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Focal segmental glomerulosclerosis and mesangial sclerosis associated with myeloproliferative disorders
1Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong.
Abstract:
The myeloproliferative disorders (MPDs) are clonal disorders of the hematopoietic stem cell and classified as polycythemia vera (PV), essential thrombocythemia (ET), or agnogenic myeloid metaplasia (AMM), depending on the main hematopoietic lineage involved. Primary renal parenchymal lesions are not commonly reported in these cases. We conducted a retrospective analysis of 138 consecutive patients with MPD to determine the frequency of renal parenchymal complications. Five patients (3.6%) (two PV, two ET, one AMM) were found to have focal segmental glomerulosclerosis (FSGS) and diffuse mesangial sclerosis, presenting as proteinuria in all the cases and progressing to chronic renal failure in two cases. A possible common risk factor was a high platelet count, because abnormal platelet activation in MPD has been shown to contribute to the development of glomerulosclerosis. The pathophysiologic basis of our observations and the implications in management of MPD patients remain to be studied.
Insights
Myeloproliferative disorders (MPDs) can cause kidney damage, specifically focal segmental glomerulosclerosis (FSGS). High platelet counts may be a contributing factor in MPD patients developing renal complications.
Area of Science:
- Nephrology
- Hematology
- Oncology
Background:
- Myeloproliferative disorders (MPDs) are clonal hematopoietic stem cell neoplasms.
- MPDs are classified as polycythemia vera (PV), essential thrombocythemia (ET), or agnogenic myeloid metaplasia (AMM).
- Primary renal parenchymal lesions are infrequently reported in MPD patients.
Observation:
- A retrospective analysis of 138 MPD patients was conducted.
- Renal parenchymal complications were identified in 3.6% of patients.
- Five patients (2 PV, 2 ET, 1 AMM) were diagnosed with focal segmental glomerulosclerosis (FSGS) and diffuse mesangial sclerosis.
Findings:
- All five patients with FSGS presented with proteinuria.
- Two patients with FSGS progressed to chronic renal failure.
- A high platelet count was identified as a potential common risk factor for developing glomerulosclerosis in MPD.
Implications:
- Abnormal platelet activation in MPD may contribute to glomerulosclerosis development.
- Further research is needed to elucidate the pathophysiologic basis of these renal complications.
- Understanding these mechanisms could inform management strategies for MPD patients with renal involvement.