T Cell Neoplasms Flashcards
Angioimmunoblastic T cell lymphoma (AITCL):
- Caused by
- Population
EBV associated
-Older adults
Enteropathy associated T cell lymphoma is strongly associated with what ancestral groups?
Welsh and Irish
Hepatosplenic T cell lymphoma (HSTCL):
-Which type is more common in young males?
gamma/delta type
- alpha/beta type
- female predominance
- wider age distribution
Angioimmunoblastic T cell lymphoma (AITCL) - Morphology:
-Cellular milieu (5)
- Aggregates of cells w/ clear cytoplasm
- Immunoblasts
- Lymphocytes
- Plasma cells
- Eosinophils
T cytotoxic LGL leukemia - Immunophenotype:
-Positive for (7)
- CD2
- CD3
- CD8
- CD16
- CD57
- granzyme B
- granzyme M
Adult T cell leukemia/lymphoma (ATCL) - Clinical presentation. (6)
- Lymphadenopathy
- Hepatosplenomegaly
- Skin rash
- HyperCalcemia
- Lytic bone lesions
- Visceral involvement (CNS, lungs, GI tract)
Angioimmunoblastic T cell lymphoma (AITCL) - Morphology:
-Architecture (4)
- Diffuse nodal effacement w/prominent postcapillary venules
- Deposition of PAS+ extracellular material
- Mixed lymphoid infiltrate
- Absence of follicles
T cytotoxic LGL leukemia is usually indolent; however aggressive disease is seen in cases with what?
CD56+ w/ blastlike morphology
Angioimmunoblastic T cell lymphoma (AITCL) - Clinical presentation:
- Onset (gradual vs abrupt)
- Signs/Symptoms (4)
-Abrupt onset Signs/Symptoms: -Constitutional (fever, night sweats, weight loss) -Lymphadenopathy -Pruritic skin rash -Pleural effusion
Enteropathy associated T cell lymphoma (EATCL) - Immunophenotype:
- Positive for (2)
- Negative for (2)
- HLA genotype
Positive: -CD3 -CD30 Negative -CD48 -CD
HLADQA10501, DQB1*0201
Cutaneous T cell lymphoma (CTCL) that involves lymph nodes is called what?
Mycosis Fungoides (MF)
Anaplastic Large cell lymphoma (ALCL) - Morphology:
- Architecture
- Cell size
- Where do anaplastic cells cluster?
- Diffuse proliferation
- Large lymps; some of which are anaplastic
- Anaplastic cells cluster near Blood Vessels
Anaplastic Large cell lymphoma (ALCL) - Prognosis factor:
-Best prognosis
Alk expression
-Alk+ has best prognosis
*WHO classification has separate categories for Alk+ and Alk- ALCL
Cutaneous T cell lymphoma (CTCL) is a ___ T cell neoplasms with ________ growth pattern.
Cutaneous T cell lymphoma (CTCL) is a CD4+ T cell neoplasms with Epidermotropic growth pattern.
T cytotoxic LGL leukemia - Immunophenotype:
-Negative for (3)
- CD4
- CD5 (neg/dim)
- CD7 (neg/dim)
ALK gene location.
Anaplastic Lymphoma Kinase (ALK)
-Chr2p23
What lymphoma arises in patients with longstanding celiac sprue?
- Often preceded by what?
- Ancestories (2)
Enteropathy associated T cell lymphoma (EATCL)
- Refractory sprue with mucosal ulceration (ulcerative jejunoileitis)
- Welsh and Irish
NPM gene location.
Nucleophosmin (NPM)
-Chr5q35
Peripheral T cell lymphoma (PTCL) - Immunophenotype:
- CD4/CD8
- CD25
- other features
- CD4+; CD8-
- CD25-
- loss of one or several pan T cell markers
T/F: Clonal TCR rearrangement is seen in 90% of ALCL cases.
True
Angioimmunoblastic T cell lymphoma (AITCL) - Immunophenotype:
- Positive for (8)
- Negative for (1+)
Positive:
- CD2
- CD3
- CD4
- CD5
- CD7
- CD10
- bcl-6
- CXCL-13
T cytotoxic LGL leukemia:
- Presentation (3)
- Population
- Autoimmune association
Presentation -Neutropenia -Splenomegaly -Polyclonal hypergammaglobulinemia Population: Older males (>60 y/o) -Rheumatoid Arthritis has higher incidence
Angioimmunoblastic T cell lymphoma (AITCL) - Hematologic/Immunologic associations (5)
- Coombs+ autoimmune hemolytic anemia
- Cold agglutinins
- Anti-smooth muscle Ab
- Rheumatoid factor
- Polyclonal hypergammaglobulinemia
Adult T cell leukemia/lymphoma (ATCL) - Immunophenotype:
- Positive for (5)
- Negative for (2)
Positive:
- CD2
- CD3
- CD4
- CD5
- CD25
Negative:
- CD8
- CD7 (usually)
Large Granular Lymphcytic Leukemia (LGL leukemia):
- Definition
- Cell types (2)
Unexplained increase in LGLs ->6 months ->2x10^9 LGLs Cell types: -T cytotoxic cells or NK cells
What population is at higher risk for Anaplastic Large cell lymphoma (ALCL)?
Children and Young Adults
*50% of childhood high grade lymphomas
Anaplastic Large cell lymphoma (ALCL) - ALK Expression:
- Correlates with what translocation
- Where is it expressed?
t(2;5)/NPM-ALK translocation
-Alk is expressed in cytoplasm and nucleus
NK cell LGL leukemia:
- EBV (+/-)
- Cytogenetics
- EBV- (in contrast to aggressive NK cell leukemia (ANKL) which is EBV+)
- TCR germline
What is the cause of Adult T cell leukemia/lymphoma (ATCL)?
-Lifetime risk?
HTLV-1
-5% (7% for males; 3% for females)
What are the most common T-cell neoplasms, in decreasing order? (4)
- Peripheral T cell lymphoma (PTCL)
- Angioimmunoblastic T cell lymphoma (AITCL)
- Anaplastic Large cell lymphoma (ALCL)
- Adult T cell leukemia/lymphoma (ATCL)
Which has a worse prognosis - Alk- ALCL or PTCL?
PTCL
NK cell LGL leukemia - Immunophenotype:
-Positive for (3)
- CD2
- CD16
- CD56
Angioimmunoblastic T cell lymphoma (AITCL) - Immunophenotype:
-Negative for (1+)
Negative:
- CD8
- Loss of one or several pan T cell markers (CD2,3,5,7)
Anaplastic Large cell lymphoma (ALCL) - Immunophenotype:
-Positive for (7)
- T cell Ags (CD2,3,5,7)
- CD4
- CD30 (membranous/Golgi)
- CD45
- Clusterin
- EMA
- Myeloid Ags (CD13/CD33)
What are the pan T cell markers? (4)
- CD2
- CD3
- CD5
- CD7
the t(2;5)/NPM-ALK rearrangement is present in what percentage of cases of ALCL?
> 95%
Nasal type NK/T cell lymphomas (NTNKT):
- growth pattern
- EBV (+/-)
- Population
- Angioinvasive growth pattern
- EBV+
- Asians, Central/South America
T-cell neoplasms:
- % of lymphoid neoplasm overall
- Highest incidence population
5% of all lymphoid neoplasms
-Asia has highest incidence
Anaplastic Large cell lymphoma (ALCL) - Small Cell Variant - Morphology:
-DDX
Composed of Large lymphoid cells
-NOT anaplastic
DDX: Peripheral T cell lymphoma (PTCL)
T cytotoxic LGL leukemia - Cytogenetics.
TCR rearranged
Angioimmunoblastic T cell lymphoma (AITCL):
-What does CD21 show?
Hyperplastic follicular dendritic cells
-Highlights the residual follicular dendritic cell meshwork
NK cell LGL leukemia - Immunophenotype:
-Negative for (2)
- CD3 (surface)
- CD4
Adult T cell leukemia/lymphoma (ATCL) - Morphology. (1)
Nuclear irregularity
-Cloverleaf or Flowerlike forms
NK cell LGL leukemia - Immunophenotype:
-Variable for (3)
- CD7
- CD8
- CD57
Describe the cells in Cutaneous T cell lymphoma (CTCL).
Small to Large lymphoid cells
-CEREBRIFORM Nuclei
NK cell LGL leukemia:
-Presentation (5)
- Neutropenia
- Anemia
- Fever
- Jaundice
- Hepatosplenomegaly
What is the immunophenotype for Follicular T Helper cells (TFH)? (3)
- CD10
- bcl-6
- CXCL-13
Cutaneous T cell lymphoma (CTCL) involving peripheral blood is called what?
Sezary Syndrome
What age/population is Aggressive NK cell leukemia (ANKL) more common in?
-What is it associated with?
Asians (mean 40 y/o)
-EBV+
Peripheral T cell lymphoma (PTCL) - Morphology:
- Architecture
- Nuclei
- Cellular milieu
- What may be prominent?
- Diffuse, polymorphic, small and large lymphoid cells
- “Cloverleaf” (multilobate nuclei)
- Eosinophils, Plasma cells, and/or histiocytes
- Postcapillary venules may be prominent