Chronic kidney disease in patients with the Philadelphia-negative chronic myeloproliferative neoplasms
Introduction
The Philadelphia-negative myeloproliferative neoplasms (MPN) consist of essential thrombocythemia (ET), polycythemia vera (PV) and myelofibrosis (MF). MPN are clonal hematopoietic stem cell derived malignancies arising due to acquired somatic mutations in signal transduction pathways [1].
The major complications associated with MPN disease are increased risk of thrombosis and haemorrhage [2]. Additionally, several case studies have described histologically verified glomerulopathy and nephrotic syndrome in MPN [3], [4], [5], [6] and a retrospective study of 138 MPN has found glomerulonephritis in 3.6% [7]. Most recently, “MPN-related glomerulopathy” has been proposed as a new histopathological diagnostic entity [3].
However, the frequency of the most common kidney disease, chronic kidney disease (CKD), has not been evaluated in MPN patients. CKD is mostly an asymptomatic disease state characterized by low estimated glomerular filtration rate (eGFR) and is associated with increased cardiovascular and overall mortality [8]. Hypothetically, the elevated cell counts (leukocytes, thrombocytes and erythrocytes) and the high levels of inflammatory cytokines shown to exist in MPN patients [9], [10], [11] might affect glomerular endothelium. The aim of this retrospective study is to evaluate the frequency of CKD and the progression patterns of kidney function in a large cohort of MPN patients.
Section snippets
Study design
One hundred forty three subjects were identified by entries in the Roskilde Hospital Pathology Bank from 2003 to 2010 with the histopathological diagnosis of MPN based on bone marrow biopsies and blood smears. Criteria of MPN diagnosis was based on the 2008 revised WHO criteria of MPN [12]. For patients diagnosed before 2008, the revised WHO criteria was applied if possible, otherwise the 2001 WHO criteria for PV, MF and ET were used [13]. Patients were given the diagnosis of ET, PV, MF or
Results
The cohort characteristics and comorbidities are presented in Table 1. An overview of eGFR in the whole cohort of patients at diagnosis and in diagnostic subgroups is presented in Table 2. At time of diagnosis 29% of patients had an eGFR < 60 mL/min/1.73 m2 and per definition had CKD. 27% had CKD stage 3 (eGFR 30–59 mL/min/1.73 m2) and 2% CKD stage 4 (eGFR 15–29 mL/min/1.73 m2). The frequency of CKD (eGFR < 60 mL/min/1.73 m2) was similar in all diagnostic group with 31%, 29%, 27% and 27% in MF, ET, PV and
Discussion
This study is the first to describe progression patterns in renal function over time in patients with MPN. In our study of a large cohort of MPN patients from a single institution the frequency of CKD at diagnosis was 29% with 27% having CKD stage 3 and 2% having CKD stage 4. The frequency of CKD in the different diagnostic groups of MPN disease was similar ranging from 27% to 31%. As expected, CKD was primarily recorded in elderly patients. Thus 15%, 36% and 38% of patients ≤59, 60–69 and ≥70
Acknowledgements
Role of funding source: This study had no sponsors.
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