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

Jonathan D Pankow1, Guillaume Richard-Carpentier2, Naval G Daver2

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

CEN Case Reports
|January 20, 2020
PubMed

Insights

This case study describes a patient with FLT3-mutated acute myeloid leukemia who developed ANCA-negative pauci-immune necrotizing glomerulonephritis likely due to midostaurin treatment. Prompt diagnosis and treatment led to patient improvement.

Area of Science:

  • Nephrology
  • Hematology
  • Oncology

Background:

  • Midostaurin is a targeted therapy for FLT3-mutated acute myeloid leukemia (AML).
  • Acute kidney injury (AKI) and pulmonary renal syndrome are potential complications in AML patients.
  • ANCA-associated vasculitis typically presents with pauci-immune glomerulonephritis.

Observation:

  • A 61-year-old male with FLT3-mutated AML treated with midostaurin developed AKI and pulmonary renal syndrome.
  • Serological tests for anti-proteinase-3, anti-myeloperoxidase, and anti-GBM antibodies were negative.
  • Renal biopsy revealed acute, pauci-immune, focal necrotizing glomerulonephritis (GN) with fibrin crescents.

Findings:

  • The patient presented with ANCA-negative pauci-immune necrotizing GN, a condition not previously associated with midostaurin.
  • Treatment with pulse methylprednisolone, intravenous cyclophosphamide, and plasma exchange resulted in resolution of symptoms.
  • This suggests a potential drug-induced nephrotoxicity secondary to midostaurin.

Implications:

  • This case underscores the necessity of early renal biopsy in patients with unexplained AKI during midostaurin therapy.
  • Prompt diagnosis and aggressive immunosuppressive therapy can lead to favorable outcomes in ANCA-negative pauci-immune GN.
  • Further research is warranted to elucidate the mechanism of midostaurin-induced nephrotoxicity.
Keywords:
ANCA negativeMidostaurinPauci-immune glomerulonephritisPulmonary renal syndrome

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