Name

T-cell prolymphocytic leukemia

ICD-O-3 Morphology

9834/3: T-cell prolymphocytic leukemia
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

None

Alternate Names

Knobby type of T-cell leukemia-Lukes
Prolymphocytic leukemia, T-cell type
T-PLL
T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel

Definition

T-cell prolymphocytic leukemia (T-PLL) is 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
Immunophenotyping
Peripheral blood smear

Genetics Data

idic(8)(p11), t(8;8)(p11-12;q12)
inv(14)(q11q32)
t(14;14)(q11;q32)
TCL1a and TCL1B at 14q32.1
TR genes are clonally rearranged
12p13 and 22q deletions

Immunophenotyping

CD1a-
CD2+
CD3 weak expression
CD4+
CD5+
CD7+
CD8-
CD52 usually depressed at high density, can be used as a target of therapy
TdT-

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

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

None

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

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 346-347

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2
Glossary