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 Module 7
Most common sites of involvement: lymph nodes, skin, bone, soft tissue, lung, and liver. Less common sites of involvement: GI, CNS.

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 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

Bone marrow involvement occurs in approximately 10-30% depending on the tests performed. The small cell variant may have a leukemic presentation with peripheral blood involvement.

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
CD2+
CD3-
CD4+
CD5+
CD30+
CD45+/-
Positive/Negative: 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
C85.7 Other specified types of non-Hodgkin's lymphoma
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)
Pain in the chest, abdomen, or bones (for no known reason)
Peripheral and abdominal lymphadenopathy
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
Recurrence and Metastases
None
Epidemiology and Mortality
Age: most frequently occurs in 30's (occurs in children and adults)
Incidence: 3% of adult and 10-20% of childhood non-Hodgkin lymphomas
Sex: slight male predominance
Survival: 5 year survival 60-80%