Histology-independent signature distinguishes kikuchi-fujimoto disease/systemic lupus erythematosus-associated lymphadenitis from benign and malignant lymphadenopathies

Gregory D. Scott, Jyoti Kumar, Jean S. Oak, Scott D. Boyd, Philipp W. Raess, Dita A. Gratzinger

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Objectives: Kikuchi-Fujimoto disease (KFD) and systemic lupus erythematosus (SLE) are benign entities with histologic features that raise concern about malignancy and infection. We searched for a histology-independent KFD/SLE signature relying on only immunophenotype and basic clinical characteristics. Methods: A histology-independent KFD/SLE signature was generated using 975 excised lymph nodes with flow immunophenotyping, including 16 cases of KFD/SLE. This signature was then evaluated in 1,198 fine-needle aspiration (FNA) specimens. Results: The top flow cytometry discriminant for KFD/ SLE was uniform CD38+ expression on CD19+ events. Immunohistochemistry demonstrated nodules of IgD+, IgM− B cells surrounding necrotizing and activated T-cell areas. A signature combining 6 flow cytometry criteria with age and sample site had a positive predictive value of 88% for KFD/SLE, which had a prevalence of 1.6%. All 4 signature-positive FNA cases with follow-up excision were KFD/SLE. At a second institution, 4 of 5 KFD/SLE cases passed the top discriminant. Conclusions: A flow cytometry signature combined with age and biopsy site identifies KFD/SLE independent of histology, suggesting a shared immune composition and independently confirming that KFD/SLE represents a distinct entity. Unexpectedly, an IgD+CD38+ small B-cell population is a distinctive feature of KFD/SLE, suggesting a possible pathologic role for anergic/autoreactive B cells.

Original languageEnglish (US)
Pages (from-to)215-224
Number of pages10
JournalAmerican journal of clinical pathology
Volume154
Issue number2
DOIs
StatePublished - Jul 7 2020

Keywords

  • Flow cytometry
  • Histiocytic necrotizing lymphadenitis
  • Kikuchi-Fujimoto disease
  • Lymph node

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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