Search Database ICD-O-3 Code Lists

Name

B-cell prolymphocytic leukemia

ICD-O-3 Morphology

9833/3: B-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)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

This neoplasm affects the peripheral blood (PB), bone marrow (BM) and spleen. Prolymphocytes must exceed 55% of lymphoid cells in PB. The leukemic cells are found in the PB, BM and spleen.

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.

Grade

Not Applicable

Module Rule

None

Alternate Names

B-PLL
Prolymphocytic leukemia, B-cell type

Definition

B-cell prolymphocytic leukemia (B-PLL) is a neoplasm of B-cell prolymphocytes affecting the peripheral blood, bone marrow, and spleen. Prolymphocytes must constitute > 55% of lymphoid cells in peripheral blood.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear

Genetics Data

IG genes clonally rearranged with unmutated IGH gene
IGHV3 and IGHV4

Immunophenotyping

CD5+ (expression/positive)
CD19+ (expression/positive)
CD20+ (expression/positive)
CD22+ (expression/positive)
CD23+ (expression/positive)
CD38+ (expression/positive)
CD79a+ (expression/positive)
CD79b+ (expression/positive)
CD200+ (expression/positive)
FMC7+ (expression/positive)
Strong surface IgM +/- IgD

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

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)

C91.3 Prolymphocytic leukemia of B-cell type (effective October 01, 2015)

Signs and Symptoms

Anemia
Massive splenomegaly
Rapidly rising lymphocyte count
Thrombocytopenia

Diagnostic Exams

Blood chemistry studies
CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Peripheral blood smear

Progression and Transformation

None

Epidemiology and Mortality

Age: 65-69 years median age
Incidence: ~1% of lymphocytic leukemias
Sex: no male or female predominance
Survival: 30-50 months median survival

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 B-cell neoplasms
Pages: 222-223

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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
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