T-cell prolymphocytic leukemia

Name
T-cell prolymphocytic leukemia
ICD-O-3 Morphology
9834/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421

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Grade
5 - T-cell
Module Rule
None
Alternate Names
Knobby type of T-cell leukemia-Lukes
Prolymphocytic leukemia, T-cell type
T-PLL
T-cell chronic lymphocytic leukemia
T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel
Definition
An aggressive T-cell leukemia characterized by the proliferation of small to medium sized prolymphocytes with a mature post-thymic T-cell phenotype involving the peripheral blood, bone marrow, lymph nodes, liver, spleen and skin.
Abstractor Notes
T-cell prolymphocytic leukemia (T-PLL) is an aggessive T-cell leukemia. Leukemic T-cells are found in the peripheral blood, bone marrow, lymph nodes, spleen, liver and sometimes skin. Cutaneous involvement consists of perivascular or more diffuse dermal infiltrations without epidermotropism. The spleen histology shows dense red pulp infiltration which invades the spleen capsule, blood vessels and atrophied white pulp. In lymph nodes, the involvement is diffuse and tends to predominate in the paracortical areas, sometimes with sparing of follicles. Prominent high-endothelial venules may be numerous and are often infiltrated by neoplastic cells.
Definitive Diagnostic Methods
Bone Marrow biopsy
Genetic testing
Immunophenotyping
Peirpheral blood smear
Genetics Data
Inversion chromosome 14 with breakpoints in the long arm at q11 and q32
TRB@ and TRG@ genes clonolly rearranged
Immunophenotyping
TdT
Treatments
Chemotherapy
Stem cell transplant
Transformations to
None
Transformations from
None
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.3 Prolymphocytic Leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.6 Prolymphocytic leukemia of T-cell type
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None