Angioimmunoblastic T-cell lymphoma

Name
Angioimmunoblastic T-cell lymphoma
ICD-O-2 Morphology
9705/3
Effective 1992 - 2000
ICD-O-3 Morphology
9705/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for dx year :

Grade
5 - T-cell
Module Rule
None
Alternate Names
AILD [OBS]
AITL
Angioimmunoblastic lymphadenopathy with dysproteinemia
Angioimmunoblastic lymphadenopathy with dysproteinemia [OBS]
Angioimmunoblastic lymphoma [OBS]
Peripheral T-cell lymphoma, AILD
Definition
Peripheral T-cell lymphoma characterized by systemic disease; a polymorphous infiltrate involving lymph nodes, with a prominent proliferation of high endothelial venules and follicular dendritic cells.
Abstractor Notes
Angioimmunoblastic T-cell lymphoma (AITL) primarily involves the lymph nodes and virtually all patients present with generalized lymphadenopathy. In addition, spleen, liver, and bone marrow are frequently involved.

AITL typically presents with advanced stage disease, generalized lymphadenopathy, hepatosplenomegaly, systemic symptoms, and polyclonal hypergammaglobulinemia. Skin rash, often with pruritis, is frequently present. Other common findings are pleural effusion, arthritis, and ascites. Patients exhibit immunodeficiency secondary to the neoplastic process, in the majority of cases the expression of B-cells positive for EBV is seen, thought to be a consequence of underlying immune dysfunction.

The clinical course is aggressive with a median survival of less than three years. Patients often succumb to infectious complications, which makes delivery of aggressive chemotherapy difficult.
Definitive Diagnostic Methods
Genetics testing
Histological confirmation
Immunophenotyping
Genetics Data
Additional X chromosome
Occasional clonal immunoglobulin gene rearrangement and correlation with EBV+ B-gene
T-cell receptor genes clonally rearranged
Trisomy3
Trisomy5
Immunophenotyping
CD2+
CD3+
CD5+
CD10+
CDXCL13+
PD-1+
Treatments
Chemotherapy
Hormone
Stem cell transplant
Transformations to
None
Transformations from
None
Corresponding ICD-9 Codes
202.7 Peripheral T-cell lymphoma
Corresponding ICD-10 Codes
C84.4 Peripheral T-cell lymphoma
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C86.5 Angioimmunoblastic T-cell lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None