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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).
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
T-cell receptor (TCR) genes show integration of HTLV-1