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 Module 7
Most common sites of involvement: lymph nodes

Help me code for diagnosis year :

Grade
5 - T-cell
Module Rule
None
Alternate Names
AILD [OBS]
AITL
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
AITL typically presents with advanced stage disease, with spleen, liver, skin and bone marrow frequently involved.

Patients exhibit immunodeficiency secondary to the neoplastic process, in the majority of cases the expansion of B-cells positive for EBV is seen, thought to be a consequence of underlying immune dysfunction.

Aggressive clinical course with patients often succumbing to infectious complications, which makes delivery of aggressive chemotherapy difficult.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Additional X chromosome
Occasional clonal immunoglobulin gene rearrangement which correlates with expanded EBV+ B-cells
T-cell receptor genes clonally rearranged
Trisomy 3
Trisomy 5
Immunophenotyping
CD2+
CD3+
CD5+
CD10+
CDXCL13+
PD-1+
Treatments
Chemotherapy
Hormone
Stem cell transplant
Transformations to
There are no known transformations
Transformations from
There are no known transformations
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
Arthritis
Ascites
Drenching night sweats
Fatigue
Fever (for no known reason)
Generalized lymphadenopathy
Hepatosplenomegaly
Pain in the chest, abdomen, or bones (for no known reason)
Pleural effusion
Polyclonal hypergammaglobulinemia
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
Weight loss (for no known reason)
Recurrence and Metastases
None
Epidemiology and Mortality
Age: middle age and elderly adults
Incidence: 1-2% of all non-Hodgkin lymphomas
Sex: no male or female predominance
Survival: <3 years medial survival