A 42 years old Asian male on evaluation of abdominal pain was diagnosed with adult polycystic kidney disease (ADPKD) in 2002. The patient's mother died of renal failure due to ADPKD. Patient had progressive deterioration of renal function and developed chronic kidney disease (CKD) stage 5 in 2015. The patient was advised renal replacement therapy (RRT) and required thrice-weekly hemodialysis since September 2015. He was evaluated for renal transplant and planned for the same in May 2016. During pre-transplant evaluation he developed fever with chills and bilateral painful cervical lymphadenopathy in the posterior triangle of neck. Ultrasound of cervical region reported presence of multiple enlarged lymph nodes in the neck along internal jugular vein on both the sides; largest node on right side measured 21x7 mm in size and on the left side largest node measured 16x5 mm in size. Ultrasound examination of axilla and groin did not reveal lymphadenopathy.
The patient underwent right cervical lymph node biopsy. Histopathology was reported to be acute necrotizing lymphadenitis. This biopsy was negative for acid-fast bacilli (AFB) and fungal stains. In the meanwhile his complete infectious screen of blood, urine, sputum and stool cultures showed no growth. The patient was given broad-spectrum third generation cephalosporins for 14 days assuming the fever to be caused by unidentifiable bacterial infection.
The fever settled and the patient was planned for a bilateral pre-transplant nephrectomy in view of bilateral large size kidney and recurrent fever. Right laparoscopic Pre-transplant nephrectomy was done on 05 February 2016. This was followed by a pre-transplant left open nephrectomy on 16 February 2016. The left side was planned for an open nephrectomy in view of the large size of the kidney. The procedure was uneventful. Incidentally, this was one of the largest kidney specimen ever recorded in literature weighing 2.35 Kg ( Figure 1a & 1b). The Guinness Book of World Records reports a kidney weighing 2.15 kg as world's largest kidney till now which was retrieved in Dhule, Maharashtra, India .
Postoperatively, the patient had continuous fever of 100 degree Fahrenheit with chills for 10-15 days; despite he receiving third generation broad-spectrum injectable antibiotics. As the patient had undergone a bilateral nephrectomy, the first diagnosis for pyrexia of unknown origin was postoperative surgical infection or intra-abdominal abscess formation. The imaging done included high-resolution computerized tomography (CT) chest (HRCT chest), CT abdomen and CT pelvis. These did not reveal any significant finding. His fever did not subside despite all the measures such as higher antibiotics, antipyretics and removal of all indwelling catheters. His indwelling catheter lines were cultured to rule out any infection, but did not grow any organism. An infectious disease specialist was consulted and he advised for a slide review of the cervical lymph node sample, which was done prior to nephrectomy.
Slide review with the specialist suggested a necrotizing histiocytic lymphadenitis (Kikuchi Disease) ( Figure 2a & 2b). Section revealed lymphoid architecture being replaced by necrotizing lesions, which are composed of karyorrhectic debris with fibrin deposits and collection of mononuclear cells. Plasma cells and neutrophils were scanty. Periodic acid-Schiff (PAS) stain was negative for fungal hyphae. Ziehl Neelsen (ZN) stain was negative for AFB with final diagnosis as necrotizing histiocytic lymphadenitis. Tests for bacteria/fungus/AFB were negative. Genexpert examination for tubercle bacilli (TB) was also negative. Immmunohistochemistry (IHC) examination for lymphoma was negative. Tests for systemic lupus erythematosus (SLE) were also negative. The key features which clinched the histological diagnosis were: karyorrhexis, fragmentation and necrosis.
The patient was started on non-steroidal anti-inflammatory drugs (NSAID), oral Prednisolone 10 mg twice a day for 15 days and continued on hemodialysis (HD). Patient responded well to this treatment measures. A team of an infection specialist, a nephrologist and a urologist managed this patient. The patient finally became afebrile and fit for renal transplantation after a treatment for 3 weeks. He underwent a living related renal transplant on 27 th of April 2016. His postoperative course after renal transplantation was uneventful and achieved a nadir creatinine of 1.05 mg/dl.