You are using an outdated browser. Please upgrade your browser to improve your experience.

You are using an outdated browser.

To ensure optimal security, this website will soon be unavailable on this browser. Please upgrade your browser to allow continued use of ACP websites.

You are here

Winning Abstracts from the 2014 Medical Student Abstract Competition: Extensive Arterial and Venous Thrombi as a Presentation of Hypereosinophilic Syndrome with a Unique Complication of Hemolytic Anemia and Poor Response to Treatment

Winning Abstracts from the 2014 Medical Student Abstract Competition: Extensive Arterial and Venous Thrombi as a Presentation of Hypereosinophilic Syndrome with a Unique Complication of Hemolytic Anemia and Poor Response to Treatment

First Authors:
Matthew P Deek* Robert Wood Johnson Medical School, Class of 2015
Mansi R. Shah, MD* - Robert Wood Johnson Medical School, Class of 2014
*Equal contribution

Idiopathic hypereosinophilic syndrome (HES) is defined as persistent eosinophilia with end organ damage in the absence of a neoplastic process or reactive eosinophilia. Major organ damage can occur due to eosinophil infiltration, which may manifest as fibrosis, thrombosis with or without thromboembolism, cutaneous or mucosal involvement, edema, and neurologic deficits. Amongst idiopathic HES is a lymphocytic variant caused by an aberrant T cell lymphocyte population that overproduces the cytokine interleukin-5. Some patients with the lymphocytic variant HES may eventually develop T-cell lymphoma.

A previously healthy 46 year old man, who recently emigrated from Dominican Republic with a diagnosis of bilateral lower extremity DVT on warfarin therapy, presented with progressively worsening bilateral lower extremity pain, cyanosis of his right foot, and 18lb unintentional weight loss over three weeks. On exam, the patient was tachycardic with bilateral lower extremity edema, had dusky discoloration of the digits of hands and feet with weak peripheral pulses. No rash, lymphadenopathy, respiratory wheezes, masses or organomegaly were present. Initial diagnostic tests revealed severe eosinophilia (WBC, 30.4x103; eosinophils 20.7x103) and anemia (Hgb 9.6g/dL). Results of vascular studies confirmed with CT showed extensive arterial thrombi of the right upper, left upper, and right lower extremity and venous thrombi of the IVC, right peroneal, right posterior tibial, right popliteal, right femoral, right external iliac, left popliteal, left femoral, left external iliac, and hepatic vein.

An extensive investigation was pursued and ruled out infectious etiologies including parasites, HIV, and hepatitis. His hospital course was further complicated by the development of hemolytic anemia for which he was treated with IVIG and required transfusion support. Subsequent hematological work up including bone marrow biopsy revealed a monoclonal T-cell population, and the patient was diagnosed with hypereosinophilic syndrome with a clonal T-cell mediated lymphoproliferative disorder (CD3-/CD4-/FIP1L1-PDGFRA-). He was treated with a trial of high dose corticosteroids and adequate control of eosinophilia was achieved. Due to his widespread arterial and venous thrombi, he was discharged on warfarin.

Two weeks after discharge, the patient returned with a gangrenous right foot and was found to have refractory hypereosinophilia (WBC, 21x103; eosinophils 5.3x103) with recurrent thrombosis, which required a transmetatarsal amputation. Weekly methotrexate 20 mg/m2 IV and dexamethasone 40mg IV were initiated as treatment. Despite continued treatment with steroids and warfarin over the next two months, his hypereosinophilia persisted and symptoms of the disease continued to progress-eventually involving his fingers.

This case represents a unique presentation of T-cell mediated HES with a lymphocytic variant. The extent of eosinophil-mediated venous and arterial thrombi is greater than typically found in the literature. Moreover, the presence of complications such as hemolytic anemia may be a marker for disease refractoriness and prognosis, and should be considered when determining treatment options.

Back to August 2014 Issue of IMpact

More Articles Like This