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