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PerCP-Cy5-5-A CD5
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PE-Cy7-A CD19 PerCP-Cy5-5-A CD11c FITC-A Kappa
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A 72-year-old man presents with leukocytosis (30 3 109/L-40 3 cytometry of marrow confirmed 2 clonal B-cell populations: the
109/L) with lymphocytosis (80%), monocytopenia, and anemia first (red) was CD191CD52 (panel J), CD221CD11c1 (panel K),
for 10 years. Peripheral smear revealed cells with cytoplasmic CD201CD251CD1031CD1231CD232 (not shown), and l light
projections and atypical lymphocytes (panel A [Giemsa stain; chain–positive (panel L), diagnostic of HCL. A second population
original magnification 31000]). A bone marrow biopsy showed (magenta) was CD191CD51 (panel J), CD221CD11c2 (panel K),
areas of trilineage hematopoiesis and an interstitial infiltrate of CD201 (moderate) CD231 (spectrum) CD432CD2002CD1032
lymphoid cells (panel B [hematoxylin and eosin (H&E) stain]) with (not shown), and k light chain–positive (panel L), representing
irregular nuclei and ample cytoplasm (panel C [H&E stain]). atypical chronic lymphocytic leukemia. B-cell clonality testing
Immunohistochemistry revealed cells double positive for by immunoglobulin heavy chain polymerase chain reaction
PAX5 (brown) and tartrate-resistant acid phosphatase (red; showed 3 significant peaks (panel M) consistent with 2 distinct
panel D), PAX5 (brown) and CD103 (red; panel E), annexin clonal B-cell populations. The patient was initially treated with
(panel F), and BRAFV600E (panel G) diagnostic of hairy cell leu- splenectomy, and later with trametinib (MEK inhibitor) and
kemia (HCL). A second distinct population dual positive for PAX5 dabrafenib (BRAF inhibitor).
(brown) and CD5 (red; panel H) in combination with LEF-1 (panel
I), and negative for cyclin D1/SOX11, indicated synchronous This case highlights the indolent course of HCL and its recog-
involvement by CD51 lymphoproliferative disorder (LPD). Flow nized association with other B-cell LPD.
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DOI 10.1182/blood-2018-12-885145
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