Diagnosis and treatment of secondary nephrotic syndrome with rash as the first symptom: a case report
Bowen Qin1, Yueqiang Li2, Dong Kuang3
1Department of Nephrology, Tianyou Hospital, Wuhan University of Science and Technology, Wuhan, China.
Background:
Membranous nephropathy (MN) is a common type of nephrotic syndrome (NS) in adults, accounting for about 20-30% of cases. Although secondary to specific factors, the coexistence of MN and mantle cell lymphoma (MCL) has been scarcely reported in clinical literature.
Case Presentation:
A 59-year-old Chinese male was admitted to the hospital with a generalized pruritic rash with bilateral lower extremity edema, which did not improve significantly after symptomatic treatment. He had undergone renal biopsy, and the diagnosis was thought to be secondary MN (SMN), therefore, we did a lymph node biopsy on the patient and found that MN was complicated with MCL. Soon after, the patient was admitted to the hematology department for a BR chemotherapy regimen (composed of bendamustine 90 mg/m2 BSA (body surface area), rituximab 375 mg/m2 BSA and dexamethasone 5 mg), and during the post-treatment follow-up, both his symptoms and renal function improved.
Conclusions:
The mechanism underlying the combination of SMN and MCL remains elusive and exceedingly rare, consequently often overlooked in clinical practice. This case serves to offer valuable clinical insights for diagnosis and treatment, while emphasizing the pivotal role of renal pathology in clinical assessment.
Insights
This case highlights the rare co-occurrence of secondary membranous nephropathy (SMN) and mantle cell lymphoma (MCL). Early diagnosis and treatment, guided by renal pathology, are crucial for improving patient outcomes in this complex condition.
Area of Science:
- Nephrology
- Hematology
- Oncology
Background:
- Membranous nephropathy (MN) is a frequent cause of nephrotic syndrome in adults.
- The co-occurrence of MN and mantle cell lymphoma (MCL) is exceptionally rare in clinical literature.
Observation:
- A 59-year-old male presented with refractory edema and rash, initially diagnosed as secondary MN (SMN).
- Subsequent lymph node biopsy revealed an underlying mantle cell lymphoma (MCL) complicating the SMN.
Findings:
- The patient received a BR chemotherapy regimen (bendamustine, rituximab, dexamethasone).
- Following treatment, significant improvement in both clinical symptoms and renal function was observed.
Implications:
- This case underscores the importance of considering hematological malignancies in cases of unexplained SMN.
- Renal pathology plays a critical role in the accurate diagnosis and management of coexisting SMN and MCL.
- The underlying mechanisms linking SMN and MCL warrant further investigation due to their rarity and clinical significance.
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