Two Cases Demonstrate Potential Renal Complications of MDS/MPN

Renal complications are not considered a classical element of myeloproliferative neoplasms (MPN) and myelodysplastic syndrome (MDS), but recent evidence suggests the complication is more common than previously thought.

In a new report published in Cureus, investigators highlighted 2 cases of MDS/MPN in which patients experienced renal complications.

The study authors explained that people with MDS/MPN experience symptoms of both conditions, including partially ineffective hematopoiesis and dysplastic changes. Recent evidence shows that as many as 11% to 29% of people with MPN or MDS/MPN have stage III or IV chronic kidney disease (CKD), and about 1 in 5 have a rapidly declining glomerular filtration rate, they said.

“Glomerular changes associated with MPN have been reported to include glomerular intracapillary hematopoietic cells, focal segmental glomerulosclerosis, and mesangial sclerosis,” they noted.

In the first of the 2 cases outlined in the report, a 45-year-old male with a history of type 2 diabetes and thrombocythemia presented at a nephrology clinic with shortness of breath, a dry cough, and edema of the face and lower limbs. The outpatient neurologist discovered proteinuria and referred the case to the authors.

Laboratory testing showed proteinuria, but the patient tested negative for hepatitis B and C, and immunological tests were negative except for C3 complement fraction. A biopsy was performed and the patient received a diagnosis of mesangiocapillary and mesangioproliferative MPN-associated glomerulopathy.

“The patient was started on corticosteroid treatment, prednisolone pulse infusion 10 mg/kg for 3 days, followed up by 5-mg tablets orally 4-0-2 [4 tablets in the morning, no tables in the afternoon, 2 tables a night], with a reduction of the bedtime dose by 1 tablet every 15 days and eventual reduction of the total dose to 2 daily tablets, as well as referred to the hematology clinic to optimize his hematological treatment, acetylsalicylic acid 500 mg daily,” the authors wrote.

On follow-up, the patient was given cyclophosphamide pulses at 10 mg/kg, but when those were ineffective, he was switched to a daily dose of 1 g of mycophenolate mofetil (CellCept), which sparked a clinical benefit.

Three months later, the patient suffered abdominal trauma and had to undergo an emergency splenectomy. The patient was stabilized and his renal function improved, but he later died following SARS-CoV-2 infection, the investigators reported.

The second patient was a 73-year-old male with lower limb and periorbital edema for the previous 3 months.

“Outpatient nephrology-prescribed laboratory tests revealed hypoproteinemia at 48.3 g/L, serum albumin at 24 g/L, and a significant amount of protein in the urine of 3.18 g/L in the 24-hour sample,” they authors said.

The patient had a history of hypertension and polycythemia vera. He underwent laboratory testing and then a renal biopsy after the tests failed to reveal any clear cause.

“MPN/MDS-associated segmental mesangial proliferative glomerulonephritis was diagnosed based on the morphology, immunological deposits, and patient medical history,” they said.

The patient was given prednisolone pulse infusions at 10 mg/kg for 3 days, followed by 5-mg tablets orally 4-0-2 with a bedtime dose reduction every 15 days. He was referred to a hematologist to re-evaluate his myeloproliferative disorder and put on mycophenolate mofetil on follow-up, at a dose of 200 mg orally/d. Like the first patient, the therapy led to clinical benefits. His medications were later changed to include acetylsalicylic acid at 500 mg daily, and the authors said he has seen steady improvement.

The study investigators said there are a number of potential reasons for the links between MDS/MPN and CKD, and patients experiencing renal complications can present with a diverse range of histological pictures.

“These patients should be monitored for early detection of low-grade proteinuria and microscopic hematuria, risk factors, and late-onset renal involvement of the chronic glomerulonephritis type,” they concluded.

Reference

Popov H, Koleva T, Stoyanov GS, Ghenev P. Myeloproliferative neoplasm and myelodysplastic syndrome-associated renal disease: a histopathological report of two cases. Cureus. Published online December 10, 2022. doi:10.7759/cureus.32388