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Glomerular involvement in myelodysplastic syndromes
R Bogdanović1, M Kuzmanović, J Marković-Lipkovski
1Institute of Mother and Child Health of Serbia, 8 R. Dakica St., 11070 Belgrade, Yugoslavia. Maloun@eunet.yu
Pediatric Nephrology (Berlin, Germany)
|January 17, 2002
Summary
This study highlights myelodysplastic syndromes (MDS) as a cause of steroid-responsive nephrotic syndrome (NS) in children, a rare but important association. Early detection and treatment are crucial for managing these pediatric kidney and blood disorders.
Area of Science:
- Pediatric Nephrology
- Hematology
- Oncology
Background:
- Glomerular diseases are documented in adults with myelodysplastic syndromes (MDS).
- Similar reports in pediatric patients are scarce, necessitating further investigation into this association.
- Myelodysplastic syndromes represent a group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis and a high risk of transformation to acute myeloid leukemia.
Observation:
- Two pediatric cases of myelodysplastic syndromes-associated steroid-responsive nephrotic syndrome (NS) are presented.
- Patient 1 exhibited MDS with myelofibrosis, presenting with pancytopenia and nephrotic syndrome, responsive to high-dose steroids but with persistent hematologic issues.
- Patient 2 had refractory anemia with monosomy 19, experiencing nephrotic syndrome and hematologic relapse responsive to corticosteroids, indicating a potential link between MDS and NS.
Findings:
- Renal biopsy findings in both pediatric patients were consistent with idiopathic nephrotic syndrome.
- A literature review identified 16 adult cases of glomerulopathy associated with MDS, manifesting as NS, renal insufficiency, and various glomerulonephritis types.
- The study suggests that myelodysplastic syndromes can be associated with corticosteroid-responsive nephrotic syndrome in children, expanding the known spectrum of MDS complications.
Implications:
- Pediatricians and hematologists should consider searching for glomerular disease in children diagnosed with myelodysplastic syndromes.
- MDS should be recognized as a potential underlying cause of nephrotic syndrome in pediatric patients, particularly those with steroid-responsive disease.
- This finding contributes to understanding the extraskeletal manifestations of myelodysplastic syndromes and informs diagnostic and therapeutic strategies in affected children.