Hepatosplenic T-cell lymphoma

Name
Hepatosplenic T-cell lymphoma
ICD-O-2 Morphology
9716/3
Effective 1995 - 2000
ICD-O-3 Morphology
9716/3
Effective 2001 and later
Reportable
for cases diagnosed 1995 and later
Primary Site(s)
C422
Primary site must be spleen (C422)

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Grade
5 - T-cell
Module Rule
None
Alternate Names
Hepatosplenic (Alpha-Beta) cell lymphoma
Hepatosplenic gamma-delta cell lymphoma
Hepatosplenic Gamma-Delta T-cell lymphoma
HSTCL
HSTL
Definition
An extranodal and systemic neoplasm derived from cytotoxic T-cells usually of Gamma Delta T-cell receptor type, medium in size, demonstrating marked sinusoidal infiltration of spleen, liver and bone marrow.
Patients frequently have a history of chronic immunosuppression, including kidney transplantation.
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.
Definitive Diagnostic Methods
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
2-5 copies of i(7)(q10)
Isochromosome 7q present
Numerical and structural aberrations of the second chromosome 7
Rearrangement TRG@, TRB@ genes
T-cell receptor (TCR) Gamma/Delta genes are clonally rearranged
Trisomy 8
Immunophenotyping
Negative: CD4, CD5, granzyme B (Beta), perforin, CD94 (dim or absent), TCR Alpha Beta Positive/Negative: CD56, CD8TCRa
Positive/Negative: CD8, CD56
Positive: CD3, TCR Delta 1, V Delta 1 epitope, TIA1, granzyme M, multiple killer immunoglobulin-like receptors (KIR) isoforms (aberrant coincident expression)
Treatments
Chemotherapy
Hormone
Stem cell transplant
Surgery (splenectomy)
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 (effective October 1, 2015 U.S. only)
C86.1 Hepatosplenic T-cell lymphoma
Signs and Symptoms
Anemia
Drenching night sweats
Fatigue
Fever (for no known reason)
Hepatomegaly
Pain in the chest, abdomen, or bones (for no known reason)
Skin rash or itchy skin
Splenomegaly
Weight loss (for no known reason)
Diagnostic Exams
Bone marrow aspiration and biopsy
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)
Incidence: <1% of all non-Hodgkin lymphomas
Sex: male predominance
Survival: <2 yeras