Extensive arterial and venous thrombosis in a patients with hypereosinophilia and vasculitis

Abstract Hypereosinophilia is part of a group of complex disorders with multisystem involvement. The end organ manifestations are variable and may be related to the pathogenesis of the hypereosinophilia. Disease related morbidity and mortality is often unrelated to the degree of eosinophilia. We p...

Full description

Saved in:
Bibliographic Details
Main Author: Arjun Datt Law
Format: Article
Language:English
Published: PAGEPress Publications 2014-08-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
Subjects:
Online Access:https://mjhid.org/index.php/mjhid/article/view/1913
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Hypereosinophilia is part of a group of complex disorders with multisystem involvement. The end organ manifestations are variable and may be related to the pathogenesis of the hypereosinophilia. Disease related morbidity and mortality is often unrelated to the degree of eosinophilia. We present a case of hypereosinophilia with eosinophilic vasculitis affecting small and medium sized vessels who presented with extensive arterial and venous thrombosis. Case Findings A 23 year old male was admitted to our centre with a history of bilateral popliteal artery thrombosis and impending gangrene of the left forefoot along with DVT of the right lower extremity. He had multiple confluent punched out ulcerated lesions on both legs with gangrenous changes affecting the left foot. Investigations revealed a total white cell count of 40100/?L with an absolute eosinophil count of 27669/?L. Pulmonary CT angiography showed a small sub-segmental pulmonary thromboembolism. A bone marrow examination was performed and showed marked increase in eosinophils & eosinophil precursors. Skin biopsy showed the presence of small vessel vasculitis with intense eosinophil infiltration into the perivascular areas but no granulomas. ANA, P-ANCA and C-ANCA were negative. Polymerase Chain Reaction (PCR) for the FIP1L1-PGDFRA, Bcr-Abl and JAK2V617F mutations were negative. A diagnosis of Hypereosinophilic Syndrome with small (and possibly medium) vessel vasculitis was made. The patient was started on intravenous methylprednisolone (1gm per day for 3 days) followed by oral prednisolone (1mg/kg/day) resulting in a dramatic response. Peripheral blood eosinophilia and skin ulcers resolved after the initiation of the above therapy Conclusion This case report and review of literature brings to light a rare but dangerous complication of hypereosinophilia, particularly in the context of the lesser known entity of eosinophilic vasculitis.
ISSN:2035-3006