Blood Res 2016; 51(1): 7  https://doi.org/10.5045/br.2016.51.1.7
Extranasal natural killer/T-cell lymphoma initially presenting as myelofibrosis
Woori Jang, and Myungshin Kim*

Department of Laboratory Medicine, Catholic Genetic Laboratory Center, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Correspondence to: Correspondence to Myungshin Kim, M.D., Ph.D., Department of Laboratory Medicine, Catholic Genetic Laboratory Center, College of Medicine, The Catholic University of Korea, 222 Banpodaero, Secho-gu, Seoul 06591, Korea, microkim@catholic.ac.kr
Received: December 17, 2014; Revised: December 26, 2014; Accepted: January 20, 2015; Published online: March 25, 2016.
© The Korean Journal of Hematology. All rights reserved.

cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
 

figure

A 45-year-old woman was admitted with fever, generalized weakness, and 2 kg weight loss in 1 month. Two years prior, she was diagnosed with JAK2 V617F-negative primary myelofibrosis (MF-2) (A, hematoxylin-eosin [H&E] stain, ×200; upper right inset, reticulin stain, ×400; lower left inset, peripheral blood, Wright stain, ×1,000) with 46,XX, add(1)(q21)[8]/46,XX[22]. Complete blood count revealed the following values: white blood cells, 0.9×109/L, with a few atypical lymphocytes; hemoglobin, 9.0 g/dL; and platelets, 21×109/L. Chest computed tomography revealed several reactive lymph nodes and hepatosplenomegaly. Bone marrow biopsy revealed diffuse fibrosis (MF-2) and histiocytosis with active hemophagocytosis (B, H&E stain, ×200; upper right inset, reticulin stain, ×400). Ten days after admission, the atypical lymphoid cells in peripheral blood reached 95% (B, lower left inset, Wright stain, ×1,000) and showed CD2, cytoplasmic CD3, CD7, CD45, CD56, and HLA-DR positivity. Chromosomal analysis revealed 46,X,-X,+der(1)ins(1;1)(q21;q32q44 or q44q32)t(1;5)(q44;q33),der(21)t(X;21)(q22;p13)[20] by multicolor fluorescent in situ hybridization analysis (C). Clonal TCR β- and γ-chain rearrangements were found in the present specimen, and identically rearranged TCR genes were found during initial diagnosis (D). NK function analysis revealed decreased NK-cell-mediated cytotoxicity (E). Accordingly, the patient was diagnosed with extranasal NK/T-cell lymphoma initially presenting as myelofibrosis.



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