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An elderly man with diarrhea and weight loss

Published:January 02, 2020DOI:https://doi.org/10.1016/j.ejim.2019.12.024

      Keywords

      1. Case description

      A 77-year-old Japanese man presented to the author's department with diarrhea and weight loss (8 kg in the past 3 months). Vital signs were normal. Furthermore, abdominal examination was normal, and no skin lesions were observed. Esophagogastroduodenoscopy and total colonoscopy confirmed the absence of malignancy and inflammation; however, contrast-enhanced computed tomography revealed left inguinal lymphadenopathy. Laboratory evaluation showed white blood cell counts and serum lactate dehydrogenase (LDH) level were elevated at 16,500 /μL (range ≤8,500) and 272 IU/L (range ≤229), respectively. Peripheral blood smear was shown in Fig. 1. What is the diagnosis?

      2. Discussion section

      The peripheral blood smear showed an atypical lymphocyte with “flower-shaped” nucleus, also known as a “flower” cell. An additional test detected a high titer (≥1:8192) of serum human T-lymphotropic virus type 1 (HTLV-1) antibody (range ≤1:15). The patient was referred to a specialized hospital, and the diagnosis of adult T-cell lymphoma/leukemia (ATL) was made by an expert hematopathologist.
      ATL, which is characterized by the presence of “flower” cells, is a rare T-cell lymphoproliferative neoplasm caused by HTLV-1 infection. Regions with the highest incidence of HTLV-1 include the southern and northern islands of Japan, the Caribbean, Central and South America, intertropical Africa, Romania, and northern Iran [
      • Mehta-Shah N.
      • Ratner L.
      • Horwitz S.M
      Adult T-Cell leukemia/lymphoma.
      ]. Patients can be infected through transplacental transmission, mother's milk, blood transfusion, and by sexual transmission of the virus [
      • Longo D.L.
      Malignancies of lymphoid cells.
      ]. Among carriers of HTLV-1, the risk of developing ATL is only 2.5%, and the latency averages 55 years [
      • Longo D.L.
      Malignancies of lymphoid cells.
      ].
      Fig 1
      Fig. 1Peripheral blood smear showed an atypical lymphocyte demonstrating marked nuclear irregularity.
      A subset of patients have a smoldering clinical course and long survival, but most patients present with an aggressive disease manifested by lymphadenopathy, hepatosplenomegaly, skin lesions, pulmonary infiltrates, hypercalcemia, lytic bone lesions, and elevated LDH levels [
      • Longo D.L.
      Malignancies of lymphoid cells.
      ]. Gastrointestinal symptoms are nearly always related to opportunistic infection [
      • Longo D.L.
      • Fauci A.S.
      The human retroviruses.
      ]. Although treatment with combination chemotherapy regimens can result in objective responses, aggressive ATL has an extremely poor prognosis [
      • Longo D.L.
      Malignancies of lymphoid cells.
      ].

      Declaration of Competing Interest

      None declared

      References

        • Mehta-Shah N.
        • Ratner L.
        • Horwitz S.M
        Adult T-Cell leukemia/lymphoma.
        J Oncol Pract. 2017; 13: 487-492
        • Longo D.L.
        Malignancies of lymphoid cells.
        Harrison's Principles of Internal Medicine. 18th Edition. McGraw-Hill, New Yolk2012: 932-933
        • Longo D.L.
        • Fauci A.S.
        The human retroviruses.
        Harrison's Principles of Internal Medicine. 18th Edition. McGraw-Hill, New Yolk2012: 1503-1505