Name

Prolymphocytic leukemia, NOS

ICD-O-1 Morphology

9825/3: Prolymphocytic leukemia
Effective 1978 - 1991

ICD-O-2 Morphology

9825/3: Prolymphocytic leukemia
Effective 1992 - 2000

ICD-O-3 Morphology

9832/3: Prolymphocytic leukemia, NOS
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421

Grade

Not Applicable

Module Rule

None

Alternate Names

PLL

Definition

General term for non-specific prolymphocytic leukemia (unknown if B-cell or T-cell).
A type of chronic lymphocytic leukemia (CLL), in which too many immature white blood cells (prolymphocytes) are found in the blood and bone marrow.

Abstractor Notes

Patients typically have systemic involvement at presentation, with bone marrow and peripheral blood involvement as well as hepatosplenomegaly, generalized lymphadenopathy, and skin infiltrates.

Due to the systemic nature of this neoplasm, leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver and skin. A high lymphocyte count (> 100 x 109/L) along with anemia and thrombocytopenia are common findings. HTLV-1 serologies are negative and serum immunoglobins are within normal limits with no paraproteins present

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear

Genetics Data

inv 14(q11,q32)

Immunophenotyping

CD1a negative
CD2 positive
CD3 positive
CD4 positive
CD7 positive
CD8 negative
TdT negative

Treatments

Chemotherapy
Hormone therapy

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

None

Diagnostic Exams

None

Progression and Transformation

None

Epidemiology and Mortality

None

Sources

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