenting WBC count in ALL is usually high (75% of cases) |
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What constitutes the bone marrow in ALL?
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What constitutes the bone marrow in ALL?
Greater than 30% of bone marrow is NUCLEATED BLAST CELLS. |
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What tissues may be involved with ALL?
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What tissues may be involved with ALL?
1) ** CNS and TESTES ("sanctuary sites") 2) Spleen and Liver (organomegaly possible) 3) Lymph nodes (adenopathy) |
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How is ALL classified?
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How is ALL classified?
ALL is classified according to morphologic features |
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From what cell lines does ALL derive?
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From what cell lines does ALL derive?
80% from B-Cells 20% from T-Cells |
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What is the TREATMENT for ALL?
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What is the TREATMENT for ALL?
Chemotherapy is the mainstay treatment for ALL |
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What are the three main types of ALL?
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What are the three main types of ALL?
L1: uniform, small lymphoblasts (esp. children) L2: heterogeneous, larger lymphoblasts (esp. adults) L3: large basophilic blasts with vacuoles (Burkitt’s leukemia) |
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hat is the INDUCTION therapy for ALL?
ALL Induction Therapy: |
hat is the INDUCTION therapy for ALL?
ALL Induction Therapy: 1) Vincristine 2) Predinsone/Dex 3) Dauno- or Doxorubicin 4) L-asparaginase |
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What special-case therapy is mandatory in ALL treatment?
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What special-case therapy is mandatory in ALL treatment?
CNS Prophylaxis, with intrathecal Methotrexate & Cranial Irradiation |
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What is the MAINTENANCE therapy for ALL?
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What is the MAINTENANCE therapy for ALL?
Low dose therapy with Methotrexate and 6-Mercaptopurine |
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What are indicators of a poor prognosis with ALL?
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What are indicators of a poor prognosis with ALL?
1) Age <1 year or >10 years 2) WBC count >50K 3) CNS involvement 4) Philadelphia translocation 5) Failure to achieve remission in <4 weeks |
