Lymphoid Neoplasms Flashcards
Neoplastic population of immature lymphocytes (blasts).
Lymphoblastic leukemia/lymphoma
Usually leukemia (BM and blood)
B-ALL
Usually lymphomic mediastinal mass
T-ALL
Most ALLs are of ___________ lineage
B cell
Most common cancer of children
Lymphoblastic leukemia/lymphoma
Incidence of pre-B ALL highest at age _____
4
Incidence of pre-T ALL highest ________
in adolescence
Abrupt stormy onset. Bone pain in kids. Hepatosplenomegaly.
Lymphoblastic leukemia/lymphoma
“hand mirror”
Lymphoblastic leukemia/lymphoma
TdT, CD19, CD10, CD79a, cCD3, surface light chain negative
Lymphoblastic leukemia/lymphoma
hyperdiploidy
t(9;22)
t(12;21)
Good prognosis PreB ALL
t(v;11q23)
MLL gene
Poor prognosis PreB ALL
Translocations involving TCR on chr 7 and 14
Pre T ALL
Complete remission 95% kids and 60-85% adults
ALLs
Less than 2, adolescent or adult presentation. High peripheral blast count. Unfavorable cytogenetics. Minimal residual disease after treatment. t(v;11q23)
Poor risk factors ALL
Ages 2-10. Low WBC count. Early Pre-B phenotype (CD19/CD10). Favorable cytogenetics.
Favorable prognostic factors
CLL/SLL
Chronic lymphotic leukemia/Small lymphocytic lymphoma
Leukemia. Leukocytosis and absolute lymphocytosis
chronic lymphocytic leukemia (CLL)
Lymphomatous
small lymphocytic leukemia SLL
Most common leukemia of adults in western world
chronic lymphocyctic leukemia
Mostly ppl older than 50. Most pts asymptomatic. WBC counts variable. Hepatosplenomegaly. Immune disruptions. Hypogammaglobulinemia, hemolytic anemia, thrombocytopenia
CLL/SLL
Small, mature lymphs with hyperclumped nuclear chromatin. Smudge cells
Chronic lymphocytic leukemia
CD19/CD5/CD23
dim CD20
dim surface light chain
Chronic lymphotic leukemia/Small lymphocytic lymphoma
Transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma to diffuse large b cell lymphoma
Richter syndrome
indolent
most pts die of other problems
CLL/SLL
Germinal center B cells that mimic normal lymphoid follicles. Second most common NHL. BM involvement.
Follicular lymphoma
Has 2 cell types (centocytes and centroblasts)
Follicular lymphoma
Small cleaved cells
centrocytes
Count for grading
Centroblasts
Has paratrabecular BM aggregates
follicular lymphoma
CD19, CD20, CD10
bright surface light chain
BCL2
BCL6
Follicular lymphoma
In normal LN, only mantle zone B and T cells stain + for
Bcl2
Not easily curable, but indolent.
Follicular lymphoma
Normal architecture with follicles of varying size. Mantel zone polarization. Normal germinal centers w/ tingable body macrophages. Normal bcl2 staining in mantle zone
Follicular hyperplasia
Architectural disruption with follicles of similar size. No mantle zone, no polarization. Abnormal follicle with no true germinal center. No tingable body macs. abnormal bcl2 staining
Follicular lymphoma
Express CD5 and overexpress cyclin D1
Mantle cell lymphoma
Hepatosplenomegaly. Mostly BM involvement. PB involvement varies. Has lymphomatoid polyposis or GI involvement
Mantle cell lymphoma
MCL
mantle cell lymphoma
CD19, CD20, CD5
bright CD20 and surface light chain
CD23 negative
mantle cell lymphoma
t(11;14)
mantle cell lymphoma
Aggressive. Most pts die of organ failure from tumor infiltration. Tx w/ rituximab (anti-CD20) and chemo combo.
BM transplant in younger pts
Majority ultimately relapse
MCL
Tumor cell recapitulates normal MALT tissue. Often extranodal
Marginal zone lymphoma
MZL
marginal zone lymphoma
In tissues with chronic inflammation. May regress if inciting agent brought under control
marginal zone lymphoma
Reactive polyclonal inflammatory rx.
polyclonal > oligoclonal > monoclonal
marginal zone lymphoma
Resembless SLL but many neoplastic cells are plasma cells. Mainly BM involvement. Secretes monoclonal IgM causing hyperviscosity
Lymphoplasmacytic lymphoma
LPL
lymphoplasmacytic lymphoma
Secretes monoclonal IgM causing hyperviscosity syndrome known as
Waldenstrom macroglobulinemia
IgM increases blood viscosity causing __________
visual and neurological sxs
Bleeding, autoimmune hemolytic anemia
B cells involving blood, BM, and splenic red pulp. Present with sxs related to cytopenias. Splenomegaly, BM involvment
Diffuse fried eggs. Round, reniform nuclei with moderate amount cytoplasm. Cells incite reticulin fibrosis
Hairy cell leukemia
HCL
hairy cell leukemia
Infections, fatigue, weakness are ________ sxs
cytopenia
Hairy B cell leukemia has PB involvement, but due to ______ can be hard to find cells
pancytopenia
Neoplastic cells sensitive to purine analog type of chemo causing long remissions
hairy cell leukemia
ALL
Lymphoblastic leukemia/lymphoma
DLBCL
diffuse large B cell lymphoma
High grade.
Mostly occurs in immunodeficient pts. Typically associated with EBV
DLBCL
Rapidly enlarging, symptomatic mass nodal or extranodal. B symptoms. Large lymphocytes. Mitotically active.
DLBCL
CD19, CD20
Surface light chain restriction
May or may not have BCL2, BCL6, or CD10
DLBCL
cMYC mutation
DLBCL
Aggressive and fatal if untreated.
DLBCL
Highest grade of high grade
Burkitt lymphoma
3 subtypes
- endemic EBV associated in Africa
- sporadic in US children
- Immunodeficient (HIV)
Burkitt lymphoma
Translocation involving MYC
Burkitt lymphoma
Usually extranodal only.
BM and PB involvement unusual
Burkitt lymphoma
Usually associated with mandibular or orbital mass
EBV associated Burkitt lymphoma
Usually associated with abdominal mass in ileocecum.
Sporadic in US children Burkitt lymphoma
Rapid cell death and turnover. Death releases Ca, uric acid, and K. Medical emergency requiring hydration, binding agents for K and uric acid
Associated with?
Tumor lysis syndrome
Associated with burkitt lymphoma
Diffuse infiltrate medium sized cells.
Cytoplasmic vacuoles
High mitotic activity (>99% proliferation)
Many macs creating “starry sky”
burkitt lymphoma
CD19, CD20, CD10
surface light chain restriction
BCL6
Burkitt lymphoma
t(8;14)
burkitt lymphoma
Aggressive but responds well to intensive systemic chemo
burkitt lymphoma