T-LPD with leukemic presentation
Quiz by Sadeq K. Ali Al-Salait
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- Q1
According to the WHO classification, mature T-cell neoplasms are categorized based on all of the following EXCEPT:
Clinical behavior (indolent vs. aggressive)
Presentation (nodal, extranodal, leukemic)
Underlying genetic abnormalities
Cell of origin (pre-thymic vs. post-thymic)
Immunophenotype (CD4+ vs. CD8+)
30s - Q2
Which chromosomal rearrangement is most characteristically associated with T-PLL and involves the TCL1 oncogene?
t(8;14)(q24;q32)
inv(14)(q11q32)
t(14;18)(q32;q21)
t(11;14)(q13;q32)
t(9;22)(q34;q11.2)
30s - Q3
A unique immunophenotypic feature seen in a significant subset of T-PLL cases, which is rare in other post-thymic T-cell malignancies, is:
CD4+ CD8+
CD4+ CD8-
CD4- CD8-
CD4- CD8+
CD3 negative
30s - Q4
What is the approximate 5-year overall survival rate for T-PLL?
>75%
~90%
~20%
<5%
~50%
30s - Q5
The pathogenesis of T-LGLL is strongly associated with constitutive activation of which signaling pathway?
WNT/β-catenin
BMP/SMAD
NOTCH
MAPK/ERK
JAK/STAT
30s - Q6
Which of the following is the most frequent autoimmune association in T-LGLL?
Rheumatoid Arthritis
Sjögren's Syndrome
Systemic Lupus Erythematosus (SLE)
Autoimmune Thyroiditis
Multiple Sclerosis
30s - Q7
The diagnostic criterion for T-LGLL includes an absolute peripheral blood LGL count greater than:
1.0 × 10⁹/L
4.0 × 10⁹/L
0.5 × 10⁹/L
10.0 × 10⁹/L
2.0 × 10⁹/L
30s - Q8
The immunophenotype of a typical T-LGLL is:
CD3+, CD4+, CD8+, CD30+
CD3+, CD4-, CD8-, TCRγδ+
CD3-, CD56+, CD16+
CD3+, CD4+, CD8-, CD57+
CD3+, CD4-, CD8+, CD57+
30s - Q9
Which genetic mutation is most commonly associated with T-LGLL and is a marker of poorer prognosis?
TET2
STAT3
JAK2
FLT3
DNMT3A
30s - Q10
A classic paraneoplastic syndrome frequently seen in the acute form of ATLL is:
Cushing's syndrome
Hypercalcemia
DIC
Syndrome of Inappropriate Antidiuretic Hormone (SIADH)
Hypoglycemia
30s - Q11
The immunophenotype of ATLL is typically:
CD3+, CD4+, CD8+, CD7+
CD3-, CD4-, CD8+, CD30+
CD3+, CD4-, CD8-, CD56+
CD3+, CD4+, CD8-, CD10+
CD3+, CD4+, CD8-, CD25+
30s - Q12
The diagnostic triad for Sézary Syndrome includes all of the following EXCEPT:
Circulating Sézary cells
Erythroderma
Generalized lymphadenopathy
Hepatosplenomegaly
30s - Q13
A key immunophenotypic aberration in Sézary cells is the loss of:
CD5
CD4
CD3
CD2
CD7
30s - Q14
Which of the following flow cytometry findings is part of the ISCL diagnostic criteria for Sézary Syndrome?
CD4+CD26- cells ≥ 30%
CD4/CD8 ratio above 5
CD4+CD7- cells ≥ 80%
CD3+CD5- cells ≥ 20%
CD8+CD57+ cells ≥ 40%
30s - Q15
Hepatosplenic T-cell lymphoma is characterized by a frequent association with:
Chronic H. pylori infection
Celiac disease
Psoriasis
Rheumatoid arthritis
Solid organ transplantation
30s