1 Department of Hematology and Oncology, Tokyo University Hospital, Japan.
2 Departments of Hematology and Laboratory Medicine, Aiseikai Yamashina Hospital, Japan.
3 Department of Infection Control and Laboratory Medicine, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Japan.
4 Division of Hematology and Oncology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Japan.
5 Center for Molecular Diagnostics and Therapeutics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Japan.
| This is an Open Access article distributed
under the terms of the Creative Commons Attribution License
(https://creativecommons.org/licenses/by-nc/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
B-cell lymphoproliferative disorder (HBLD) is one of chronic polyclonal
B-cell lymphocytosis. We report a 47-year-old female Japanese patient
diagnosed as having HBLD based on lymphocytosis with hairy cell
appearance and characteristic phenotypes including CD11c+ and without
B-cell monoclonality. She was a non-smoker and possessed HLA-DR4. She
has been closely followed up without treatment and lymphoma development
for over five years. Although this disease is quite rare and has been
reported, to our knowledge, in only 13 Japanese cases, an accurate
diagnosis, particularly differential diagnosis from persistent
polyclonal B-cell lymphocytosis or hairy cell leukemia-Japanese variant
is essential for the prevention of unnecessary treatments.
|Figure 1. Peripheral blood smears (A) and electron microscopic examination (B) show atypical lymphocytes with partially hairy projections and abundant irregularly shaped cytoplasms. No ribosome-lamella complex was found.|
|Figure 2. Detection of immunoglobulin heavy chain (IGH) gene recombinations using multiplex polymerase chain reaction (PCR) using a tube A primers set (VH FR1) and one consensus primer (JH) in accordance with the BIOMED-2 protocol. Many different IGH PCR products without a monoclonal peak were detected.|
|Table 1. Comparison of previously reported cases of hairy B-cell lymphoproliferative disorder (HBLD), persistent polyclonal B-cell lymphocytosis (PPBL), and hairy cell leukemia-Japanese variant (HCL-Jv).|