Anaplastic large cell lymphoma, ALK-positive

Name
Anaplastic large cell lymphoma, ALK-positive
ICD-O-2 Morphology
9714/3
Effective 1992 - 2000
ICD-O-3 Morphology
9714/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for diagnosis year :

Grade
5 - T-cell
Module Rule
None
Alternate Names
ALCL, ALK+
Anaplastic large cell lymphoma [OBS]
Anaplastic large cell lymphoma, CD30+
Anaplastic large cell lymphoma, NOS
Anaplastic large cell lymphoma, small cell variant
Anaplastic large cell lymphoma, T cell and Null cell type
Large cell (Ki1+) lymphoma [OBS]
Definition
ALCL is a T-cell lymphoma consisting of lymphoid cells that are usually large with abundant cytoplasm and pleomorphic, often horseshoe-shaped nuclei. Most cases express cytotoxic granule-associated proteins. The majority are positive for the Anaplastic large cell lymphoma kinase (ALK) protein.
Abstractor Notes
Grade is T-cell (5) unless pathologist specifically designates as a B-cell (see G2 rule)

Note: This neoplasm has a small cell variant. (See below.) Anaplastic large cell lymphoma, ALK-positive (ALCL, ALK+) frequently involves both lymph nodes and extranodal sites. The most commonly involved extranodal sites include skin, bone, soft tissue, lung, and liver. Involvement of the GI system and CNS is rare. Mediastinal disease is less frequent than in Classical Hodgkin lymphoma. The evidence of bone marrow involvement is approximately 10%. The small cell variant may have a leukemic presentation with peripheral blood involvement. The majority of patients present with stage III-IV disease with peripheral blood and/or abdominal lymphadenopathy often associated with infiltrates and involvement of bone marrow. Patients often have B-symptoms, especially high fever. The immunophenotyping is vital in differentiating this neoplasm from large B-cell lymphoma with immunoblastic/plasmablastic features expressing ALK protein, rhabdomyosarcoma, and inflammatory myofibroblastic tumors.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
T-cell receptor (TCR) genes are clonally rearranged
t (2;5) (p23;q35)
Immunophenotyping
Negative: CD3
Positive: ALK, CD2, CD4, CD5, CD30
Positive/Negative: CD45, CD56, EMA, granzyme B, perforin, TIA-1
Treatments
Chemotherapy
Hormone
Stem cell transplant
Transformations to
None
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None