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)

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.

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.

Definitive Diagnostic Methods

FISH
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

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

Immunophenotyping

Alpha beta T-cell receptor negative
CD3 positive
CD4 negative
CD5 negative
CD8 positive and negative
CD56 positive and negative
CD94 absent or dim
Gamma delta-T cell receptor positive
Granzyme B negative
Granzyme M expression
TIA1 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

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

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

Diagnostic Exams

CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan

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)
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, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

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