Crescentic glomerulonephritis associated with NK-large granular lymphocytic leukemia: A case report
Zengyuan Luo1, Zaiyu Wang1, Ze Wu2
1Department of Nephrology, Xiangya Hospital, Central South University, Changsha, China.
Insights
This case report details a rare instance of nephrotic syndrome in a patient with Natural Killer Large Granular Lymphocytic Leukemia (NK-LGLL). The patient presented with acute kidney injury and Epstein-Barr virus infection, highlighting a poor prognosis and the need for renal monitoring in NK-LGLL.
Area of Science:
- Hematology
- Nephrology
- Oncology
Background:
- Chronic lymphoproliferative disorder of natural killer cells (now NK-large granular lymphocytic leukemia, NK-LGLL) is a rare, indolent hematological disease.
- Renal involvement in NK-LGLL is exceptionally uncommon.
- This report focuses on a unique case of NK-LGLL presenting with nephrotic syndrome.
Purpose of the Study:
- To report a rare case of nephrotic syndrome in a patient with NK-LGLL.
- To describe the clinical presentation, diagnostic findings, and genetic mutations associated with this rare condition.
- To emphasize the importance of renal function monitoring in NK-LGLL patients.
Main Methods:
- A 54-year-old woman presented with nephrotic syndrome, acute kidney injury, and Epstein-Barr virus infection.
- Diagnosis involved bone marrow examination showing clonal NK cell proliferation and renal biopsy revealing pauci-immune crescentic glomerulonephritis with NK-LGLL infiltration.
- Targeted exome sequencing identified pathogenic N642H mutation in STAT5B, along with RELN and INTS1 mutations.
Main Results:
- The patient exhibited nephrotic syndrome and acute kidney injury secondary to pauci-immune crescentic glomerulonephritis with NK-LGLL infiltration.
- Genetic analysis revealed a pathogenic N642H mutation in STAT5B, suggesting a potential driver mutation.
- Despite the diagnosis, the patient unfortunately died from cerebral hemorrhage before treatment could be initiated.
Conclusions:
- This case highlights a unique presentation of crescentic glomerulonephritis associated with NK-LGLL, characterized by STAT5B mutation, EBV infection, and a poor prognosis.
- The findings suggest that NK-LGLL can present with severe renal manifestations, deviating from its typical indolent course.
- Close monitoring of renal function is crucial for patients diagnosed with NK-LGLL, especially those with atypical presentations or genetic mutations.
Rationale:
Chronic lymphoproliferative disorder of natural killer cells is a rare heterogeneous indolent hematological disease, characterized by persistent clonal increase of mature NK cells with a typical large granular lymphocyte pattern. Chronic lymphoproliferative disorder of natural killer cells was revised to NK-large granular lymphocytic leukemia (NK-LGLL) in 2022 WHO classification. Renal involvement in NK-LGLL is extremely rare. Here, we report a woman diagnosed with NK-LGLL and nephrotic syndrome.
Patient Concerns:
A 54-year-old woman had no obvious symptoms except for persistent peripheral lymphocytosis and neutropenia before kidney involvement. Then she presented with nephrotic syndrome, acute kidney injury and Epstein-Barr virus infection.
Diagnoses:
Bone marrow displayed clonal increase of mature NK cells with a typical large granular lymphocyte pattern. Renal biopsy showed pauci-immune crescentic glomerulonephritis and renal infiltration by NK-LGLL after exclusion of other diseases. Pathogenic N642H mutation of STAT5B was detected by targeted exome sequencing. A319T mutation in RELN and R500W mutation in INTS1 were also identified. Hence, she was diagnosed with crescentic glomerulonephritis associated with NK-LGLL.
Interventions And Outcome:
We planned to combine methylprednisolone and cyclophosphamide in the treatment of this case. Unfortunately, our patient died of severe cerebral hemorrhage shortly after the diagnosis of NK-LGLL. We had no opportunity to use immunosuppressive drugs for therapy.
Lessons:
In short, we report a unique case diagnosed with crescentic glomerulonephritis associated with NK-LGLL, with pathogenic N642H mutation in STAT5B, Epstein-Barr virus infection and poor prognosis, different from typical inert type. Close monitoring of renal function is suggested for similar NK-LGLL patients.
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