Search Database ICD-O-3 Code Lists

Name

Hepatosplenic T-cell lymphoma (HSTCL)

ICD-O-3 Morphology

9716/3: Hepatosplenic T-cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C422
Primary site must be spleen (C422)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Hepatosplenic T-cell lymphoma (HSTCL) is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)

Hepatosplenic T-cell lymphoma originates in the spleen and often involves the liver, and bone marrow. Peripheral lymph nodes are uncommonly involved. A leukemic phase is uncommon at initial diagnosis but can occur, particularly late in the clinical course.

Splenectomy is the surgical treatment.

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

Hepatosplenic alpha-beta cell lymphoma
Hepatosplenic gamma-delta cell lymphoma

Definition

Hepatosplenic T-cell lymphoma is an aggressive mature T-cell lymphoma characterized by a proliferation of cytotoxic T cells within the spleen, liver, and bone marrow. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Isochromosome 7q present
Loss of 7p22.1-p14.1 and gain of 7q22.11
Overexpression of NK cell–associated molecules, FOS, VAV3, Syk, and S1PR5

Immunophenotyping

CD2+ (expression/positive)
CD3+ (expression/positive)
CD4- (no expression/negative)
CD5- (no expression/negative)
CD8+/- (positive and negative)
Gamma delta-T cell receptor positive
Granzyme B- (no expression/negative)
Granzyme M+ (expression/positive)
Positive cytotoxic markers (TIA1, perforin, granzyme M, granzyme B)
TCRγδ+ (positive/expression)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Surgery

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)

C84.5 Other and unspecified T-cell lymphomas

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

C86.1 Hepatosplenic T-cell lymphoma (effective October 01, 2015 - September 30, 2024)
C86.10 Hepatosplenic T-cell lymphoma, not having achieved remission (effective October 01, 2024)
C86.12 Hepatosplenic T-cell lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Anemia
Drenching night sweats
Fatigue
Fever (for no known reason)
Hepatomegaly
Leukopenia
Pain in the chest, abdomen, or bones (for no known reason)
Skin rash or itchy skin
Splenomegaly
Thrombocytopenia
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
PET (positron emission tomography) scan
Splenectomy

Progression and Transformation

Relapses seen in vast majority of patients who respond initially to chemotherapy

Epidemiology and Mortality

Incidence: 1.4-2% of peripheral T-cell lymphomas
Age: 35 years median age (mostly adolescent and young adults)
Sex: male/female predominance based on subtype
Survival: <2 yeras

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mature T-cell and NK-cell neoplasms
Pages: Part B: 727-729

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

PDQ® Adult Treatment Editorial Board. PDQ Peripheral T-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <03/13/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq. Accessed <03/31/2025>. [PMID: 37437079]
Section: Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq#_2289
Glossary