exam 3 Flashcards
Precursor B lymphoblastic leukemia/ lymphoma
a neoplasm of lymphoblast’s committed to the B - cell lineage
B-LBL vs B-ALL
B-LBL: lymphoma mass, with or without minimal blood and BM involvement
Precursor B lymphoblastic leukemia epidemiology
B-LBL: 10% of lymphoblastic lymphoma, 75% of cases in patients under 18 years old
B-ALL: 75% of cases in children under 6 years old
Clinical features of B-ALL and B-LBL
B-ALL: WBC decreased, normal or markedly elevated, Anemia, thrombocytopenia, lymphadenopathy, hepatosplenomegaly, bone pain, arthralgias
B-LBL: skin, bone, soft tissue, and lymph node
Sites of involvement and morphology in Precursor B LL
B-ALL: blood and bone marrow in all cases
B-LBL: skin, bone, soft tissue, and lymph node, rare in mediastinal mass
Immunophenotype in Precursor BLL
TdT, HLA-DR
CD19, CD79a, CD10, CD24
t(4;11)(q21;q23) cases are typically negative for CD10 and CD24
Variably positive for CD20 and CD22
CD45 may be negative
Cytoplasmic Mu chain in pre-B ALL
Precursor BLL genetics
t(9;22)(q34;q11.2): 3-4% of cases, in most childhood cases associated with 190 kd, BCR/ABL fusion tyrosine kinase, unfavorable prognosis
t(4;11)(q21;q23): associated with AF4/MLL, 2-3% of cases, unfavorable prognosis
t(1;19)(q23;p11.3): associated with PBX/E2A. 6% of cases( 25% of pre - B ALL), unfavorable prognosis
t(12;21)(p13;q22): associated with TEL/AML-1, 16-29% of cases, favorable prognosis
Hyperdiploidy vs. Hypodiploidy
Hyperdiploidy: 20-25% of cases, favorable prognosis (>50)
Hypoploidy: 5% of cases, unfavorable prognosis (<50)
B - LL Differential diagnosis
Precursor T-ALL and AML: resolved with immunophenotyping
Hematogones ( normal B precursors): mimic blasts morphology, positive for TdT, CD34, CD10, pan B markers, in very young patients or in older patients with neuroblastoma, post - chemo, iron deficiency, ITP
Burkitt lymphoma
Blastoid variant of mantle cell lymphoma (positive for bcl-1, negative for TdT
Prognosis of B-LL
B-ALL: good prognosis in the pediatric group, 80% of patients cured, poorer diagnosis in adult group with more unfavorable genetic results
B-LBL: median survival of 60 months
Precursor T lymphoblastic leukemia/lymphoma
A neoplasm of lymphoblast committed to the T-cell lineage
lymphoma vs. lymphoblastic leukemia
Lymphoma: mass, without or minimal blood and BM involvement
Lymphoblastic leukemia: extensive BM and blood involvement (>25% BM cells)
Precursor T-LL epidemiology
15% of childhood ALL
25% of adult ALL
80-95% of lymphoblastic lymphoma
More in adolescents and males
Clinical features of T-LL
Leukemia: high WBC
Lymphoma: large mediastinal mass ( or other tissue mass), rapid growth, pleural fluid involvement
T-LL sites of involvement
Leukemia: blood and BM, all cases
Lymphoma: mediastinal mass: 50%, others include LN< skin, liver, spleen, Waldeyers ring, CNS, gonads
Morphology of T-LL
Similar to B-ALL/ LBL
High number of mitotic figures
A small number of LBL cases wiht eosinophilia, myeloid hyperplasia, some associated with t(8;13)(p11.2;q11-22), some of them developed myeloid malignancy
Cytochemistry of T-LL
acid phosphatase, TdT, PAS: nuclear
T-LL immunophenotype
TdT positive, cCD3 (the only lineage specific marker), CD4,8: double negative or positive, Variable surface: CD1a,2,3,4,7,10,79a,13,33,117( rare), TCR: may have rearrangement, not lineage specific
T-LL genetics
TCR loci ( 1/3 of T-ALL): 14q11.2 (alpha, delta), 7q35( beta), 7p(14-15)(gamma)
Genes:MYC (8q24.1): TAL (1p32), RBTN1(LMO1)(11p15), RBTN2(LMO2)(11p13), HOX11(10q24), LCK(1p34.3-35)
T-LL differential diagnosis
B-ALL and AML(M0): by immunophenotyping, not morphology
Hematogones: mimic blasts
Burkitt
Blastoid variant of mantle cell lymphoma
T-LL prognosis
survival comparable to B-ALL with current treatment
Fetal RBS analysis by Flow Cytometry
test performed in order to quantitate circulating fetal red cells in maternal blood, test indications are placental injury from trauma or rh incompatibility, usually performed to be a quantitative confirmation for the fetal screen done in blood bank.
Fetal RBC procedure
moms blood is drawn and a CBC to determine amount of blood to be used, blood and 3 levels of controls, RBC membranes are fixed using glutaraldehyde, cells are washed, holes are punched in the cell membrane using triton, cells are incubated with a cytoplasmic (intracellular) monoclonal antibody to fetal hemoglobin
Fetal RBC equations
% of fetal RBC’s = # of fetal cells counted/ # mom cells counted x 100
mls of bleed = % of fetal cells x 50