Precursor B and T cell Neoplasms Flashcards
What is Acute Lymphoblastic Leukemia/Lymphoma (ALL)?
Neoplastic population of immature lymphocytes - lymphoblasts
Most ALL’s are pre-B - usually leukemia
Pre T ALL usually lymphomic - mediastinal mass
What is the mos common cancer of children?
ALL
What are the demographics associated with ALL?
Hispanic > White > Black
Boys > Girls
Children > adults
What are the clinical features of ALL?
Neoplastic cells crowd out normal marrow cells - marrow failure
Bone Pain
Generalized adenopathy, hepatosplenomaegaly
CNS sx
Pre-T associated with airway compression
What is the phenotype of of ALL cells?
CD34 and TdT positive
CD45 negative
MPO negative
What antigens are present on Pre-B ALL cells?
CD19
CD22
CD10
What antigens are present on Pre-T ALL cells?
CD2, cCD3, CD5, CD7
Possibly CD4/8
What translocations and genes are associated with B-ALL?
t(12;21) - ETV6 and RUNX1 genes - disturb differentiation and maturation
t(9;22) - BCR and ABL1 genes - tyrosine kinase activity
What mutation is associated with T-ALL?
NOTCH1 gene mutation - essential for normal T cell development
What is the treatment/prognosis of ALL?
Aggressive chemo plus CNS prophylaxis
Children - complete remission >95%
Adults - 35-40% cure
Molecular detection after therapy is associated with worse outcome
What are favorable prognostic factors for ALL?
Low WBC
Hyperdiploidy
Trisomies 4, 7, 10
t(12;21)
What are poor prognostic factors for ALL?
t(9;22) in Pre-B ALL
What is the most common leukemia of adults in the western world?
Chronic lymphocytic leukemia
What are the clinical features of CLL/SLL?
Patients > 50 years
Nonspecific generalized adenopathy, hepatosplenomegaly
Immune disruption - infections = hypogammaglobulinemia
Hemolytic anemia or thromobytopenia due to autoantibodies created by non-neoplastic B-cells due to immune dysregulation
What is the morphology of CLL on a peripheral blood smear?
Small mature lymphs with hyperclumped nuclear chromatin; smudge cells
What is the immunophenotype of CLL/SLL?
CD19/CD5/CD23
CD20 - dim
Surface light chain restricted - dim
What is Richter syndrome?
Transformation of CLL/SLL to Diffuse Large B cell lymphoma
Rapidly enlarging lymph node and/or spleen
Most patients survive <1 year
What is follicular lymphoma?
Second most common NHL
Strongly associated with translocations involving BCL2
What are the clinical features of follicular lymphoma?
Painless adenopathy, generalized or localized
BM almost always involved - stage 4
What is the histologic pattern of lymph nodes in follicular lymphoma?
Nodular or nodular and diffuse pattern
Centrocytes and centroblasts
What proteins are present in follicular lymphoma and can be used as diagnosis?
BCL2 and BCL6 expression
What translocation is associated with follicular lymphoma?
t(14;18)
14- IGH
18- BCL2
What is the prognosis of follicular lymphoma?
Incurable, but indolent
What do mantle cell lymphoma cells overexpress?
Aberrantly express CD5
Overexpression of Cyclin D1
What are the clinical features of mantle cell lymphoma?
Painless, generalized lymphadenopathy
BM involvement
Lymphomatoid Polyposis = GI involvement
What is the immunophenotype of mantle cell lymphoma?
CD19/CD20/CD5
Bright surface light chain
What is the prognosis of mantle cell lymphoma?
Not curable, 3-5 years
Most patients die of organ failure due to tumor infiltration
