Eosinophilic polymyositis

Layzer, Robert B.; Shearn, Martin A.; Satya‐Murti, Saty

doi:10.1002/ana.410010106

Cited by 74 publications

(38 citation statements)

References 21 publications

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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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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

View full text Add to dashboard Cite

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“…3 recently described cases of eosinophilic polymyositis (Layzer et al, 1977) had more severe muscular involvement than reported in eosinophilic fasciitis, with pronounced proxi mal weakness and increased values of CPK. These cases had extensive visceral involvement with severe prognosis.…”

Section: Discussionmentioning

confidence: 90%

Polymyositis in Eosinophilic Fasciitis

Bjelle¹,

Henriksson²,

Hofer³

1980

Eur Neurol

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A patient with typical clinical, laboratory and biopsy findings of eosinophilic fasciitis and fulfilling criteria for myositis is reported. Muscular involvement in eosinophilic fasciitis is reviewed and relations to hypereosinophilic syndromes and rheumatoid diseases are discussed. The importance of clinical recognition of eosinophilic fasciitis and histopathological examination of the fascia in the diagnosis of myopathies are underlined.

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“…Layzer et al [8] described three patients with symptoms of polymyositis and eosinophilic infiltration of the muscle tissue. These patients also had systemic manifestations such as heart failure, heart block, Raynaud's phenomenon, subungual splinter haemorrhages, skin rash and anaemia.…”

Section: Discussionmentioning

confidence: 99%

“…In the other patient, who had no history of atopic illness, the eosinophil count ranged between 10 % and 21 %. Layzer et al [8] regarded eosinophilic polymyositis as part of the spectrum of IHES. Stark [9] described a case of eosinophilic polymyositis characterized by tender muscle swelling, creatine kinase elevation, and electromyographic features typical of polymyositis.…”

Section: Discussionmentioning

confidence: 99%

MRI findings of muscle involvement in idiopathic hypereosinophilic syndrome

1999

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A 40-year-old white man presented with fever, muscle pain, skin nodules and persistent hypereosinophilia over a period of 1 year. In addition, he had ventricular arrhythmias with episodes of tachycardia. Besides a lack of response to antiparasitic therapy, laboratory and pathological data excluded the diagnosis of trichinosis or any other parasitic infection. The patient's course of the disease over the previous 1(1)/2 years was compatible with hypereosinophilic syndrome. In a muscle biopsy several eosinophilic perivascular and leucocytic intravascular infiltrates were found, indicative of muscle involvement by the disease. This is a report on the MRI findings of muscle involvement in idiopathic hypereosinophilic syndrome.

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Eosinophilic polymyositis

Cited by 74 publications

References 21 publications

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

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

Polymyositis in Eosinophilic Fasciitis

MRI findings of muscle involvement in idiopathic hypereosinophilic syndrome

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