Search Database ICD-O-3 Code Lists

Name

Prolymphocytic leukemia (PPL), NOS [OBS}

ICD-O-3 Morphology

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

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Prolymphocytic leukemia (PPL), NOS [OBS} is not included in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B23)

See 9823/3 for a diagnosis of "prolymphocytic progression of CLL/SLL."

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

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

None

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.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear

Genetics Data

None

Immunophenotyping

None

Treatments

Chemotherapy
Hormone therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C91.3 Prolymphocytic leukemia

Corresponding ICD-10-CM Codes (U.S. only)

C91.Z Other lymphoid leukemia (effective October 01, 2015 - September 30, 2024)
C91.Z0 Other lymphoid leukemia not having achieved remission (effective October 01, 2024)
C91.Z1 Other lymphoid leukemia, in remission (effective October 01, 2024)
C91.Z2 Other lymphoid leukemia, in relapse (effective October 01, 2024)

Signs and Symptoms

None

Diagnostic Exams

None

Progression and Transformation

None

Epidemiology and Mortality

None

Sources

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Glossary