Novel Detection of CALR-Mutated Cells in Myeloproliferative Neoplasm-Related Glomerulopathy With Interstitial Extramedullary Hematopoiesis: A Case Report

Maruyama, Kouichi; Nakagawa, Naoki; Suzuki, Ayana; Kabara, Maki; Matsuki, Motoki; Shindo, Motohiro; Iwasaki, Shigeo; Ogawa, Yoshihide; Hasebe, Naoyuki

doi:10.1053/j.ajkd.2019.05.016

Cited by 9 publications

(15 citation statements)

References 11 publications

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“…According to these hypotheses, the main glomerular pathological finding in our patients and those previously reported was mesangial sclerosis and hypercellularity 12 . In a subset of patients, glomerular injuries culminate in overt FSGS, as previously described in patients with ET or MPN 30 . Intra-capillary hematopoietic cells were also identified in 23-36% of patients 12 , but we did not observe signs of chronic thombopathic microangiopathy, Even if a significant association has been established between CMML and autoimmunity (both systemic vasculitis 31 and auto-antibodies 32 ), we did not observe neither a high prevalence of extra-renal manifestations nor renal vasculitis.…”

Section: J O U R N a L P R E -P R O O Fmentioning

confidence: 63%

“…The chronic tubulo-interstitiel pattern observed on the kidney biopsy can be attributed to both ET and chronic lesions related to amyloidosis. Secondly, we could not perform exhaustive immunostaining, including CD61 and CALR staining 30 , in all biopsies, but our recent results highlighted the need to better understand how myeloid clonal cells can trigger renal fibrosis.…”

Section: Discussionmentioning

confidence: 99%

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Kidney Involvement in Patients With Chronic Myelomonocytic Leukemia or BCR-ABL–Negative Myeloproliferative Neoplasms

Béllière

Colombat

Kounde

et al. 2021

Kidney International Reports

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Introduction The identification of specific molecular signatures and the development of new targeted drugs have changed the paradigm of onco-nephrology, now allowing a multiscale approach of kidney involvement related to hematologic malignancies relying on combined hematologic and molecular assessments. In this study, we aimed to refine the spectrum of kidney disorders associated with chronic myelomonocytic leukemia (CMML) or BCR-ABL–negative myeloproliferative neoplasms (MPNs), 2 very rare conditions scarcely described. Methods Case series. Patients with myeloid neoplasms who were referred to Toulouse University Hospital Nephrology Unit and were diagnosed with acute kidney injury (AKI), chronic kidney disease (CKD), or urine abnormalities were retrospectively included. Results Eighteen patients (males n =13, CMML n =8, essential thrombocytosis [ET] n =7, polycythemia vera [PV] n =1, and myelofibrosis n =2) developed kidney disease 7.7±2 years after the diagnosis of the malignancy. Twelve patients had AKI at presentation. Eight patients had glomerular presentation (high-range proteinuria 33%, microscopic hematuria 56%). Kidney biopsy ( n =14) showed various patterns, including pauci-immune glomerulosclerosis ( n =5), extramedullary hematopoiesis ( n =6), or tubular atrophy and interstitial fibrosis with polymorphic inflammation ( n =8). Immunostaining of CD61 confirmed the infiltration of megakaryocytes within glomeruli or interstitium in 5 of 8 patients. Other pictures of glomerulopathy were identified in 3 patients (IgA nephropathy n =2, AA amyloidosis n =1). Massive kidney infiltration by CMML was identified in 1 patient. After a mean follow-up of 24±6 months, malignancy was considered as stable in 11 patients (61%), but 22% of patients had progressed to end-stage renal failure. The remaining had persistently reduced kidney function. No correlation between the malignancy and the renal presentation and outcomes could be identified. Conclusions Kidney complications of CMML/MPN are heterogenous, and kidney biopsy may help to identify new molecular targets to prevent the development of kidney fibrosis.

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Section: J O U R N a L P R E -P R O O Fmentioning

confidence: 63%

Section: Discussionmentioning

confidence: 99%

Kidney Involvement in Patients With Chronic Myelomonocytic Leukemia or BCR-ABL–Negative Myeloproliferative Neoplasms

Béllière

Colombat

Kounde

et al. 2021

Kidney International Reports

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“…In addition, in patients with MPN, moderate to severe chronic kidney disease (CKD) is associated with thrombosis, 19,20 disease severity 20 and reduced survival 21 . CKD in MPN may be caused by an underlying glomerulonephritis 22 such as focal segmental glomerulosclerosis, mesangial sclerosis and hypercellularity and renal interstitial extramedullary haematopoiesis 23–25 . Clonal myelopoiesis has just recently been associated with CKD in the UK Biobank using whole exome sequencing but without measurement of CALR mutations 26 .…”

Section: Introductionmentioning

confidence: 99%

Clonal haematopoiesis of indeterminate potential and impaired kidney function—A Danish general population study with 11 years follow‐up

Larsen

Skov

Kjær

et al. 2022

European J of Haematology

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“…Furthermore, renal improvement (>10%) was directly related to failure-free survival in this study (hazard ratio 1.4, 95% CI 1.1–2, p = 0.02) [7]. When other PMF patients were treated with JAK inhibitors, the urinary protein was improved, but complete remission was not achieved [8, 9]. We expected to change dramatically with ruxolitinib administration in this case.…”

Section: Discussionmentioning

confidence: 82%

Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor

Asou

Asakawa

Araki

et al. 2021

Case Rep Nephrol Dial

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Extramedullary hematopoiesis is widely known to occur in patients with primary myelofibrosis (PMF). Autopsy studies on individuals with PMF revealed that extramedullary hematopoiesis occurred in the kidneys in 35% of the cases, but there is little awareness regarding such lesions. A 63-year-old man was diagnosed with PMF based on a detailed examination of persistent high white blood cells. An examination of the patient’s medical records revealed an increased white blood cell count, deterioration of kidney function, and urinary protein excretion developed simultaneously. Thus, a kidney biopsy was performed. Advanced lymphocyte invasion was recognized in the interstitial tissue, and the tubular structure was highly disrupted. Based on these findings, he was diagnosed with interstitial nephritis. However, because of the large number of cells with nuclear atypia in the stroma, additional immunohistochemical staining was also performed, such as glycophorin A, naphthol AS-D, myeloperoxidase, and CD42b. As a result, invasion of three lineages of immature cells, erythroblasts, megakaryocytes, and granulocytes, was identified. Renal dysfunction resulting from interstitial cellular infiltration due to extramedullary hematopoiesis was therefore diagnosed. Treatment with ruxolitinib was initiated after a renal biopsy and the rate of decline in renal function was slightly reduced. Although, in myeloproliferative disorders, proliferative glomerular lesions are widely considered to be renal disorders, there is little awareness regarding interstitial lesions. Extramedullary hematopoiesis of the kidney in PMF is not uncommon, but 40% of cases are reportedly misdiagnosed as interstitial nephritis. Because extramedullary hematopoiesis can be controlled by ruxolitinib, early detection is important.

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Novel Detection of CALR-Mutated Cells in Myeloproliferative Neoplasm-Related Glomerulopathy With Interstitial Extramedullary Hematopoiesis: A Case Report

Cited by 9 publications

References 11 publications

Kidney Involvement in Patients With Chronic Myelomonocytic Leukemia or BCR-ABL–Negative Myeloproliferative Neoplasms

Kidney Involvement in Patients With Chronic Myelomonocytic Leukemia or BCR-ABL–Negative Myeloproliferative Neoplasms

Clonal haematopoiesis of indeterminate potential and impaired kidney function—A Danish general population study with 11 years follow‐up

Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor

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