Glomerular Complications in Primary Myelofibrosis and the Role of Renal Transplantation: Systematic Review of The Current Evidence.

Despite growing evidence of the prevalence and impact of renal complications in patients with myeloproliferative neoplasms (MPNs), these complications remain underrecognized. At diagnosis, 29% of MPN patients are known to have CKD and many have a high rate of renal failure progression. A distinct entity, MPN-related glomerulopathy, has been described, characterized by mesangial sclerosis, segmental sclerosis, and chronic thrombotic microangiopathy-like changes. Furthermore, an association between Myelofibrosis (MF), a subtype of MPN, and IgA nephropathy has been reported, though limited data exist on its clinical course and management. The present study reports a case of a 49-year-old man diagnosed with myelofibrosis and IgA nephropathy, which led to progressive renal insufficiency and ultimately end-stage renal disease (ESRD). Kidney biopsy showed IgA nephropathy with features of MPN-related glomerulopathy. Due to subsequent progressive impairment in renal function, the patient required and underwent successful renal transplantation with a favorable postoperative outcome. While treatment strategies for MPNs often include JAK2 inhibitors, their role in slowing renal disease progression remains unclear. Furthermore, renal transplantation in MPN patients with ESRD has not been thoroughly studied and should be further investigated, however, this case indicates that kidney transplant is a potential treatment option with appropriate consideration for immunosuppression. In summary, renal dysfunction in patients with MPNs should lead to careful consideration of glomerular involvement, particularly in individuals with unexplained proteinuria or progressive CKD. Kidney biopsy can provide essential diagnostic information, and renal transplantation should be considered as a treatment option in those with ESRD. Future studies are warranted to define the best management of MPN-associated renal disease and post-transplant immunosuppression in this patient population.