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Effective
2001 and later
Reportable
for cases diagnosed
1992 and later
Primary Site(s)
See Module 4: Rules PH7, PH8
Most common sites of involvement: skin, lung, liver, GI tract, CNS and bone marrow
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Adult T-cell leukemia/lymphoma (ATLL) has widespread lymph node and peripheral blood involvement. It is very rare for a patient to present with only bone marrow involvement.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Grade
Not Applicable
Module Rule
Module 4: PH7, PH8
Alternate Names
Adult T-cell lymphoma
Definition
Adult T-cell leukemia/lymphoma (ATLL) is a mature T-cell neoplasm most often composed of highly pleomorphic lymphoid cells. The disease is usually widely disseminated and is caused by the human retrovirus HTLV-1. Most ATLL patients present with widespread lymph node involvement as well as involvement of peripheral blood. The histology shows remarkable pleomorphism, with several morphological variants having been described. The leukemic cells often show a multilobed appearance of so-called flower cells. Neoplastic cells show monoclonal integration of HTLV-1 and expression T-cell-associated antigens.
Several clinical variants of ATLL have been identified
1. The acute variant is most common and is characterized by a leukemic phase, often with a markedly elevated white blood cell count, skin rash, and generalized lymphadenopathy. Hypercalcemia, with or without lytic bone lesions, is a common feature. Patients with acute ATLL have systemic disease accompanied by hepatosplenomegaly, constitutional symptoms, and elevated lactate dehydrogenase (LDH). Cutaneous lesions are common.
2. The lymphomatous variant is characterized by prominent lymphadenopathy but without peripheral blood involvement. Most patients present with advanced stage disease similar to the acute form, although hypercalcemia is seen less often. Cutaneous lesions are common.
3. The chronic variant is frequently associated with an exfoliative skin rash. An absolute lymphocytosis may be present, but atypical lymphocytes are not numerous in the peripheral blood. Hypercalcemia is absent.
4. In the smoldering variant, the WBC count is normal with > 5% circulating neoplastic cells. ATLL cells are generally small, with a normal appearance. Patients frequently have skin or pulmonary lesions, but there is no hypercalcemia. Progression from the chronic or acute variant occurs in 25% of cases, usually after a long duration.
Several clinical variants of ATLL have been identified
1. The acute variant is most common and is characterized by a leukemic phase, often with a markedly elevated white blood cell count, skin rash, and generalized lymphadenopathy. Hypercalcemia, with or without lytic bone lesions, is a common feature. Patients with acute ATLL have systemic disease accompanied by hepatosplenomegaly, constitutional symptoms, and elevated lactate dehydrogenase (LDH). Cutaneous lesions are common.
2. The lymphomatous variant is characterized by prominent lymphadenopathy but without peripheral blood involvement. Most patients present with advanced stage disease similar to the acute form, although hypercalcemia is seen less often. Cutaneous lesions are common.
3. The chronic variant is frequently associated with an exfoliative skin rash. An absolute lymphocytosis may be present, but atypical lymphocytes are not numerous in the peripheral blood. Hypercalcemia is absent.
4. In the smoldering variant, the WBC count is normal with > 5% circulating neoplastic cells. ATLL cells are generally small, with a normal appearance. Patients frequently have skin or pulmonary lesions, but there is no hypercalcemia. Progression from the chronic or acute variant occurs in 25% of cases, usually after a long duration.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
HTLV-1 monoclonal integration
TR genes are clonally rearranged
Immunophenotyping
CCR4+ (expression/positive)
CD2+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD5+ (expression/positive)
CD7- (no expression/negative)
CD8+ (expression/positive)
CD25+ (expression/positive)
FOXO3+ (expression/positive)
Treatments
Chemotherapy
Immunotherapy
Radiation therapy
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.5 Adult T-cell leukemia
Corresponding ICD-10-CM Codes (U.S. only)
C91.5 Adult T-cell lymphoma/leukemia (HTLV-1-associated) (effective October 01, 2015)
Signs and Symptoms
Elevated white blood cell count
Exfoliative skin rash
Fatigue
Fever
Hepatosplenomegaly
Hypercalcemia
Lymphadenopathy
Lymphocytosis
Pneumocystitis
Weight loss
Widespread lymph node involvement
Diagnostic Exams
Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Lymph node biopsy
Peripheral blood smear
PET (positron emission tomography) scan
Progression and Transformation
Progression from the chronic or smoldering to the acute variant occurs in 25% of cases, but usually after a long duration.
Epidemiology and Mortality
Age: only in adults, 58 years median age
Country: Southwestern Japan, Caribbean, Central Africa
Sex: female predominance
Survival: 2 weeks to greater than 1 year depending on prognostic factors
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 363-367
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 363-367
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
