Search Database ICD-O-3 Code Lists

Name

Hepatosplenic T-cell lymphoma

ICD-O-3 Morphology

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

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C422
Primary site must be spleen (C422)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Patients with hepatosplenic T-cell lymphoma present with marked splenomegaly. The spleen is enlarged with diffuse involvement of the red pulp without any gross lesions. Diffuse hepatic enlargement is present as well.

Lymphadenopathy usually not present. Bone marrow frequently involved. Peripheral blood involvement uncommon at presentation but may occur later in 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.

Grade

Not Applicable

Module Rule

None

Alternate Names

Hepatosplenic (Alpha-Beta) cell lymphoma
Hepatosplenic gamma-delta cell lymphoma
Hepatosplenic Gamma-Delta T-cell lymphoma
HSTCL
HSTL

Definition

Hepatosplenic T-cell lymphoma (HSTL) is an aggressive subtype of extranodal lymphoma characterized by a hepatosplenic presentation without lymphadenopathy and a poor outcome. The neoplasm results from a proliferation of cytotoxic T cells, usually of gamma delta T-cell receptor type. It is usually composed of medium-sized lymphoid cells, demonstrating marked sinusoidal infiltration of spleen, liver, and bone marrow.

Definitive Diagnostic Methods

FISH
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

TR genes are clonally rearranged
Isochromosome 7q present
Trisomy 8
2-5 copies of i(7)(q10)

Immunophenotyping

Alpha beta T-cell receptor- (no expression/negative)
CD3+ (expression/positive)
CD4- (no expression/negative)
CD5- (no expression/negative)
CD8+/- (positive and negative)
CD56+/- (positive and negative)
CD94- (no expression/negative) or dim
Gamma delta-T cell receptor positive
Granzyme B- (no expression/negative)
Granzyme M+ (expression/positive)
TIA1+ (expression/positive)

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-9 Codes

202.8 Other lymphoma

Corresponding ICD-10 Codes

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)

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
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

Age: 35 years median age (mostly adolescent and young adults)
Incidence: <1% of all non-Hodgkin lymphomas
Sex: male predominance
Survival: <2 yeras

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: 381-382

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

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary