Myeloproliferative Neoplasm and Myelodysplastic Syndrome-Associated Renal Disease: A Histopathological Report of Two Cases

Myeloproliferative neoplasms (MPN) are clonal disorders of hematopoietic stem cells with a proliferation of one or more myeloid lineage and mature cell overproduction, while myelodysplastic syndrome (MDS)/MPN simultaneously show aspects of MDS and MPN, leading to partially ineffective hematopoiesis with associated dysplastic changes. This spectrum of disorders includes chronic myeloid leukemia, polycythemia vera, primary myelofibrosis, and essential thrombocythemia. MDS/MPN are classically not associated with renal complications; however, an accumulating body of evidence suggests that multiple growth factors, cytokines, endothelial damage, and an activated complement system in these patients can induce glomerulopathy, as nearly a third of these patients present with advanced renal disease on diagnosis, which is unlikely to be age or hypertension-related. In this report, we present two cases of patients with MPN/MDS, a 45-year-old male with essential thrombocythemia and a 73-year-old male with polycythemia vera, both of whom developed generalized edema and were referred to our institution from their outpatient nephrologists due to accompanying proteinuria. Renal biopsy of the first patient revealed mesangiocapillary and mesangioproliferative MPN-associated glomerulopathy. In contrast, the second patient was diagnosed with MPN/MDS-associated segmental mesangial proliferative glomerulonephritis and renal vasculature drug toxicity. Both patients were started on treatment - corticosteroid as per consensus.