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

Help me code for dx year :

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, usually with hepatomegaly, but with no lymphadenopathy. Bone marrow is almost constantly involved.

The spleen is enlarged with diffuse involvement of the red pulp without any gross lesions. Diffuse hepatic enlargement is present as well. Patients present with hepatosplenomegaly and systemic symptoms, usually marked thrombocytopenia, often anemia and leukopenia. Peripheral blood involvement is uncommon at presentation but may occur later in the clinical course.

The disease course is aggressive. Patients may respond initially to chemotherapy but relapses are seen in the vast majority of cases. The median survival is <2 years. Removal of the spleen and stem cell transplants have been used in treatment of this disease.
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
Transformations to
None
Transformations from
None
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None