Adult T-cell leukemia/lymphoma.

Area of Science:

• Oncology
• Virology
• Hematology

Background:

• Adult T-cell leukemia/lymphoma (ATLL) is a rare CD4(+) T-cell neoplasm.
• It is caused by human T-lymphotropic virus type 1 (HTLV-1), with 20 million infected globally.
• Endemic areas include southern Japan, Africa, the Caribbean, and Latin America; cases in the US are often in immigrants.

Purpose of the Study:

• To review the salient features of ATLL to aid in its recognition.
• To guide a comprehensive diagnostic workup for this rare entity.
• To address the diagnostic challenges associated with ATLL.

Main Methods:

• This is a review article.
• It synthesizes information on ATLL epidemiology, subtypes, and clinical presentation.
• Focus is on features aiding diagnosis and workup.

Main Results:

• ATLL has four subtypes: acute, lymphomatous (aggressive), chronic, and smoldering (indolent).
• The disease predominantly affects adults and is rare in children.
• Recognition and diagnosis can be challenging due to its rarity.

Conclusions:

• ATLL requires careful diagnostic consideration, especially in endemic populations or immigrants.
• Understanding the subtypes and clinical spectrum is crucial for appropriate management.
• This review provides key features for improved ATLL recognition and workup.