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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 [
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 [
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