Adult T-cell Leukemia/Lymphoma

Adult T-cell Leukemia/Lymphoma

Aaron Auerbach, MD, PhD

This clinical photograph shows an ulcerating lesion image, which is 2.5 cm in greatest dimension, from the foot of a person from the Caribbean region with ATLL, HTLV-1(+).

This ATLL involves the mucosa. Note the small lymphocytes with irregular hyperchromatic nuclei infiltrating squamous mucosa, forming Pautrier-like microabscesses image. (Courtesy C. Bueso-Ramos, MD.)



  • Adult T-cell leukemia/lymphoma (ATLL)


  • Adult T-cell leukemia


  • T-cell lymphoma/leukemia of regulatory T cells (CD4[+], CD25[+], FOXP3[+]) caused by human T-cell leukemia virus type 1 (HTLV-1)


Infectious Agents

  • Human T-cell leukemia virus type 1

    • Progresses to leukemia/lymphoma in < 5% of infected individuals

    • Long latency period

      • Most exposed as infants/children

      • Tumor often after 20 years of viral exposure

    • Transmitted through blood or breast milk

    • Also causes tropical spastic paraparesis

    • Encodes Tax (a viral oncoprotein)

      • Plays a role in development of ATLL

      • Activates transcription factors for T-cell proliferation genes



  • Age

    • Adults, most commonly in 50s or 60s

  • Gender

    • Male:female = 1.5:1.0

  • Ethnicity

    • Endemic to southwest Japan, Caribbean islands, South America, and central Africa


  • Skin lesions

    • Develop in 50% of ATLL patients

    • Usually multiple nodules/tumors, papules, plaques, or macules

      • Papules may cover large segments of body

    • Rarely, erythroderma, purpura, prurigo, or erythema multiforme

  • Other sites of disease include lymph nodes, peripheral blood, bone, spleen and sometimes lung, liver, and CNS

    • Most patients have generalized lymphadenopathy

    • Hypercalcemia from increased osteoclast bone resorption

  • 4 clinical presentations: Acute, chronic, lymphoma, and smoldering

    • Acute subtype

      • Most common subtype

      • Severe peripheral blood lymphocytosis, ↑ WBC, ↑ LDH, skin lesions, lymphadenopathy

      • Hypercalcemia and lytic bone lesions

      • Immunodeficiency leads to other opportunistic infections

    • Chronic subtype

      • Lymphocytosis, but ↓ WBC than acute subtype

      • No hypercalcemia, exfoliative skin rash

    • Lymphomatous subtype

      • ↑ lymphadenopathy; usually no peripheral blood involvement

      • Less hypercalcemia than acute subtype; skin often involved

    • Smoldering subtype

      • > 5% tumor cells in peripheral blood, even though normal WBC count

      • Tumor cells small with less pleomorphism and lobation

      • No hypercalcemia or lymphadenopathy

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Adult T-cell Leukemia/Lymphoma

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