Case Report: Kikuchi-Fujimoto disease: a diagnostic and therapeutic dilemma following pretransplant nephrectomy for a 2.35 Kg kidney.

Arvind P Ganpule,Ravindra Sabnis,Abhishek G Singh,Mahesh Desai,Gopal R Tak,Shailesh Soni,Jaspreet Singh Chabra

doi:10.12688/f1000research.8992.1

Arvind P Ganpule, Ravindra Sabnis + Show 5 more

Open Access

https://doi.org/10.12688/f1000research.8992.1

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Journal: F1000Research	Publication Date: Jun 17, 2016
License type: CC BY 4.0

Affiliation: Muljibhai Patel Urological Hospital

Abstract

Kikuchi-Fujimoto disease (KFD) is an extremely rare disease with a worldwide distribution and higher prevalence in Asians. It is a benign and self-limiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Lymph node histopathology is diagnostic and treating physicians should be aware of this entity as it may mimic other systemic diseases like systemic lupus erythematosus, tuberculosis, malignant lymphoma, and more rarely adenocarcinoma. Key features on lymph node biopsy are fragmentation, necrosis and karyorrhexis. Treatment includes symptomatic care, analgesics-antipyretics, corticosteroids and spontaneous recovery occurs in 1 to 4 months. We report a case of adult polycystic kidney disease (ADPKD) with end stage renal disease and episodes of fever and cervical lymphadenopathy. The infectious screen was negative and on extensive workup, the patient was found to have histiocytic-necrotizing lymphadenitis, which clinched the diagnosis of KFD.

Highlights

Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign and self-limiting disease that mainly affects young women
Diagnosis of KFD should be kept in mind in patients who present with fever and cervical lymphadenopathy
We report a case of KFD in a patient with adult polycystic kidney disease who underwent bilateral pre-transplant nephrectomy

Summary

Introduction

Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign and self-limiting disease that mainly affects young women. Recognition of this condition is critical as it can mimic tuberculosis, lymphoma, or even adenocarcinoma. Diagnosis of KFD should be kept in mind in patients who present with fever and cervical lymphadenopathy. This presentation may not be a rarity in immunocompromised pre and post transplant patients. We report a case of KFD in a patient with adult polycystic kidney disease who underwent bilateral pre-transplant nephrectomy

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