ALK-positive large B-cell lymphoma

Name
ALK-positive large B-cell lymphoma
ICD-O-3 Morphology
9737/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Module 7
Most common sites of involvement: lymph nodes, mediastinal mass, nasopharynx, tongue, stomach, bone and soft tissues.

Help me code for diagnosis year :

Grade
6 - B-cell
Module Rule
None
Alternate Names
ALK-positive LBCL
ALK-positive plasmablastic B-cell lymphoma
Large B-cell lymphoma expressing the ALK kinase and lacking the t(2;5) translocation
Definition
This is a neoplasm of ALK-positive monomorphic large immunoblast-like B cells, sometimes with plasmablastic differentiation. This lymphoma is very rare (<1% of DLBCL) with less than 40 cases reported to date.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9684/3.)

Most patients present with Stage III/IV. Because these tumors are negative for CD20 antigen, they are insensitive to rituximab.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Karyotyping
Genetics Data
CLTC-ALK fusion gene
Immunoglobulin genes are clonally rearranged
t(2;17)(p23;q23)
Immunophenotyping
ALK-positive
CD3-
CD20-
CD30-
CD45-
CD79a-
CD138+
EMA
VS38+
Treatments
Chemotherapy
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
200.6 Anaplastic large cell lymphoma
Corresponding ICD-10 Codes
C83.3 Diffuse non-Hodgkins lymphoma, large cell (diffuse)
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C84.6 Anaplastic large cell lymphoma, ALK positive
Signs and Symptoms
Drenching night sweats
Fatigue
Fever (for no known reason)
Mediastinal mass
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
Cytogenetic analysis
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Recurrence and Metastases
None
Epidemiology and Mortality
Age: 36 years median age (9-70 years range)
Incidence: <1% of DLBCL lymphomas
Sex: male predominance
Survival: median survival 11 months; >156 months survival for children