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Abstract
Myeloproliferative neoplasms such as essential thrombocythemia (ET) have been associated with glomerular disease on rare instances. A case of ET associated with immunoglobulin A nephropathy (IgAN) is described in a 57-year-old man with a history of hypertension. Progressively worsening renal function was noted in the patient along with unexplained mild thrombocytosis. Pathological review of renal biopsy identified IgAN concurrently with newly diagnosed JAK2-mutated ET. The patient was started on aspirin therapy and closely monitored for his renal function. A literature review of the association of ET and renal disease revealed nine cases of ET associated with IgAN, focal segmental glomerulosclerosis, and fibrillary glomerulonephritis. Comparison of the pathological features of the renal biopsies within the cases noted mesangial proliferation as a common finding, which has been described to be potentiated by platelet-derived growth factor (PDGF). This commonality may represent a link between ET and glomerular disease which deserves further attention in future cases. Improved management of such cases depends on the recognition of the combined occurrence of ET and glomerular diseases and uncovering the shared pathogenesis between platelets and glomeruli.
Highlights
Essential thrombocythemia (ET) is a type of myeloproliferative neoplasm (MPN) that results in an increased number of platelets in circulation
Polycythemia vera (PV), a form of MPN that leads to an increased concentration of hemoglobin and hematocrit, is associated with a JAK2V615F mutation that has been seen in approximately 95% of cases
MPN patients who are on cytoreductive therapies with profound renal impairment, which does not recover with dosage decrease, may benefit from renal biopsies to identify a concurrent glomerulonephropathy
Summary
Essential thrombocythemia (ET) is a type of myeloproliferative neoplasm (MPN) that results in an increased number of platelets in circulation. The current 2016 WHO classification for diagnosis of ET requires major criteria of a platelet count equal to or over 450 × 103/L, demonstration of JAK2, MPL, or CALR mutation, bone marrow biopsy with mature megakaryocyte proliferation but without significant production of neutrophils, erythrocytes, or reticulin fibers, and lack of meeting criteria for other myeloproliferative diseases. Polycythemia vera (PV), a form of MPN that leads to an increased concentration of hemoglobin and hematocrit, is associated with a JAK2V615F mutation that has been seen in approximately 95% of cases. Past cohorts studying ET patients found that these mutations (JAK2, CALR, and MPL) are seen in 62-64%, 22-24%, and 4%, respectively [4, 5]. Described here is a case of a 57-year-old man diagnosed with ET that was subsequently diagnosed with IgAN
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