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CD27-positive hairy cell leukemia-Japanese variant

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Abstract

We report a very rare case of a 45-year-old Japanese male patient with hairy cell leukemia-Japanese variant (HCL-JV) expressing CD27. The patient showed a high number of abnormal peripheral lymphocytes, thrombocytopenia, and severe splenomegaly but no lymphadenopathy. Histology of the resected spleen showed small-sized lymphoma cells diffusely infiltrating the red pulp without follicle formation. By immunohistochemistry, lymphoma cells were negative for CD3, CD5, CD8, CD10, CD34, cyclin-D1, and annexin A1 but positive for CD20 and BCL2. BRAF V600E mutation was not observed. Bone marrow aspirate showed preserved normal hematopoietic cells with invasion of lymphoma cells in an interstitial pattern without obvious nodules. The cells had abundant pale cytoplasm and round nuclei with inconspicuous nucleoli. After natural drying, the cells had unevenly distributed microvilli. Flow cytometric analysis demonstrated positivity for CD11a, CD11c, CD19, CD20, CD22, CD27, surface IgG, and λ but not for CD2, CD3, CD4, CD5, CD7, CD8, CD10, CD21, CD23, CD25, CD30, CD34, CD38, CD43, CD56, CD57, CD103, IgD, IgM, and κ. Monoclonal expansion of B cells was confirmed by an immunoglobulin heavy chain (IgH) rearrangement band as demonstrated by Southern blot hybridization. The lymphoma cells had unevenly distributed long, large, and broad-based microvilli, which resembled splenic diffuse red pulp small B cell lymphoma (SDRPL) cells. CD27 expression is extremely rare in HCL-JV, but the young age of the patient and high peripheral WBC counts were similar to HCL-JV, which suggests, in this case, an intermediate disease between SDRPL and HCL-JV.

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Author notes

  1. Rie Tabata

    Present address: Department of Hematology and Oncology, Hyogo Prefectural Amagasaki General Medical Center, 2-17-77 Higashinaniwa-cho, Amagasaki, Hyogo, 660-8550, Japan

Authors and Affiliations

  1. Department of Hematology and Oncology, Hyogo Prefectural Tsukaguchi Hospital, Hyogo, Japan

    Rie Tabata

  2. Hyogo College of Medicine Cancer Center, Hyogo, Japan

    Chiharu Tabata

  3. Department of Surgery, Hyogo Prefectural Tsukaguchi Hospital, Hyogo, Japan

    Hideaki Iwama

  4. Department of Pathology, Hyogo Prefectural Tsukaguchi Hospital, Hyogo, Japan

    Ryoji Yasumizu

  5. Department of Anatomic and Diagnostic Pathology, Dokkyo Medical University School of Medicine, Tochigi, Japan

    Masaru Kojima

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  1. Rie Tabata
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  3. Hideaki Iwama
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  4. Ryoji Yasumizu
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Correspondence to Rie Tabata.

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Supplementary Fig. 1

Flow cytometric analysis of peripheral blood Flow cytometric analysis of peripheral blood cells revealed that the cells were positive for CD11a, CD11c, CD18, CD19, CD20, CD22, CD27, CD40, CD44, CD45, CD49d, CD54, CD58, CD86, FMC7, HLA-DR, cytoplasmic CD79a, surface IgG, and λ, weakly positive for CD11b, CD80, and cytoplasmic CD68, and partially positive for CD29. They were negative for CD2, CD3, CD4, CD5, CD7, CD8, CD10, CD13, CD14, CD16, CD21, CD23, CD24, CD25, CD28, CD30, CD34, CD38, CD40L, CD43, CD56, CD57, CD62L, CD103, CD122, CD126, CD138, CD152, IgD, IgM, and κ. (JPEG 1856 kb)

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Tabata, R., Tabata, C., Iwama, H. et al. CD27-positive hairy cell leukemia-Japanese variant. Virchows Arch 468, 375–379 (2016). https://doi.org/10.1007/s00428-015-1881-x

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  • DOI: https://doi.org/10.1007/s00428-015-1881-x

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