Central nervous system infiltration by HTLV-1-associated T-cell leukemia/lymphoma in an AIDS patient

A 59-year-old woman was admitted for progressive ataxia and decreased consciousness, which had commenced two months previously. The patient had human immunodeficiency virus (HIV)/humanT-cell lymphotropic virus-1 (HTLV-1) co-infection for 10 years with regular use of antiretroviral therapy, resulting in satisfactory virological control (undetectable HIV load, CD4+ T lymphocyte count: 354 cells/mm³). Laboratory data revealed leukocytosis (142.3 × 10⁹/L - 78% lymphocytes, some with “flower cell” morphology [Figure 1]), hypercalcemia, elevated serum lactate dehydrogenase, and acute renal dysfunction without anemia or thrombocytopenia. Cranial computed tomography scans revealed calcification in basal ganglia. Cerebral spinal fluid (CSF) analysis revealed 30 cells/mm³ (86% atypical lymphocytes). Cytomegalovirus and Toxoplasma gondii IgM and IgG antibody screening were negative. No infectious agents were identified by CSF direct analysis and culture.

FIGURE 1:
Atypical lymphocytes in peripheral blood showing classical “flower cell” morphology. Leishman stain, magnification × 1000.

Blood and CSF lymphocyte immunophenotyping by flow cytometry revealed positivity for CD3, CD4, CD5, CD25, and CD38 markers, and negativity for CD8 (Figure 2)¹. A diagnosis of central nervous system infiltration (lymphomatous meningitis) by HTLV-1-associated adult T-cell leukemia/lymphoma (acute subtype) was considered². No test for detecting clonal integration of the HTLV-1 pro-virus within tumor cells was conducted. Systemic and intrathecal chemotherapy were administrated. The patient died due to Pseudomonas aeruginosa infection 25 days later.

FIGURE 2:
Blood lymphocyte immunophenotyping by flow cytometry. Lymphocytes (marked by red color in dot-plots) were CD3+ (A), CD5+ (B), and CD25+ (C). D: CD3+ T-lymphocytes with CD4+/CD8- phenotype (marked by pink color in dot-plots) in 97.3% of cells analyzed.

The spectrum of complications associated with HTLV-1 infection is broad, with predominant hematological and neurological manifestations³. The detection of lymphocytes with “flower cell” morphology may be useful for investigation of HTLV-1 infection. Guidelines for standardizing follow-up of patients with HTLV-1 infection should be considered for early detection of potential infection-related complications.

REFERENCES:

• ¹
  Bazarbachi A, Suarez F, Fields P, Hermine O. How I treat adult T-cell leukemia/lymphoma. Blood. 2011;118(7):1736-45.
• ²
  Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). Br J Haematol. 1991;79(3):428-37.
• ³
  Araújo AQ, Leite AC, Lima MA, Silva MT. HTLV-1 and neurological conditions: when to suspect and when to order a diagnostic test for HTLV-1 infection? Arq Neuropsiquiatr. 2009;67(1):132-8.

Publication Dates

• Publication in this collection
  12 June 2020
• Date of issue
  2020

History

• Received
  23 Feb 2020
• Accepted
  22 Apr 2020