Adult T-cell leukemia/lymphoma

Name
Adult T-cell leukemia/lymphoma
ICD-O-2 Morphology
9827/3
Effective 1992 - 2000
ICD-O-3 Morphology
9827/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Abstractor Notes and Module 4: Rules PH7, PH8

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Grade
5 - T-cell
Module Rule
Module 4: PH7, PH8
Alternate Names
Acute adult T-cell leukemia/lymphoma
Adult T-cell leukemia
Adult T-cell leukemia/lymphoma (HTLV-1 positive)
Adult T-cell leukemia/lymphoma (HTLV-1)
Adult T-cell lymphoma
ATLL
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
Definition
ATLL is a peripheral T-cell neoplasm most often composed of highly pleomorphic lymphoid cells. 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) present with widespread lymph node involvement as well as involvement of the peripheral blood. It is very rare for a patient to present with only bone marrow involvement (primary site bone marrow). 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 skin is the most common extralymphatic site of involvement. The disease is usually systemic, involving the spleen and extranodal sites including skin, lung, liver, GI tract, and CNS. HTLV-1 is usually linked to ATLL, but HTLV-1 infection alone is not sufficient to result in neoplastic transformation of infected cells. Several clinical variants have been identified: acute, lymphomatous, chronic, and smoldering ATLL.

Acute ATLL variant is the most common and is characterized by a leukemic phase often with a markedly elevated WBC, skin rash, and generalized lymphadenopathy. Hypercalcemia with or without lytic bone lesions is a common feature. Patients with acute ALL have a systemic disease accompanied by hepatosplenomegaly and constitutional symptoms. Most patients have an associated T-cell immunodeficiency with frequent opportunistic infections.

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 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.

The chronic variant is frequently associated with an exfoliative skin rash. While an absolute lymphocytosis may be present, atypical lymphocytes are not numerous in the peripheral blood. Hypercalcemia is absent.

In the smoldering variant of ALL the WBC count is normal 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. Progression from the chronic or smoldering to the acute variant occurs in 25% of cases, but usually after a long duration.
Definitive Diagnostic Methods
Bone Marrow biopsy
Histological confirmation
Immunophenotyping
Genetics Data
T-cell receptor (TCR) genes show integration of HTLV-1
Immunophenotyping
CD2+
CD3+
CD5+
CD7-
CD25+
Treatments
Chemotherapy
Immunotherapy
Radiation
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.5 Adult T-cell leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.5 Adult T-cell lymphoma/leukemia (HTLV-1 associated)
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None