Adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma
ICD-O-2 Morphology
9827/3: Adult T-cell leukemia/ lymphoma (HTLV-1 positive)
Effective 1992 - 2000
ICD-O-3 Morphology
9827/3: Adult T-cell leukemia/ lymphoma (HTLV-1 positive)
Effective 2001 and later
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

Not Applicable
Module Rule
Module 4: PH7, PH8
Alternate Names
Acute adult T-cell leukemia/lymphoma
Adult T-cell leukemia/lymphoma (HTLV-1 positive)
Adult T-cell leukemia/lymphoma (HTLV-1)
Lymphomatous adult T-cell leukemia/lymphoma
Smoldering adult T-cell leukemia/lymphoma
T-cell lymphoma, small cell type, pleomorphic medium and large cell type (HTLV-1+)-Kiel
ATLL is a peripheral T-cell neoplasm most often composed of highly pleomorphic lymphoid cells.

The degree of circulating cells in the bone marrow does not correlate with the circulating neoplastic cells in the peripheral blood suggesting that the neoplastic cells in the bone marrow are recruited from other organs such as the skin.

The disease is usually widely disseminated, and is caused by the human retrovirus, human T-cell leukemia virus type 1 (HTLV-1).
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.

Several variants of this neoplasm have been identified:
1. Acute ATLL variant-characterized by a leukemic phase with systemic disease accompanied by hepatosplenomegaly and constitutional symptoms. Hypercalcemia with or without lytic bone lesions are common. Most patients have an associated T-cell immunodeficiency with frequent opportunistic infections. HTLV-1 is usually linked to ATLL, but HTLV-1 infection alone is not sufficient to result in neoplastic transformation of infected cells. Most common variant.

2. Lymphomatous variant-characterized by prominent lymphadenopathy but without peripheral blood involvement. Most patients present with advanced stage disease similar to the acute form, although hypercalcemia is less often seen. Cutaneous lesions are common in the lymphomatous form. They are clinically diffuse and include erythematous rashes, papules, and nodules. Larger nodules may show ulceration.

3. Chronic variant-characterized by an exfoliative skin rash. While an absolute lymphocytosis may be present, atypical lymphocytes are not numerous in the peripheral blood. Hypercalcemia is absent.

4. Smoldering variant-characterized by normal white blood count (WBC) with >5% of circulating neoplastic cells. ATLL cells are generally small with a normal appearance. Patients frequently have skin or pulmonary lesions, but there is no hypercalcemia.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Histologic confirmation
Genetics Data
T-cell receptor (TCR) genes show integration of HTLV-1
Radiation therapy
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
202.7 Peripheral T-cell lymphoma (Lymphoma presentation)
Corresponding ICD-10 Codes
C91.5 Adult T-cell leukemia
C84.4 Peripheral T-cell lymphoma, not classified (Lymphoma presentation)
Corresponding ICD-10-CM Codes (U.S. only)
C91.5 Adult T-cell lymphoma/leukemia (HTLV-1 associated) (effective October 01, 2015)
C84.4 Peripheral T-cell lymphoma, not classified (Lymphoma presentation) (effective October 01, 2015)
Signs and Symptoms
Elevated white blood cell count
Exfoliative skin rash
Skin or pulmonary lesions
Weight loss
Widespread lymph node involvement
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