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Unique case of ANCA-negative pauci-immune necrotizing glomerulonephritis with diffuse alveolar hemorrhage, potentially associated with midostaurin

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Abstract

We present a 61-year-old male with FLT3-mutated acute myeloid leukemia treated with midostaurin who developed acute kidney injury requiring hemodialysis and pulmonary renal syndrome. Antibodies to proteinase-3, myeloperoxidase, and glomerular basement membrane were negative. Renal biopsy confirmed acute pauci-immune focal necrotizing glomerulonephritis (GN) with fibrin crescents indicating rapidly progressing glomerulonephritis. He improved with pulse methylprednisolone, intravenous cyclophosphamide, and plasma exchange with resolution of hemoptysis. This case highlights the importance of prompt renal biopsy to guide early initiation of life-saving therapies. To our knowledge, this is the first reported case of ANCA-negative pauci-immune necrotizing GN likely secondary to midostaurin.

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Funding

This case study was not funded.

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Authors and Affiliations

  1. Renal Diseases and Hypertension, McGovern Medical School UT Houston, 6431 Fannin, MSB 5.134, Houston, TX, 77030, USA

    Jonathan D. Pankow & Jaya Kala

  2. Department of Leukemia, MD Anderson Cancer Center, Houston, TX, USA

    Guillaume Richard-Carpentier & Naval G. Daver

  3. Department of Pathology and Laboratory Medicine, McGovern Medical School UT Houston, Houston, TX, USA

    William F. Glass II

Authors
  1. Jonathan D. Pankow
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  2. Guillaume Richard-Carpentier
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  3. Naval G. Daver
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  4. William F. Glass II
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  5. Jaya Kala
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Corresponding author

Correspondence to Jaya Kala.

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Pankow, J.D., Richard-Carpentier, G., Daver, N.G. et al. Unique case of ANCA-negative pauci-immune necrotizing glomerulonephritis with diffuse alveolar hemorrhage, potentially associated with midostaurin. CEN Case Rep 9, 147–151 (2020). https://doi.org/10.1007/s13730-020-00443-3

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  • DOI: https://doi.org/10.1007/s13730-020-00443-3

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