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Periorbital lesions after allogenic hematopoietic stem cell transplantation: Acute graft versus host disease? – a case report

Posted on: Junho 10, 2019 Posted by: admin Comments: 0

Periorbital lesions after allogenic hematopoietic stem cell transplantation: Acute graft versus host disease? – a case report

Gonçalo Alves

Introduction:

Graft versus host disease (GVHD) represents a major cause of mortality and morbidity for patients who received allogenic hematopoietic stem cell transplantation (HSCT). The immunologic reaction mainly affects the liver, skin and the gastro-intestinal tract, with a diverse clinical picture. The correct and prompt diagnosis is not always easy, due to other common complications associated to the compromised immune system.

Case report:

We present a 49 years-old female, with the diagnosis of Acute Myeloid Leukemia, that made two allogenic HSTC from a related matched donor.
Seven days after the second HSCT, she developed smooth bilateral eyelid edema and chemosis. Main diagnoses suspected were leukemic infiltration versus drug hypersensitivity reaction, without ruling out cellulitis.
Orbit CT showed bilateral symmetrical episclerotic collections with mild pre-septal soft-tissue densification, findings confirmed in a orbit MRI made the day after, made short due to rapid clinical deterioration (odynophagia and a diffuse rash).
For the next seven days, the systemic disease rapidly progressed, with a marked increase of serum liver enzimes (bilirubin above 6 mg/dl), diffuse erythroderma with complains of dysesthesia, and mucositis stage IV with severe dysphagia.
The patient died fourteen days after the second bone marrow transplantation.

Discussion:

To our knowledge, periorbital involvement as initial presentation in GVHD is rare.
Despite not being possible to rule out drug hypersensitivity reaction, the clinical features and the imaging findings of symmetrical periconjunctival fluid collections with scarce soft tissue edema, in a patient with recent allogenic HSTC, suggests the diagnosis of GVHD.
From our perspective, and although the lack of histologic examination, the imaging results fit the previous reported CT findings and add some MRI characterization of the periorbital manifestation of the disease.
When clinically evident, orbit imaging can contribute to an earlier and more accurate GVHD diagnosis, as well as treatment optimization.