Case Reports

Human T-Lymphotropic Virus 1 Associated With Adult T-Cell Leukemia/Lymphoma

Author and Disclosure Information

Adult T-cell leukemia/lymphoma (ATLL) is an uncommon neoplasm of mature T lymphocytes associated with infection by human T-lymphotropic virus 1 (HTLV-1), which is increasing in incidence in areas of the United States with large immigrant populations. Human T-lymphotrophic virus 1 infection is asymptomatic in most patients and has been associated with ATLL as well as tropical spastic paraparesis. Because there is considerable histologic overlap with other cutaneous T-cell lymphomas, high suspicion and clinical features must be present to make the diagnosis of ATLL.

Practice Points

  • Adult T-cell leukemia/lymphoma (ATLL) is an uncommon neoplasm of mature T lymphocytes associated with infection by human T-lymphotropic virus 1.
  • In the United States, ATLL is increasing in incidence in areas with large immigrant populations.
  • High suspicion and clinical features must be present to make the diagnosis of ATLL due to considerable histologic overlap with other cutaneous T-cell lymphomas.


 

References

Adult T-cell leukemia/lymphoma (ATLL) is an uncommon neoplasm of mature T lymphocytes associated with infection by human T-lymphotropic virus 1 (HTLV-1),1-3 which is increasing in incidence in areas of the United States with large immigrant populations.4 Human T-lymphotrophic virus 1 infection is asymptomatic in most patients and has been associated with ATLL as well as tropical spastic paraparesis.5 We present a case of rapid-onset ATLL in an 82-year-old Japanese man who had immigrated to the United States.

Case Report

An 82-year-old Japanese man who had immigrated to the United States presented with papules and nodules on the neck, trunk, and arms of 4 weeks’ duration. Minimal pruritus was associated with the lesions, which were otherwise asymptomatic. The patient reported that he was generally healthy, and a review of systems was negative.

Physical examination revealed numerous erythematous and violaceous papules and nodules on the right side of the neck (Figure 1A), chest, back, abdomen, groin, left arm (Figure 1B), and medial thighs. Bilateral axillary and inguinal lymphadenopathy also was noted.

Figure1

Figure 1. Numerous erythematous and violaceous papules and nodules on the right side of the neck (A) and left arm (B) in a patient with adult T-cell leukemia/lymphoma.

A biopsy from the abdomen revealed a dense, atypical, pandermal lymphoid infiltrate comprised of medium-sized lymphocytes with oval nuclei, fine chromatin, and pale cytoplasm (Figure 2). Mitotic figures and apoptotic cells also were observed. Immunostaining was strongly and diffusely positive for CD4 (Figure 3A), B-cell lymphoma 2 (Bcl-2)(Figure 3B), CD3, and programmed death 1, and was negative for CD8, CD10, CD20, CD30, and myeloperoxidase.

Figure2

Figure 2. Histopathology revealed a dense, atypical, pandermal lymphoid infiltrate comprised of medium-sized lymphocytes with oval nuclei, fine chromatin, and pale cytoplasm (H&E, original magnification ×20).

Figure3

Figure 3. Immunostaining was strongly and diffusely positive for CD4 (A)(original magnification ×40) and B-cell lymphoma 2 (Bcl-2)(B)(original magnification ×40).

A bone marrow biopsy revealed an atypical T-cell population on flow cytometry. Western blot analysis for HTLV-1 antibodies was positive. Complete blood cell count and complete metabolic panel were within reference range.

Clinical and histopathologic findings fit the diagnosis of ATLL. The patient was referred to hematology/oncology, but the rapid progression of lesions continued, and the patient died within 4 months of initial presentation.

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