Circulating blastoid cells: acute leukemia, prolymphocytic leukemia, or something else?
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LETTER TO THE EDITOR
Circulating blastoid cells: acute leukemia, prolymphocytic leukemia, or something else? Yue Zhao 1,2 & Catherine Rehder 2 & Endi Wang 2 Received: 9 August 2020 / Accepted: 9 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Dear Editor, A 58-year-old male presented with mild fatigue and about 5 kg weight loss. Computed tomography revealed splenomegaly without significant lymphadenopathy. Laboratory evaluation demonstrated marked leukocytosis with moderate anemia and mild thrombocytopenia (WBC, 171.2 × 109/L; hemoglobin, 9.3 g/dL; platelets, 113 × 109/L). Peripheral blood film showed absolute leukocytosis with many circulating abnormal mononucleated cells. These abnormal cells were medium to large in size with high nuclear–cytoplasmic ratios, round, indented or convoluted nuclear contours, slightly clumped chromatin, and large prominent nucleolus. Their cytoplasm was scant to moderate, with some containing fine azurophilic granules (Fig. 1a–c). These cytomorphologic features were equivocal between blasts and circulating lymphoma cells. Therefore, the case was considered hematolymphoid neoplasm, pending flow cytometric analysis for classification. The flow cytometry detected a monoclonal B-cell population (82%) that was positive for CD19, CD20, CD22 (Fig. 1d), and restricted kappa light chain (Fig. 1e); in addition, the monoclonal B cells expressed CD5 (Fig. 1d), but was negative for CD10 and CD23. Subsequent bone marrow examination demonstrated extensive involvement by lymphoma/leukemia with 87% monoclonal B cells detected by flow cytometry. The immunophenotypic profile and cytomorphologic features raised a possibility of B-cell prolymphocytic leukemia (BPLL). Unexpectedly, chromosomal analysis demonstrated a
* Endi Wang [email protected] 1
Department of Pathology, First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, People’s Republic of China
2
Department of Pathology, Duke University Medical Center, DUMC Box 3712, M-345 Davison Bldg (Green Zone) Duke Hospital South, Durham, NC, USA
complex karyotypic abnormalities with t(11:14)(q13;q32) as a part: 45,XY,del(1)(p13p32),del(2)(q31q33), t(11;14)(q13;q32),del(14)(q22q24),add(17)(p13),-19[7]/ 45,idem,add(21)(q22)[8]/46,XY[5]. Fluorescence in situ hybridization (FISH) was positive for CCND1/IGH fusion in metaphase cells and in 93% of the total interphase cells (Fig. 1f), endorsing the diagnosis of leukemic presentation of mantle cell lymphoma with prolymphocytoid morphology. In addition, FISH demonstrated deletion of TP53 gene in 96.5% of the interphase nuclei (data not shown). The patient was treated with R-DHAX protocol (rituximab, dexamethasone, highdose cytarabine, and oxaliplatin), and reached complete remission after completing 4 cycles (3 months after the diagnosis). He subsequently received high-dose chemotherapy with BEAM regimen (NCNU, etoposide, cytarabine, and melphalan) followed by autologous hematopoietic stem cell transplant 6 months after the diagnosis.
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