Ocular Manifestations of Familial Lymphohistiocytosis

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

“…This makes sense, since the choroid is a richly vascularized tissue that is frequently involved in systemic inflammatory and neoplastic disorders. Other documented sites are the optic nerve, retina, vitreous, anterior chamber, and trabecular meshwork [26,27,29]. From our perspective, another interesting finding is that the ocular involvement in each of the cases we present displayed not only a bilateral distribution but was also symmetric in terms of severity in each eye.…”

“…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.…”

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

“…This makes sense, since the choroid is a richly vascularized tissue that is frequently involved in systemic inflammatory and neoplastic disorders. Other documented sites are the optic nerve, retina, vitreous, anterior chamber, and trabecular meshwork [26,27,29]. From our perspective, another interesting finding is that the ocular involvement in each of the cases we present displayed not only a bilateral distribution but was also symmetric in terms of severity in each eye.…”

“…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.…”

Hemophagocytic Lymphohistiocytosis With Isolated Central Nervous System Reactivation and Optic Nerve Involvement

“…Fundal changes such as papilledema and vitreous opacities resembling severe uveitis have been previously reported. 11 Case reports documenting a spectrum of ophthalmic histopathological findings have been published, 12 but to our knowledge, magnetic resonance imaging findings of optic nerve involvement have not been previously reported in patients with hemophagocytic lymphohistiocytosis. The thickened and enhancing optic nerve seen in our patient may represent lymphohistocytic infiltration described in previous studies.…”

Haemophagocytic Lymphohistiocytosis: A Case Report and Short Overview

“…19 Retinal hemorrhages, swelling of the optic disc and infiltration of the choroid have been reported in infants with HLH. 20 Diffuse peripheral neuropathy with pain and weakness secondary to myelin destruction by macrophages may also occur. The workup for HLH includes a complete and differential blood count, renal function tests, liver function tests, fasting triglycerides, international normalized ratio, partial thromboplastin time, fibrinogen, and ferritin.…”

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

“…This makes sense, since the choroid is a richly vascularized tissue that is frequently involved in systemic inflammatory and neoplastic disorders. Other documented sites are the optic nerve, retina, vitreous, anterior chamber, and trabecular meshwork [26,27,29]. From our perspective, another interesting finding is that the ocular involvement in each of the cases we present displayed not only a bilateral distribution but was also symmetric in terms of severity in each eye.…”

“…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.…”

Hemophagocytic Lymphohistiocytosis With Isolated Central Nervous System Reactivation and Optic Nerve Involvement

“…Fundal changes such as papilledema and vitreous opacities resembling severe uveitis have been previously reported. 11 Case reports documenting a spectrum of ophthalmic histopathological findings have been published, 12 but to our knowledge, magnetic resonance imaging findings of optic nerve involvement have not been previously reported in patients with hemophagocytic lymphohistiocytosis. The thickened and enhancing optic nerve seen in our patient may represent lymphohistocytic infiltration described in previous studies.…”

Haemophagocytic Lymphohistiocytosis: A Case Report and Short Overview

“…19 Retinal hemorrhages, swelling of the optic disc and infiltration of the choroid have been reported in infants with HLH. 20 Diffuse peripheral neuropathy with pain and weakness secondary to myelin destruction by macrophages may also occur. The workup for HLH includes a complete and differential blood count, renal function tests, liver function tests, fasting triglycerides, international normalized ratio, partial thromboplastin time, fibrinogen, and ferritin.…”

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

“…This makes sense, since the choroid is a richly vascularized tissue that is frequently involved in systemic inflammatory and neoplastic disorders. Other documented sites are the optic nerve, retina, vitreous, anterior chamber, and trabecular meshwork [26,27,29]. From our perspective, another interesting finding is that the ocular involvement in each of the cases we present displayed not only a bilateral distribution but was also symmetric in terms of severity in each eye.…”

“…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.…”

Hemophagocytic Lymphohistiocytosis With Isolated Central Nervous System Reactivation and Optic Nerve Involvement

“…Fundal changes such as papilledema and vitreous opacities resembling severe uveitis have been previously reported. 11 Case reports documenting a spectrum of ophthalmic histopathological findings have been published, 12 but to our knowledge, magnetic resonance imaging findings of optic nerve involvement have not been previously reported in patients with hemophagocytic lymphohistiocytosis. The thickened and enhancing optic nerve seen in our patient may represent lymphohistocytic infiltration described in previous studies.…”

Haemophagocytic Lymphohistiocytosis: A Case Report and Short Overview

“…19 Retinal hemorrhages, swelling of the optic disc and infiltration of the choroid have been reported in infants with HLH. 20 Diffuse peripheral neuropathy with pain and weakness secondary to myelin destruction by macrophages may also occur. The workup for HLH includes a complete and differential blood count, renal function tests, liver function tests, fasting triglycerides, international normalized ratio, partial thromboplastin time, fibrinogen, and ferritin.…”