Gleich syndrome: a systematic review

Haber, Richard M.; Chebl, Joanna Abi; Gemayel, Maria El; Salloum, Antoine

doi:10.1111/ijd.14963

Cited by 14 publications

(11 citation statements)

References 33 publications

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“…R‐HES was diagnosed in 8% of subjects although a number in the GI and EP groups could have been listed in this section. One patient with R‐HES had lymphocytic‐HES and one had Gleich's syndrome 23,24 . Four patients had a raised IgG4 with one diagnosed as having IgG4 disease on the basis of a liver biopsy 25 .…”

Section: Resultsmentioning

confidence: 99%

The causes of a peripheral blood eosinophilia in a secondary care setting

Wardlaw

Wharin

Aung

et al. 2021

Clin Experimental Allergy

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Background A peripheral blood eosinophilia of greater than 1.0 × 109/L is relatively unusual and offers a clue to the underlying diagnosis. In 2003, we established a specialist service to diagnose unexplained eosinophilia. Objective To describe the causes of an eosinophilia in our service and the diagnostic algorithm we developed. Methods Subjects were referred by physician colleagues across a range of specialties and undertook standard investigations following a semi‐structured protocol. Data were extracted from a bespoke database. Results Three hundred and eighty two subjects were referred over a 17‐year period. Average age was 54 years and 183 (48%) of subjects were female, with 21 of 25 (84%) females in the idiopathic eosinophilic pneumonia group (p < 0001), 22 of 30 (73%) females in the gastrointestinal disease group (p < .008), but 11 of 37 (30%) females in the eosinophilic granulomatosis with polyangiitis group (p < .04). A diagnosis was assigned after systematic evaluation using a pre‐defined algorithm in 361 (94.5%) of cases. Fungal allergy (82 subjects: 21%), parasitic infection (61 subjects: 16%) and severe eosinophilic asthma (50 subjects: 13%) were the three commonest individual diagnoses. Hypereosinophilic syndrome (HES) disease including eosinophilic granulomatosis with polyangiitis (EGPA) accounted for 85 subjects (20%) of which seven subjects (2%) had myeloproliferative disease (M‐HES). A high IgE was common, and 79 (91%) of subjects with complete data who had an IgE of ≥1000 IU/L had fungal allergy or parasite infection. The serum tryptase was raised in 44 of 302 (14.5%) of individuals across all diagnostic groups, though none had mastocytosis. Conclusion A diagnosis of an unexplained eosinophilia can usually be determined using as semi‐structured algorithm. Parasitic infection and fungal allergy often with severe eosinophilic asthma were common causes, whereas HES, particularly myeloproliferative, disease was relatively rare.

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Section: Resultsmentioning

confidence: 99%

The causes of a peripheral blood eosinophilia in a secondary care setting

Wardlaw

Wharin

Aung

et al. 2021

Clin Experimental Allergy

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“…Although skin biopsies are not routinely performed, they usually reveal the presence of a superficial perivascular eosinophilic infiltrate with occasional neutrophils and eosinophil granule protein deposition. 2 Diagnosis is based on clinical and laboratory findings, with a peripheral absolute eosinophil count .1500/mm 3 on at least 2 occasions as the main criteria. 5 An initial evaluation should exclude common causes of hypereosinophilia, and additional workup includes computed tomography scan, bone marrow aspirate and biopsy, and cytogenetic analyses to rule out underlying malignancies.…”

Section: Discussionmentioning

confidence: 99%

Intermittent Soft-Tissue Tumor Enlargement in Episodic Angioedema With Eosinophilia Syndrome

Fernández¹,

Ocampo-Candiani²,

Watts‐Santos³

et al. 2022

The American Journal of Dermatopathology

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:Episodic hypereosinophilia and angioedema syndrome, also known as Gleich syndrome, is a rare entity characterized by recurrent episodes of eosinophilia, angioedema, urticaria, fever and weight gain with spontaneous resolution. It is classified as an idiopathic hypereosinophilic syndrome. Unlike other hypereosinophilic syndromes, it has a low risk for internal organ damage. We report the case of a 42-year-old male with a 28-year history of recurrent erythematous wheals and plaques and persistent hypereosinophilia. Physical examination revealed a well-defined subcutaneous nodule on his right lower limb that increased in size with each episode of angioedema. Histopathology evidenced a lipoma with intense eosinophil infiltration within the mature adipose tissue, while the specimen of the wheal revealed scarce perivascular and interstitial eosinophilic inflammatory infiltrate. Diagnosis of episodic angioedema with eosinophilia syndrome was made based on clinical and laboratory findings.

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“…Gleich syndrome (GS) and eosinophilic fasciitis (EF) are very rare conditions in which eosinophils play an important pathogenetic role 1,2 . We report for the first time a patient with characteristic features of GS as well as EF who responded promptly to mepolizumab.…”

Section: Figurementioning

confidence: 96%

“…Blood smears and bone marrow examination, including chromosome and mutation analyses, excluded hematological malignancies. Parasitoses, fungal infections, hypereosinophilic syndrome, IgG4‐related disease, and Churg‐Strauss syndrome were discussed as differential diagnoses 1–5 . The clinic‐pathological findings were most suggestive for GS/EF overlap.…”

Section: Figurementioning

confidence: 99%