Ocular Findings in a Patient with Virus-associated Hemophagocytic Syndrome

Yao, Huiping; Liu, Yan; Ding, Wei; Xu, Youlong; Cheng, Yu

doi:10.3109/09273948.2012.665123

Cited by 10 publications

(4 citation statements)

References 4 publications

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“…One patient developed severe uveitis leading to significant reduced visus. While HLH-associated uveitis has been previously reported in one case report [ 13 ], we cannot rule out other causes for this condition like CMV infection or cidofovir treatment.…”

Section: Discussionmentioning

confidence: 82%

Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents—a single centre experience of 20 years

et al. 2018

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Haemophagocytic lymphohistiocytosis (HLH) is a possibly life-threatening syndrome of immune dysregulation and can be divided into primary (hereditary) and secondary forms (including malignancy-associated HLH (M-HLH)). We retrospectively analysed epidemiological, clinical, virological and laboratory data from patients with M-HLH treated at our department between 1995 and 2014. Out of 1.706 haemato-/oncologic patients treated at our department between 1995 and 2014, we identified 22 (1.29%) patients with secondary HLH (1.3–18.0, median 10.1 years; malignancy induced n = 2; chemotherapy induced n = 20). Patients with acute myeloblastic leukaemia (AML) developed HLH significantly more often than patients with acute lymphoblastic leukaemia (ALL) (10/55, 18.2% vs. 6/148, 4.1%, p = 0.0021). As possible viral triggers, we detected BKV (53.8% of the tested patients), HHV-6 (33.3%), EBV (27.8%), CMV (23.5%), ADV (16.7%) and PVB19 (16.7%) significantly more frequently than in haemato-/oncologic patients without HLH. Despite lacking evidence of concurrent bacterial infection, C-reactive protein (CRP) and procalcitotnin (PCT) were elevated in 94.7 and 77.7% of the patients, respectively. Ferritin and sIL2R were markedly elevated in all patients. HLH-associated mortality significantly (p = 0.0276) decreased from 66.6% (1995–2004) to 6.25% (2005–2014), suggesting improved diagnostic and therapeutic management. Awareness of HLH is important, and fever refractory to antibiotics should prompt to consider this diagnosis. Elevated ferritin and sIL2R seem to be good markers, while inflammatory markers like CRP and PCT are not useful to discriminate viral triggered HLH from severe bacterial infection. Re-/activation of several viruses may play a role as possible trigger.

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Section: Discussionmentioning

confidence: 82%

Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents—a single centre experience of 20 years

et al. 2018

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“…Of interest, intraocular involvement is an uncommon presentation of HLH [17,18,19,20,21,22,23,24,25], and, to our knowledge, histologic findings have been previously illustrated only in a handful of cases , all of them pediatric at presentation (ranging in the age from 9 weeks to 7 years, median 2.1 years) (Table 3) [26,27,28,29]. Except for the adult onset, the clinical and histologic findings in our present cases are somewhat similar to those previously reported.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases

Vizcaíno¹,

Eberhart²,

Rodríguez³

2017

Ocul Oncol Pathol

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Background/Aims: Hemophagocytic lymphohistiocytosis (HLH) is an infrequent inflammatory multisystemic syndrome. Only rare cases with ophthalmic involvement describing their pathologic features have been previously reported. Methods: We report 3 cases of adult-onset HLH with bilateral ocular involvement and describe their clinicopathologic features. Results: Three adult males - 2 with a history of viral infection - developed persistent fever, fatigue, bone marrow abnormalities, and irreversible multiorgan failure. Visual impairment was also documented in 2 cases. Complete autopsies were performed. Ophthalmic pathology demonstrated a bilateral histiocytic infiltrate with scant lymphocytes affecting the uvea. Focal extension to the retina, optic nerve, and trabecular meshwork were also identified, as well as hemophagocytosis in 1 case. Macrophages showed strong immunoreactivity for CD163 antibody and lacked BRAF p.V600E mutant protein. Conclusion: HLH is an unusual disorder associated with several systemic conditions. Histologic features in the eye are poorly documented, with prior reports restricted to children. Our 3 adult cases are reported using updated criteria and, despite the difference in age, show changes similar to those observed in the pediatric population.

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“…We searched PubMed for previously reported patients who developed ocular lymphohistiocytic involvement, selecting papers in which ocular signs and symptoms could be ascribed to HLH, either familial or infection-induced and excluding those in which ophthalmic manifestations were part of the pre-existing clinical picture. Posterior segment abnormalities were detected, including retinal hemorrhages requiring vitrectomy, disc edema, acute posterior multifocal placoid pigment epitheliopathy, and multiple bilateral serous pigment epithelial detachments with macular edema, resembling ocular Vogt–Koyanagi–Harada disease ( 9 – 11 ). Moreover, other manifestations included subconjunctival hemorrhage, ocular surface infection, orbital cellulitis, and pseudotumor ( 12 ).…”

Section: Review Of Other Forms Of Hlh Ocular Infiltrationmentioning

confidence: 99%

Case report: EBV-related eye orbits and sinuses lymphohistiocytic infiltration responsive to rituximab in a patient with X lymphoproliferative syndrome type 1

Giardino,

Lanni,

Mascolo

et al. 2024

Front. Immunol.

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Background and aimsX lymphoproliferative syndrome type 1 (XLP1) is a rare inborn error of immunity due to mutations of SH2D1A, encoding for slam-associated protein (SAP). The clinical phenotype includes severe mononucleosis, hemophagocytic lymphohistiocytosis (HLH), and B-cell lymphomas.MethodsWe report the case of a child affected with XLP1 who presented with an incomplete HLH, triggered by Epstein–Barr virus (EBV) and treated with rituximab, involving orbits and paranasal sinuses.ResultsThe lesion was indistinguishable from lymphoma, complicating diagnosis and treatment. In addition, considering the high incidence of lymphoma in patients with XLP1, histology helped define its nature, driving therapeutic choices.ConclusionWe described an unusual presentation of incomplete HLH in a patient affected with XLP1: an EBV-driven infiltration of the orbits and paranasal sinuses. This led us to a challenging differential diagnosis of lymphoma-associated hemophagocytic syndrome, which can be frequently observed in patients with XLP1. Considering the extremely poor prognosis of this clinical finding, we sought for a prompt diagnosis and managed to obtain it and to immediately establish the right treatment on the basis of the pathological finding.

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Ocular Findings in a Patient with Virus-associated Hemophagocytic Syndrome

Cited by 10 publications

References 4 publications

Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents—a single centre experience of 20 years

Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents—a single centre experience of 20 years

Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases

Case report: EBV-related eye orbits and sinuses lymphohistiocytic infiltration responsive to rituximab in a patient with X lymphoproliferative syndrome type 1

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