Renal thrombotic microangiopathy and FIP1L1/PDGFRα-associated myeloproliferative variant of hypereosinophilic syndrome

Langlois, Anne Lyse; Shehwaro, Nathalie; Rondet, Claire; Benbrik, Youssef; Maloum, Karim; Gueutin, Victor; Rouvier, Philippe; Izzedine, Hassane

doi:10.1093/ckj/sft067

Cited by 3 publications

(4 citation statements)

References 15 publications

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“…The pathogenesis of HES is mediated by “piecemeal degranulation” or eosinophil activation and secretion of the granule cationic proteins (such as eosinophil peroxidase, eosinophil cationic protein, eosinophil - derived neurotoxin, and MBP1) and eosinophil-expressed cytokines (such as RANTES [regulated on activation, normal T expressed and secreted] and interleukin [4, 9]). Eosinophil granule cationic proteins have the capability to activate inflammatory cells such as mast cells to induce inflammatory mediators and direct tissue-damaging cytotoxicity.…”

Section: Discussionmentioning

confidence: 99%

“…Renal involvement in HES varies from 7% to 36%; however, kidney injury mediated by thrombotic microangiopathy (TMA) is rare [2]. To the best of our knowledge, only two cases of idiopathic HES [3] and one case of myeloproliferative-variant HES [4] have been reported. None of the reported cases achieved normal kidney function after treatment.…”

Section: Introductionmentioning

confidence: 99%

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Acute kidney injury secondary to thrombotic microangiopathy associated with idiopathic hypereosinophilic syndrome: a case report and review of the literature

et al. 2019

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Background Renal involvement in idiopathic hypereosinophilic syndrome is uncommon. The mechanism of kidney damage can be explained as occurring via two distinct pathways: (1) thromboembolic ischemic changes secondary to endocardial disruption mediated by eosinophilic cytotoxicity to the myocardium and (2) direct eosinophilic cytotoxic effect to the kidney. Case presentation We present a case of a 63-year-old Caucasian man who presented to our hospital with 2 weeks of progressively generalized weakness. He was diagnosed with idiopathic hypereosinophilic syndrome with multiorgan involvement and acute kidney injury with biopsy-proven thrombotic microangiopathy. Full remission was achieved after 8 weeks of corticosteroid therapy. Conclusion Further studies are needed to investigate if age and absence of frank thrombocytopenia can serve as a prognostic feature of idiopathic hypereosinophilic syndrome, as seen in this case.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Acute kidney injury secondary to thrombotic microangiopathy associated with idiopathic hypereosinophilic syndrome: a case report and review of the literature

et al. 2019

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“…Low evidence exists for an association of PBE and thrombotic microangiopathies (TMA). Eight reported cases of TMA with PBE were diagnosed as HES [17,102,103,104,105,106]. Recently, one case of atypical HUS with AIN and PBE was reported and the first two case reports of concomitant atypical HUS and EGPA were published [107,108].…”

Section: Eosinophilia In Kidney Diseasementioning

confidence: 99%

Eosinophilia and Kidney Disease: More than Just an Incidental Finding?

Gauckler

Smıth

Mayer

et al. 2018

JCM

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Peripheral blood eosinophilia (PBE), defined as 500 eosinophils or above per microliter (µL) blood, is a condition that is not uncommon but often neglected in the management of patients with chronic kidney disease (CKD), acute kidney injury (AKI), or patients on renal replacement therapy (RRT). The nature of PBE in the context of kidney diseases is predominantly secondary or reactive and has to be distinguished from primary eosinophilic disorders. Nonetheless, the finding of persistent PBE can be a useful clue for the differential diagnosis of underdiagnosed entities and overlapping syndromes, such as eosinophilic granulomatosis with polyangiitis (EGPA), IgG4-related disease (IgG4-RD), acute interstitial nephritis (AIN), or the hypereosinophilic syndrome (HES). For patients on RRT, PBE may be an indicator for bio-incompatibility of the dialysis material, acute allograft rejection, or Strongyloides hyperinfection. In a subset of patients with EGPA, eosinophils might even be the driving force in disease pathogenesis. This improved understanding is already being used to facilitate novel therapeutic options. Mepolizumab has been licensed for the management of EGPA and is applied with the aim to abrogate the underlying immunologic process by blocking interleukin-5. The current article provides an overview of different renal pathologies that are associated with PBE. Further scientific effort is required to understand the exact role and function of eosinophils in these disorders which may pave the way to improved interdisciplinary management of such patients.

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“…Histopathologic patterns described were as follows: eosinophilic interstitial nephritis, membranous nephropathy (MN), crescent glomerulonephritis (CGN), focal segmental glomerulosclerosis (FSGN), immunotactoid glomerulonephritis (IGN), and renal thrombotic microangiopathy (TMA) [ 6 , 7 ]. Two possible pathophysiological mechanisms of renal involvement in IHES are considered, one of them is ischemic renal damage secondary to mural thrombus of cardiac origin, mediated by eosinophilic cytotoxicity and leading to a state of pro-coagulation and vascular dysfunction (mainly the cases of TMA), and the other is by a direct eosinophilic cytotoxic effect due to eosinophilic mass cell infiltrates [ 7 , 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

An Unusual Case of Immune Complex-Mediated Membranoproliferative Glomerulonephritis as Renal Manifestation of Idiopathic Hypereosinophilic Syndrome: A Case Report and Literature Review

Cieza-Terrones,

De La Flor,

Requejo

et al. 2024

Medicines

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Background: Idiopathic hypereosinophilic syndrome (IHES) is a disorder characterized by abnormal and persistent peripheral blood hypereosinophilia (eosinophil count ≥ 1.5× 109/L and ≥10% eosinophils) with duration ≥ 6 months, associated organ damage, and/or dysfunction attributable to tissue eosinophilic infiltrate of unknown cause. IHES affects different organs such as the heart, lungs, nervous system, and skin, with renal involvement being rare in this condition. Case Presentation: We present a case of a young patient with IHES and immune complex-mediated membranoproliferative glomerulonephritis with nephrotic syndrome, as a rare renal manifestation. We discuss the clinical, analytical, and histopathologic renal and hematologic features, comparing them with other reported cases in the literature.

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Renal thrombotic microangiopathy and FIP1L1/PDGFRα-associated myeloproliferative variant of hypereosinophilic syndrome

Cited by 3 publications

References 15 publications

Acute kidney injury secondary to thrombotic microangiopathy associated with idiopathic hypereosinophilic syndrome: a case report and review of the literature

Acute kidney injury secondary to thrombotic microangiopathy associated with idiopathic hypereosinophilic syndrome: a case report and review of the literature

Eosinophilia and Kidney Disease: More than Just an Incidental Finding?

An Unusual Case of Immune Complex-Mediated Membranoproliferative Glomerulonephritis as Renal Manifestation of Idiopathic Hypereosinophilic Syndrome: A Case Report and Literature Review

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