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Abstract: TH-PO077

Interesting Biopsy of a Patient with AKI on a Background of Systemic Mastocytosis

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials


  • O'Brien, Sorcha, St James's Hospital, Dublin, Ireland
  • Griffin, Brenda, St James's Hospital, Dublin, Ireland
  • Dorman, Anthony M., Beaumont Hospital, Dublin, Ireland

Systemic mastocytosis (SM) is a rare disorder characterised by infiltration of clonally derived mast cells in bone marrow and other extracutaneous organs. The most common genetic mutation in SM is KIT D816 V and is resistant to Imatinib, other KIT mutations have been identified. F522C accounts for 1% of all SM-associated KIT mutations and has been reported to be sensitive to Imatinib.

Case Description

A 72-year-old woman was diagnosed with systemic mastocytosis (KIT F522C mutation) following bone marrow biopsy showing mast cell infiltrate and stained positive for CD117. She was commenced on antihistamines and Montelukast. Following the results of the bone marrow sample showing F522C mutation, she was commenced on Imatinib. She initially had a good response to treatment, however she subsequently lost response to Imatinib and required frequent courses of tapering steroids.
Two years after diagnosis, she presented to emergency department (ED) with an acute history of facial flushing and oliguria. Her bloods on presentation to ED showed elevated creatinine and tryptase levels. She initially required renal replacement therapy for several days, renal function then recovered. A renal biopsy was arranged. The biopsy showed acute tubular necrosis (ATN) and inflammatory cell infiltration of the interstitium. Immunohistochemical staining showed focal aggregates of CD117 positive cells indicating an infiltrate of mast cells (~10%).
Following discussion with the patient, she opted not for further management. She became oliguric and developed disseminated intervascular coagulation without evidence of bleeding. She was transferred to a hospice for where she passed away.


Renal involvement in SM is rare and has not been described much in the literature. Consideration needs to be given to other causes of AKI in SM, eg. ATN or acute interstitial nephritis secondary to Imatinib. Renal mastocytosis is typically progressive and leads to chronic kidney disease/end stage kidney disease despite treatment.

Renal cortex with CD117 positive mast cells