Immunotactoid Glomerulopathy Associated with Idiopathic Hypereosinophilic Syndrome

Choi, Yeong-Jin; Lee, Jeong Deuk; Yang, Ki Hwa; Woo, Je Young; Kim, Byung Kee; Bang, Byung Kee; Shim, Sang In

doi:10.1159/000013362

Cited by 23 publications

(15 citation statements)

References 13 publications

(33 reference statements)

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“…However, it is thought to be similar to histologic involvement by HES on other organs by mass effect due to the proportion of eosinophils and eosinophil cytotoxicity (2). In fact, these reports are characterized by eosinophilic infiltration in renal biopsy, which does not occur in our patient (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). While focal segmental glomerulosclerosis and HES may be two coexisting diseases-yet unrelated in our patient-the low proportion of cases reported in the literature of glomerular involvement of HES, compels us to consider the possibility of physiopathological mechanisms not yet known may account for such coexistence.…”

Section: Discussionsupporting

confidence: 51%

“…Kidney involvement is related to vascular disorder, interstitial nephritis, electrolyte disturbances, CharcotLeyden crystals or glomerular diseases (2). Few cases of HES with glomerular lesions have been reported in medical literature (4)(5)(6)(7)(8)(9)(10)(11). We describe a case of HES with nephrotic syndrome secondary to focal segmental glomerulosclerosis.…”

Section: Introductionmentioning

confidence: 98%

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Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome

Herrera-Añazco¹,

Matias-tasayco

Arellano-Bravo

et al. 2017

J Nephropathol

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Background: Renal complication in hypereosinophilic syndrome (HES) is rare, with literature scarcely reporting association of this syndrome with glomerular involvement. While the direct effect of eosinophilic infiltration in tissues has been linked to histological damage of the HES, other mechanisms may account for renal involvement too. Case Presentation: We present a case of a 17-year-old male patient, with progressive edema, contact reactive erythematous skin lesions, acute kidney injury, nephrotic syndrome and progressive eosinophilia. His bone marrow biopsy revealed moderate hyperplasia with severe eosinophilia and atypical lymphocytes. His renal biopsy revealed glomeruli, enlarged in volume with mesangial expansion and hypercellularity and segmental thickening of capillary loops. Likewise, some glomeruli showed peripheral hyalinosis with synechiae to Bowman's capsule. Tubules showed cloudy swelling, mild tubular atrophy and hyaline cylinders. Interstitial area showed infiltrated lymphomononuclear cells, focal with no evidence of eosinophils. Blood vessels were unaltered. Immunofluorescence identified glomeruli with granular mesangial IgM deposition. After corticosteroid treatment, eosinophilia and creatinine values regress to normal range. Conclusions: While our case may suggest the coexistence of two unrelated diseases, further studies are required to assess the pathophysiology of glomerular involvement in HES. Given the possibility that mechanisms other than the direct effect of eosinophils are involved in certain patients

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Section: Discussionsupporting

confidence: 51%

Section: Introductionmentioning

confidence: 98%

Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome

Herrera-Añazco¹,

Matias-tasayco

Arellano-Bravo

et al. 2017

J Nephropathol

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“…The best-characterized examples in the FrenchAmerican-British classification of AML are M4Eo inv (16) Musculoskeletal N/A Arthritis, 79,80 effusions, 80 bursitis, 81 synovitis, 82 Raynaud phenomena, 83 digital necrosis, 84 polymyositis/myopathy 85,86 Renal N/A Acute renal failure with Charcot-Leyden crystalluria, 87 nephrotic syndrome, 88 immunotactoid glomerulopathy, 89 crescentic glomerulonephritis 90 Modified from Weller and Bubley 21 and from Brito-Babapulle 17 with permission from Elsevier. N/A indicates not available.…”

Section: Cytogenetic and Molecular Features Of Hematologic Malignancimentioning

confidence: 99%

The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management

Gotlib

Cools

Malone

et al. 2004

Blood

262

205

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Idiopathic hypereosinophilic syndrome (HES) and chronic eosinophilic leukemia (CEL) comprise a spectrum of indolent to aggressive diseases characterized by unexplained, persistent hypereosinophilia. These disorders have eluded a unique molecular explanation, and therapy has primarily been oriented toward palliation of symptoms related to organ involvement. Recent reports indicate that HES and CEL are imatinib-responsive malignancies, with rapid and complete hematologic remissions observed at lower doses than used in chronic myelogenous leukemia (CML). These BCR-ABL-negative cases lack activating mutations or abnormal fusions involving other known target genes of imatinib, implicating a novel tyrosine kinase in their pathogenesis. A bedside-to-benchtop translational research effort led to the identification of a constitutively activated fusion tyrosine kinase on chromosome 4q12, derived from an interstitial deletion, that fuses the platelet-derived growth factor receptor-␣ gene (PDGFRA) to an uncharacterized human gene FIP1-like-1 (FIP1L1). However, not all HES and CEL patients respond to imatinib, suggesting disease heterogeneity. Furthermore, approximately 40% of responding patients lack the FIP1L1-PDGFRA fusion, suggesting genetic heterogeneity. This review examines the current state of knowledge of HES and CEL and the implications of the FIP1L1-PDGFRA discovery on their diagnosis, classification, and management. IntroductionProtean biologic and clinical presentations characterize idiopathic hypereosinophilia (HES). HES is similar to other diseases given the moniker "diagnosis of exclusion," in that limited understanding of the pathogenesis of the disease has hampered therapeutic advances. The demonstration of increased myeloblasts or clonality or the development of either granulocytic sarcoma or acute myeloid leukemia helps clarify the origin of some cases of chronic eosinophilic leukemia. 1 In a subset of patients, hypereosinophilia is related to excessive secretion of eosinophilopoietic cytokines from a clonal population of lymphocytes. 2 The identification of FIP1-like-1-platelet-derived growth factor receptor-␣ (FIP1L1-PDGFRA) in cases of HES/CEL adds to a growing list of activated fusion tyrosine kinases linked to the pathogenesis of chronic myeloproliferative disorders. 3 It is unique, however, because it is the first description of a gain-of-function fusion protein resulting from a cryptic interstitial deletion between genes rather than a reciprocal chromosomal translocation. The FIP1L1-PDGFR␣ fusion protein transforms hematopoietic cells, and its kinase activity is inhibited by imatinib at a cellular 50% inhibitory concentration (IC 50 ) 100-fold lower than BCR-ABL. 3 Acquisition of an imatinib resistance mutation in the adenosine triphosphate (ATP)-binding domain of PDGFRA in a relapsed patient previously responsive to imatinib supports a critical role for FIP1L1-PDGFR␣ in the pathogenesis of disease and demonstrates that FIP1L1-PDGFR␣ is the therapeutic target of imatinib. 3 The identification of t...

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“…There is not much literature on renal histological pattern in IHES [12]. Various histological patterns reported by various authors include Ischemic lesions secondary to intrarenal thrombus formation [13], crescentic glomerulonephritis [14], mesangial expansion [15], proliferative glomerulonephritis [13], immuno-tactoid glomerulopath [11], glomerular sclerosis [6], interstitial nephritis with eosinophilic infil-trates [8,10] and membranous nephropathy [11].…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Hypereosinophilic Syndrome Presenting as IgA Nephropathy with Nephrotic Range Proteinuria

Shah¹,

Koul²,

Shah³

et al. 2013

OJNeph

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Idiopathic hypereosinophilic syndrome (IHES) is a disorder characterized by increased eosinophil count (eosinophilia) along with organ dysfunction secondary to organ infiltration of eosinophils and release of inflammatory markers [1][2][3][4], with no obvious cause for eosinophilia. The onset of symptoms is insidious in most of the cases and eosinophilia is detected incidentally. However, in others, the initial manifestations are severe and life-threatening due to the rapid evolution of cardiac or neurologic complications [5]. Renal involvement is rarely reported [6] in IHES. Herein we reported a case of IHES with predominant renal involvement as nephrotic syndrome with focal necrotizing IgA nephropathy.Keywords: Idiopathic Hypereosinophilic Syndrome; Eosinophilia; Renal Involvement; Nephrotic Syndrome Case ReportA 50-year old male presented with a 3-week history of a non-productive cough, associated with intermittent low grade fever, periorbital puffiness and lower extremity edema. Patient denied any history of breathlessness, chest-pain, hemoptysis, orthopnea, paroxysmal nocturnal dyspnea, drop in urine output, skin rash or arthritis. His past history was insignificant and he denied any history of drug intake.Hisexamination revealed mild pallor, with periorbitalpuffiness, lower extremity pitting edema and mild splenomegaly. He had hemoglobin of 11 g/dL with a total leucocyte count of 9.2 × 10 9 /cumm and an ESR of 23 mmhr. His differential count included 46% polymorphs 11% lymphocytes and 41% eosinophils with AEC count of 3800 which repeatedly was high and ranged from 3800 to 5500. A peripheral blood film showed features of normochromic normocytic anaemia with no abnormal cells. His serum creatinine was 1.7 mg/dL with a creatinine clearance of 44 ml/min. Liver functions showed hypoporoteinemia with hypoalbuminemia. He had an active urinary sedimentand urine which showed presence of eosinophils on wright's staining. 24 hours urinary protein was 5.04 g/24hours which was reproduced twice. Repeated stool examination for the presence of ova/parasite was negative.Radiograph of the chest was normal. High resolution computerized tomography of the chest did not reveal any abnormality. His electrocardiograph and echocardiography were normal. Ultrasonography of abdomen was normal as well.Patient was investigated further to look for eosinophilic infiltration of other organ systems in view of persistant hypereosinophilia.Patient was subjected to bronchoscopy to obtain bronchoalveolar lavage for eosinophils which revealed 62% eosinophils. Bone marrow aspiration and biopsy revealed 56% eosinophils with no dysplasia and blasts. p-ANCA andc-ANCA were within normal limits. Serum tryptase levels were within normal limits.Patient was subjected to percutaneous renal biopsy to look for the etiology of nephrotic range proteinuria. Renal biopsy revealed, marked interstitial edema and patchy inflammation including aggregates of eosinophils admixed with mononuclear cells and occasional neutrophils. An eosinophilic infiltrate wa...

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Immunotactoid Glomerulopathy Associated with Idiopathic Hypereosinophilic Syndrome

Cited by 23 publications

References 13 publications

Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome

Focal segmental glomerulosclerosis in a patient with hypereosinophilic syndrome

The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management

Idiopathic Hypereosinophilic Syndrome Presenting as IgA Nephropathy with Nephrotic Range Proteinuria

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