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Adult T-cell leukemia/lymphoma
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Adult T-cell leukemia/lymphoma

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Contributors: Nina Haghi MD, Carla Casulo MD, Paritosh Prasad MD
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Synopsis

Adult T-cell leukemia/lymphoma (ATLL) is a mature peripheral T-cell neoplasm associated with human T-cell lymphotropic virus type 1 (HTLV-1) infection. HTLV-1 is endemic in southwestern Japan, the Caribbean, and parts of central Africa. ATLL occurs exclusively in adults (average patient age is 58 years old) with a male to female ratio of 1.5:1.

Four clinical variants exist on a spectrum: acute (most common), lymphomatous, chronic, and smoldering. They vary based on their presentation and prognosis.

The acute variant (~60% of cases) is characterized by a high WBC count, generalized lymphadenopathy, hepatosplenomegaly, high lactate dehydrogenase (LDH), and hypercalcemia (with or without lytic bone lesions). Bone marrow involvement is seen in up to one-third of cases. It has a poor prognosis, and the median survival is approximately 8 months.

The lymphomatous variant (~20% of cases) presents with significant lymphadenopathy and absence of peripheral blood involvement. The median survival is 11 months.

The chronic variant (~10% of cases) usually presents with an exfoliative skin rash, mild lymphadenopathy, and leukocytosis and has a median survival of 32 months, while the smoldering variant (~10% of cases) shows a normal WBC count with presence of circulating neoplastic cells and a median survival of 55 months. Occasionally, skin involvement is the only finding in the smoldering subtype.

Skin rash can be present in any of the variants, as well as pulmonary lesions. Cutaneous findings are seen in around 40%-70% of patients with ATLL. These are heterogeneous and may include a widespread papular eruption, patches and plaques (may resemble mycosis fungoides), erythroderma, and, rarely, a purpuric variant.

Patients with ATLL are immunosuppressed and at risk of developing opportunistic infections.

Codes

ICD10CM:
C91.00 – Acute lymphoblastic leukemia not having achieved remission

SNOMEDCT:
110007008 – Adult T-cell leukemia/lymphoma

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Last Reviewed: 03/19/2018
Last Updated: 04/17/2018
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Adult T-cell leukemia/lymphoma
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Adult T-cell leukemia/lymphoma (Acute) : Hepatosplenomegaly, Erythema, Lytic bone lesions, Tumor, Smooth papules, WBC elevated, Fine scaly plaques, Ca elevated
Clinical image of Adult T-cell leukemia/lymphoma
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