T-cell prolymphocytic leukemia

T-cell prolymphocytic leukemia
ICD-O-3 Morphology
9834/3: T-cell prolymphocytic leukemia
Effective 2001 and later
for cases diagnosed 2001 and later
Primary Site(s)
Primary site must be bone marrow (C421)

5 - T-cell
Module Rule
Alternate Names
Knobby type of T-cell leukemia-Lukes
Prolymphocytic leukemia, T-cell type
T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel
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 aggressive 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.

There is diffuse lymph node involvement 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
Peripheral blood smear
Genetics Data
Inversion chromosome 14 with breakpoints in the long arm at q11 and q32
TRB@ and TRG@ genes clonolly rearranged
Stem cell transplant
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.3 Prolymphocytic Leukemia
Corresponding ICD-10-CM Codes (U.S. only)
C91.6 Prolymphocytic leukemia of T-cell type (effective October 01, 2015)
Signs and Symptoms
Progression and Transformation
Epidemiology and Mortality
Age: 65 years median age (range 30-94)
Incidence: 2% of cases of mature lymphocytic leukemias
Survival: aggressive course, median survival time 1 year