IntroductionHemophagocytic lymphohistiocytosis (HLH) is a potentially lethal immune system dysregulation in children and adults. Failure to properly inhibit the immune response leads to constant and excessive activity of the cytotoxic T-cells, natural killer (NK) cells, and macrophages. Inflammatory reactions caused by the intense activity of the cellular immune system and cytokine storm in tissues lead to multiple organ failure in this disease. HLH is categorized as primary, which defines as the presence of a predisposing genetic mutation in the immune system, and reactive to an infectious, inflammatory, or malignant trigger. The diagnosis is challenging as it has no clinical or laboratory pathognomonic features. Fever, organomegaly, liver dysfunction, cytopenias, coagulopathy, hemophagocytosis, and neurologic dysfunction are common manifestations of HLH.Ocular involvement is relatively rare in HLH. Unilateral panuveitis, Purtscher retinopathy, trabecular meshwork involvement, and choroidal infiltration with secondary extension to the retina and optic nerve head (ONH) have been reported previously.This report aims to introduce a child with HLH and bilateral ONH infiltration.
A 49-year-old male presented with a gradually progressive loss of vision in both eyes. Funduscopic examination revealed bilateral optic disc swelling and two yellowish elevated choroidal lesions in the LE. Ophthalmic imaging raised suspicion of choroidal metastasis. Adenocarcinoma of the lung was detected in further systemic evaluation.
Introduction:Brucellosis is a zoonotic infectious disease with a wide range of manifestations including malaise, anorexia, fever, and profound muscular weakness, as described by Marston in 18601. It is caused by the Gram-negative coccobacillus, Brucella, and remains endemic in some developing countries, such as Iran. There are 6 types of brucella that 4 of which include Brucella melitensis, Brucella abortus, Brucella canis, and Brucella suis were recognized as pathogens involving humans. Brucella melitensis was described as the most common and virulent pathogen worldwide. The first case of ocular brucellosis in a human being was described by Lemaire in 1924 2, presented with bilateral optic neuritis and external ophthalmoplegia in a patient with brucella meningitis.Ocular manifestations of acute and chronic infection include anterior and posterior uveitis, panuveitis, keratitis, conjunctivitis, papillitis, cataract, maculopathies, glaucoma, and ocular muscle paresis. Modern treatments of ocular brucellosis, intraocular as well as systemic antibiotics, have improved the prognosis of the disease3. Herein, we present a patient with endogenous endophthalmitis caused by Brucella Melitensis (B.Melitensis), which is very rare and unusual.