Renal involvement in primary myelofibrosis (PMF) is rare. Clinically, glomerular disease associated with PMF and other forms of myeloproliferative neoplasm (MPN) is characterized by renal insufficiency and high proteinuria. Few studies have observed extramedullary hematopoiesis (EMH) and glomerulopathy associated with PMF. In this article, we report a rare case of PMF-related glomerulopathy with renal EMH in a patient who presented with renal dysfunction and heavy proteinuria.
TefferiA.Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management. Am J Hematol2021; 96(1): 145–162.
2.
SordoRDBrugnanoRCovarelliC, et alNephrotic syndrome in primary myelofibrosis with renal extramedullary hematopoiesis and glomerulopathy in the JAK inhibitor era. Clin Nephrol2017; 5: 70.
3.
RajasekaranANgoTTAbdelrahimM, et alPrimary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib. BMC Nephrol2015; 16(1): 121.
4.
AlexanderMPNasrSHKurtinPJ, et alRenal extramedullary hematopoiesis: interstitial and glomerular pathology. Mod Pathol2015; 28(12): 1574–1583.
5.
SaidSMLeungNSethiS, et alMyeloproliferative neoplasms cause glomerulopathy. Kidney Int2011; 80(7): 753–759.
6.
HwangJWDesterkeCLoisel-DuwattezJ, et alDetection of hematopoietic stem cell transcriptome in human fetal kidneys and kidney organoids derived from human induced pluripotent stem cells. Front Cell Dev Biol2021; 9: 668833.
7.
Büttner-HeroldMStichtCWiechT, et alRenal disease associated with myeloproliferative neoplasms and myelodysplastic syndrome/myeloproliferative neoplasms. Histopathology2021; 78(5): 738–748.
8.
HasselbalchHC.The role of cytokines in the initiation and progression of myelofibrosis. Cytokine Growth Factor Rev2013; 24(2): 133–145.
9.
KimHJJungMLimBJ, et alPrimary myelofibrosis-related glomerulopathy with renal extramedullary hematopoiesis. J Nephrol2023; 36(8): 2387–2390.
10.
OstojicAVrhovacRVerstovsekS.Ruxolitinib: a new JAK1/2 inhibitor that offers promising options for treatment of myelofibrosis. Future Oncol2011; 7(9): 1035–1043.
11.
StratiPAbdelrahimMSelametU, et alRuxolitinib therapy is associated with improved renal function in patients with primary myelofibrosis. Ann Hematol2019; 98(7): 1611–1616.
