Volume 77, Issue 2 pp. 262-274
Original Article

Immunopathology of Kikuchi–Fujimoto disease: A reappraisal using novel immunohistochemistry markers

Narittee Sukswai

Narittee Sukswai

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

Department of Pathology, Chulalongkorn University, Bangkok, Thailand

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Hye Ra Jung

Hye Ra Jung

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

Department of Pathology, Keimyung University, Dongsan Medical Center, Seoul, South Korea

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Samir S Amr

Samir S Amr

Department of Pathology, Johns Hopkins Aramco Healthcare, Dhahran, Saudi Arabia

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Siok Bian Ng

Siok Bian Ng

Department of Pathology, National University Hospital, Singapore

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Salwa S Sheikh

Salwa S Sheikh

Department of Pathology, Johns Hopkins Aramco Healthcare, Dhahran, Saudi Arabia

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Kirill Lyapichev

Kirill Lyapichev

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

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Siba El Hussein

Siba El Hussein

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

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Sanam Loghavi

Sanam Loghavi

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

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Rose Lou Marie C Agbay

Rose Lou Marie C Agbay

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

Department of Pathology, The Medical City Hospital, Manila, Philippines

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Roberto N Miranda

Roberto N Miranda

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

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L Jeffrey Medeiros

L Jeffrey Medeiros

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

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Joseph D Khoury

Corresponding Author

Joseph D Khoury

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TV, USA

Address for correspondence author: Address for correspondence author: Joseph D. Khoury, MD, The University of Texas MD Anderson Cancer Center, Department of Hematopathology, 1515 Holcombe Boulevard, MS-072, Houston, TX 77030, USA. e-mail: [email protected]

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First published: 19 December 2019
Citations: 17
N.S. and H.R.J. contributed equally to this work.

Abstract

Aims

Kikuchi–Fujimoto disease (KFD) is a self-limited disease characterised by destruction of the lymph node parenchyma. Few studies have assessed the immunohistological features of KFD, and most employed limited antibody panels that lacked many of the novel immunohistochemistry markers currently available.

Methods and results

We used immunohistochemistry to reappraise the microanatomical distribution of plasmacytoid dendritic cells (pDCs), follicular helper T cells and cytotoxic T cells, B cells, follicular dendritic cell (FDC) meshworks, and histiocytes in lymph nodes involved by KFD. The study group consisted of 138 KFD patients (89 women; 64.5%) with a median age of 27 years (range, 3–50 years). Cervical lymph nodes were most commonly involved, in 108 (78.3%) patients. The numbers of pDCs were increased, predominantly around and within apoptotic areas and the paracortex, and tapering off within xanthomatous areas. pDCs formed sizeable tight clusters, most notably around apoptotic/necrotic areas. T cells consisted mostly of CD8-positive cells with predominant expression of T-cell receptor-β. There were notable increases in the numbers of CD8-positive T cells within lymphoid follicles, and their numbers correlated with alterations in FDC meshworks (P < 0.001). The number of follicular helper T cells was decreased within distorted FDC meshworks. CD21 highlighted frequent distortion of FDC meshworks, even in lymph node tissue that was distant from apoptotic/necrotic areas. Distorted FDC meshworks spanned all morphological patterns, and FDC meshwork characteristics (intact; distorted; remnant/nearly absent) correlated with morphological patterns (P < 0.01).

Conclusions

The immunohistological landscape of KFD is complex and characterised by increased numbers of pDCs that frequently cluster around apoptotic/necrotic foci, increased numbers of cytotoxic T cells, and substantial distortion of FDC meshworks.

Conflicts of interest

None of the authors has declared any competing financial interests.

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