Bari University Medical School Piazza Giulio Cesare 11, 70124,Bari
silvanaguerriero@gmail.com
Dr Silvana Guerriero was born in Venice ,Italy, on 5/2/56. Dr. Silvana Guerriero graduated in medicine and surgery at the Bari University , in Italy in 1980 obtaining graduation marks 110/110 cum laude, discussing a thesis on “ Experimental retinal detachment : comparison between sonographic imaging and histological preparations”. Dr. Guerriero obtained the license to practice medicine in1980 with 100/100 marks and specialized in Ophthalmology at the Eye Clinic of the University of Bari in July 1984 with 50/50 marks and praise. From 1983 to 19988 Dr: Guerriero was employed as researcher associate of the National Research Counsil at the Eye Clinic of Bari. From 01/01/1990 to 12/31/1990 she obtained a permanent position at the Bari University as Technical Graduate of the Ophthalmology Clinic. In 1990 she became Researcher (Medical Director) at the Department of Ophthalmology, University of Bari, Italy. Since 1988, Dr. Guerriero is responsible for the Service of Ultrasonography of the Ophthalmologic department of Bari University. She is also responsible for the service for Ocular Inflammatory Disease, and Ocular Rare Disease. Since 2003 Dr Guerriero collaborates with the Department of Medical Biochemistry , Biology and Physics of the University of Bari and the Institute of Bioenergetics and Biomembranes (IBBE) of the National Research Council for the study of mitochondrial disease and hereditary optic nerve disease, in particular of Leber Optic Hereditary Atrophy. Since 1994, Dr. Guerriero regularly teaches in the graduated school of Ophthalmology and for the degree in speech therapy and orthoptic.
Abstract: Purpose: To evaluate circulating CD4(+)CD25(+) regulatory T-cell populations in patients with autoimmune uveitis and to assess whether T-regulatory cell populations correlate with clinical features. Methods: Sixty-four patients with noninfectious uveitis were enrolled. Following isolation and purification of peripheral blood mononuclear cells, Tregs were analyzed by assessing FOXP3 expression on T cells with an APC anti-human FOXP3 staining kit. Samples were simultaneously stained with anti-CD25-PeCy7, anti-CD3-FITC, anti-CD4-PercP, and anti-CD152 (CTLA-4)-PE monoclonal antibodies. Results: A decrease in CD4(+)CD25(+)FOXP3(+) T cells was detected in patients with active uveitis compared with healthy controls (p < .05). In patients achieving clinical remission, the percentage of CD4(+)CD25(+)FOXP3(+) T cells increased up to values comparable to those of healthy controls, in step with an increased expression of CTLA4. Conclusion: Therapy of autoimmune uveitis results in the increase of T-regulatory lymphocyte population and restoration of their functional state. These changes are likely to contribute to the patients' clinical improvement or disease regression.
Abstract: Leber Hereditary Optic Neuropathy is associated to mitochondrial DNA (mtDNA) ND-mutations, mostly homoplasmic. However, these mutations are not sufficient to explain the peculiar features of penetrance and the tissue-specific expression of the disease and are believed to be causative in association with unknown environmental or other genetic factors. Discerning between clear-cut pathogenetic variants, such as those that appear to be heteroplasmic, and less penetrant variants, such as the homoplasmic, remains a challenging issue that we have addressed using next generation sequencing approach. We set up a protocol to quantify MTND5 heteroplasmy levels in a family in which the proband manifests a LHON phenotype. Furthermore, to study this mtDNA haplotype, we applied the cybridization protocol. The results demonstrate that the mutations are mostly homoplasmic whereas the suspected heteroplasmic feature of the observed mutations is due to Nuclear mitochondrial Sequences (NumtS) coamplification.
Abstract: A 65-year-old woman presented with a long standing, progressive exophthalmos of the right eye. Her medical history was significant for Churg Strauss syndrome, and was treated with immunosuppressive therapy. She had undergone two previous orbital biopsies showing inflammatory reactive lymphoid hyperplasia. A diagnosis of orbital inflammation in Churg-Strauss syndrome was suspected, and the immunosuppressive therapy was increased. Because of the lack of response to therapy, a further biopsy was performed, by lateral orbitotomy approach. Biopsy of the mass revealed a granular cell tumor composed of S-100 positive cells with an acidophilic granular cytoplasm and peripheral lymphocytic infiltration. A granular cell tumor, which is very rare in the orbit, should be considered in the differential diagnosis of orbital tumors, and if suspected, an excisional biopsy must be undertaken. Typical histopathological aspect of the granular cell tumor is characterized by the presence of S-100 positive closely packed polygonal cells with a granular cytoplasm.
Abstract: Purpose: To describe a rare case of conjunctival leiomyosarcoma initially diagnosed as a poorly differentiated squamous cell carcinoma. Methods: Clinical, light microscopic, immunohistochemical, and ultrastructural findings are reported. Results: A 56-year-old Caucasian woman was referred with a history of a progressive, rapidly growing mass in her left eye. Biopsy of the mass and histology yielded a first diagnosis of a poorly differentiated conjunctival squamous cell carcinoma. Orbital exenteration was performed 2 weeks later. Macroscopically, the exenteration specimen showed a soft mass completely involving the conjunctiva and extending to the eyelids and orbital structures. Histological examination revealed a malignant tumour composed of atypical, predominantly epithelioid large cells. Immunohistochemical and ultrastructural studies combined with the light microscopic findings contributed to clarify the diagnosis of epithelioid leiomyosarcoma. The patient was started on chemotherapy and radiotherapy, but died a few months later from widespread metastases. Conclusions: primary involvement of the orbit by a leiomyosarcoma is rare, but this eventuality should be considered in the differential diagnosis of rapidly growing orbital and conjunctival masses.
Abstract: Purpose: To describe the genotype and phenotype in a 9-year-old boy with bilateral retinopathy. Methods: The patient, his healthy (by history) nonconsanguineous parents and his sister were examined by best-corrected visual acuity, matrix frequency doubling technology, monocular static field analysis, fundus autofluorescence imaging, optical coherence tomography, Ganzfeld electroretinography (ERG), pattern ERG, multifocal ERG, electro-oculography and genotyping of the BEST1 gene. Results: The patient presented with an Arden ratio of 1.25, an unremarkable ERG and fluorescent yellow deposits distributed throughout the fundus suggestive of autosomal recessive bestrophinopathy (ARB). Genotyping revealed a homozygous nonsense mutation in BEST1 (p.R200X). The parents and the sister, who were heterozygous mutation carriers, presented with normal ophthalmological function. Conclusions: ARB is a rare retinal disorder. We contribute a novel patient report indicative of ARB, assessed by clinical examination and confirmed by genotyping of BEST1, to the short list of ARB cases in the literature.
Abstract: PURPOSE:: To report a case of sympathetic ophthalmia (SO) after a severe Acanthamoeba keratitis. METHODS/DESIGN:: Interventional case report. RESULTS:: A 59-year-old Caucasian woman, wearing contact lenses, developed a severe Acanthamoeba keratitis in the left eye, which involved the limbus, and required 8 months of intensive antiamoeba therapy; the condition resolved leaving a painful, phthisical eye with complete corneal neovascularization. Six months later, the patient presented with pain, blurred vision, and photophobia in the right eye. Slitlamp examination of the right eye revealed granulomatous uveitis. On the suspicion of an SO, treatment with high-dose topical and oral corticosteroids and immunosuppressants was started. After 3 months, the eye is stable, with a visual acuity of 20/50, and the patient is taking prednisolone 7.5 mg per day and cyclophosphamide 50 mg per day. CONCULSIONS:: Development of SO in the absence of previous trauma or surgery is rare. Our case is the first report of a clinically diagnosed SO after an episode of severe Acanthamoeba keratitis.
Abstract: To describe ultrasound biomicroscopy (UBM) features in a patient with Axenfeld-Rieger syndrome (ARS) and cataract before and after anterior segment surgery using intraocular lens (IOL) and aniridic ring implantation (Morcher Aniridia rings 50 D).
Abstract: Leber's hereditary optic neuropathy (LHON) is a maternally inherited, monosymptomatic disorder, characterized by severe central vision loss and optic atrophy that most frequently affects young men. The classic LHON phenotype is associated to three mitochondrial DNA mutations, mostly homoplasmic, in the Mt-ND4, Mt-ND6, and Mt-ND1 genes, encoding for complex I subunits of the mitochondrial respiratory chain. Rare cases have been described in the literature in association with variable central nervous system involvement in a syndromic form called LHON 'plus.' In the present study, we report the case of a 16-year-old boy with the 3460/ND1 mutation who presented with epilepsy, migraine, and mental retardation as non-ophthalmic features. We also investigated his relatives who all had the 3460/ND1 mutation.
Abstract: Doubts still exist concerning the mechanisms involved in ocular ischemic syndrome (OIS) and its dependence on carotid disease. We report findings from 14 surgical patients undergoing carotid artery reconstruction for symptomatic cerebrovascular disease. All of them had fluorescein angiography (FA) of the eye ipsilateral to the carotid operation before surgery and 3 months after to provide information regarding retinal circulation time. Before the surgical procedure, the mean circulation time was 29.4 ± 9.4 seconds (CI 95%: 24.5-34.3). After 3 months, a significant (P < .001) decrease in the circulation time was observed: 18.9 ± 8.4 seconds (CI 95%: 14.5-23.4). The present series demonstrates that carotid revascularization surgery improved retinal flow in approximately 80% of the patients.
Abstract: Purpose:To describe a rare case of an orbital undifferentiated sarcoma arising in an eviscerated eye socket with a silicone implant. Methods: The clinical and histopathological findings of the case are reviewed and presented. Results: A patient who had undergone post-traumatic evisceration of the right eye and a silicone prosthesis implant 8 years earlier, presented to our observation with a painful eye, on which a brownish, bleeding mass, measuring 1âcm in diameter, was evident.After removal of the silicone prosthesis, the mass was found to arise from the scleral socket. Histopathological analysis demonstrated a malignant tumor composed of markedly pleomorphic cells with a high mitotic rate. An undifferentiated high-grade pleomorphic sarcoma was diagnosed. Complete right orbital exenteration was performed and the patient underwent radiotherapy Conclusions:This is the first report of an undifferentiated high-grade pleomorphic sarcoma arising from a scleral socket with a silicone implant.
Abstract: The authors present a rare form of orbital cavitary rhabdomyosarcoma in which lymphangioma was mistakenly diagnosed on the basis of echography and MRI. Rhabdomyosarcoma can usually be differentiated from lymphangioma by echographic and MR imaging, because cavitation is very rare in orbital rhabdomyosarcoma, but rhabdomyosarcoma should be suspected whenever the clinical presentation of a rapidly progressive unilateral exophthalmos is observed in a child.
Abstract: Aicardi syndrome is characterized by agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. The authors describe the case of a girl with chorioretinal lacunae, seizures, and cerebral cyst, but a normal corpus callosum. Incomplete forms of Aicardi syndrome are reported in the literature, but typical choroidal lacunae are always present and are pathognomonic.
Abstract: PURPOSE: To report a masquerade syndrome secondary to multiple myeloma iris infiltration. DESIGN: Observational case report. METHODS: A 74-year-old Caucasian woman presenting with unilateral hypertensive uveitis and past medical history significant for multiple myeloma underwent aqueous cytology and a trabeculectomy with iridectomy. RESULTS: Cytology revealed atypical plasma cells in the aqueous while the anatomopathologic analysis of iris tissue demonstrated a substitution of the iris tissue by neoplastic plasma cells. After surgery and a new cycle of chemotherapy best-corrected visual acuity and intraocular pressure improved. CONCLUSIONS: Masquerade syndrome should always be considered in elderly uveitis. A correct diagnosis can be life-saving.
Abstract: Orbital pseudotumor is a benign, idiopathic, non-infectious and non-neoplastic clinical syndrome characterized by the presence of an inflammatory mass at orbital level with no identifiable cause. The disease is rarely observed in the pediatric population. This article describes a relapsing bilateral orbital pseudotumor in a young girl. The diagnostic implications and treatment strategies are discussed.
Abstract: PURPOSE: To report ultrabiomicroscopy (UBM) evidence of choroid effusion as a cause of acute transient myopia in the onset of systemic lupus erythematosus. DESIGN: Observational case report. METHODS: A Caucasian 16-year-old girl was referred to the Hematology Department for progressive anemia, intermittent fever, and blurred vision. RESULTS: Systemic lupus erythematosus was confirmed by hematological investigations. UBM examination evidenced choroid effusion as the cause of a transient myopic shift. CONCLUSIONS: Early diagnosis, thanks to the performance of an ultrasound examination, enabled the timely institution of therapy and hence a rapid resolution of the symptoms.
Abstract: A 15-year-old boy underwent neurological and ophthalmological evaluation. At birth, a severe bilateral microphthalmia, micropenis, and scrotal hypoplasia were diagnosed. Ophthalmologic examination showed right anophthalmia and severe left microphthalmia. Radiological examination showed normal orbital and skull structures. Magnetic resonance imaging (MRI), revealed the absence of the right eye, left microphthalmia, optic nerve hypoplasia, aplasia of the optic chiasm, and tracts. Audiometric examination and electroencephalogram were normal. There was no mental retardation. The chromosomal examination was normal. The patient is examination was also negative for any type of known risk factors.
Abstract: PURPOSE: Verify the recovery of visual capacity after the administration of a combination of LDL apheresis (LA) and conventional therapy (CT). Design, prospective and interventional case series. METHODS: 20 patients affected by NAION were randomly subdivided into two groups of 10 patients (Group 1 and Group 2). Group 1 underwent three sessions of LA associated with CT, whereas group 2 received only CT. At discharge and at the 6 months follow-up visit, assessment in both groups was made of the best corrected visual acuity (BCVA) and the computerised visual field (CVF), comparing the findings with those at admission in each patient. RESULTS: Only the mean deviation (MD) at CVF was statistically improved in group 1 as compared with group 2 at discharge, judged against the values at admission (-11.08+/-6.51 vs -16.53+/-10.03, P=0.039; -17+/-5.24 vs -14.14+/-9.42, respectively). However, this increase was not confirmed at 6 months (-16.83+/- -10.72, group 1; -13.56+/-3.60 group 2). CONCLUSION: In NAION, LA induced a short term improvement in the MD, but by 6 months this had disappeared.
Abstract: BACKGROUND: To report a case of masquerade syndrome presenting as bilateral uveitis in an HCV positive patient, and to highlight the difficulties in distinguishing between chronic uveitis and malignancy-induced inflammation. CASE REPORT: In January 2005 a 54-year-old Caucasian man was referred to the Ophthalmological Department for bilateral visual loss, severe vitritis, and a significant cataract in both eyes. His clinical history was significant for HCV infection. The uveitis treated with low dose of steroids and immunosuppressors, yielding a partial remission of the symptoms. One year later he developed a Primary Central Nervous System Lymphoma. In January 2007 he returned to our department for cataract surgery. The patient underwent phacoemulsification of the cataract in the right eye, intraocular lens implantation and intravitreal injections of 4 mg triamcinolone acetonide. After one month fundus biomicroscopy showed a solid lesion at the posterior pole, consistent with a retinal relapse of the Primary Central Nervous System Lymphoma. Restaging investigations were unremarkable and ruled out a disease relapse, and a diagnostic vitrectomy showed only rare inflammatory cells. In view of the progressive swelling of the retinal lesions we decided to treat the patient with intravitreal Methotrexate. Complete remission of the retinal lesions with retinal scarring was achieved after 12 months. In May 2008 the patient underwent phacoemulsification of the cataract in the left eye and intraocular lens implantation. A vitreal tap was performed and was positive for rare abnormal cells CD45+, CD20-. Vitreous sampling did not yield enough cells for a diagnosis of monoclonality. No systemic or intravitreal therapy was performed because of the absence of central nervous system relapses, the small number of atypical cells found in the vitreous sample and the absence of retinal masses. After three months the patient developed a central nervous system relapse of the lymphoma and rapidly died. CONCLUSION: In elderly patients suffering from uveitis a masquerade syndrome should always be suspected. Vitreous sampling may not yield enough cells for diagnosis and the vitritis may be steroid-sensitive, at least initially. This makes a differential diagnosis between chronic uveitis and malignancy-induced inflammation very difficult.
Abstract: Aicardi syndrome is characterized by agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. The authors describe the case of a girl with chorioretinal lacunae, seizures, and cerebral cyst, but a normal corpus callosum. Incomplete forms of Aicardi syndrome are reported in the literature, but typical choroidal lacunae are always present and are pathognomonic.
Abstract: Thus far there is little data available concerning Acanthamoeba associated amoebic keratitis (AK) from Italy. In order to understand the incidence of Acanthamoeba in patients with ocular infections and to characterize the isolates at the molecular level, ocular specimens and contact lenses or lens case solutions from 140 patients were analysed by culture and by an 18S rRNA (Rns) gene-based PCR method. Nineteen (13.6%) patients showed Acanthamoeba culture positive samples. Eleven out of the 14 genetically characterized isolates were assigned to the T4 genotype. Three isolates, two of them from patients with keratitis responding to specific anti-Acanthamoeba therapy, were identified as belonging to the T15 genotype. This finding represents the first association between the T15 genotype and human amoebic keratitis. PCR amplification of the 18S ribosomal DNA proved to be a sensitive method, potentially able to detect Acanthamoeba without the need of long culture incubation, and thus considerably useful for clinical applications.
Abstract: OBJECTIVES: We report ultrasound biomicroscopy (UBM) evidence of Descemet's membrane rupture in a patient affected by Acanthamoeba keratitis without corneal perforation. METHODS: A 38-year-old woman who was a habitual wearer of monthly disposable soft contact lens was admitted to our unit for a severe ulcerated corneal abscess. Two days after admission, the patient presented acute stromal hydrops. Ultrasound biomicroscopy examination revealed a Descemet's membrane rupture and an inflammatory reaction in the anterior chamber. Smears with Gram 17 staining and cultural examination revealed the presence of Acanthamoeba. We started specific therapy with propamidine 0.1% and polyhexamethylene biguanide 0.02% eyedrops; we suspended propamidine therapy after 2 weeks form the onset, when the ulcer had recovered, and we continued topical therapy with polyhexamethylene biguanide 0.02% for 6 months. We performed UBM examinations at each control visit during the follow-up that lasted for 18 months. RESULTS: At 1-month's follow-up, the symptoms and corneal alterations had significantly improved, while UBM revealed a spontaneous reattachment of the endothelium-Descemet layer. At 12 months' follow-up, best-corrected visual acuity had improved from hand movements at onset to 20/40, and no sign of intraocular involvement was evidenced. CONCLUSIONS: Acanthamoeba is a ubiquitous protozoan that can affect the cornea, even colonizing the deep layers, causing rupture of the Descemet's membrane. Ultrasound biomicroscopy examination confirmed the severe pathogenicity of this parasite and confirmed that only a prompt diagnosis can limit the damage caused by this affection.
Abstract: In ocular ischemic syndrome, the ischemic condition is due to markedly reduced blood flow and increased vascular resistance of retrobulbar circulation, as in the central retina and posterior ciliary arteries. Chronic hypoxia affects even the iris and ciliary body. The hypoperfusion of the ciliary body leads to a relative hypotony, presumably related to decreased aqueous humor production. Histopathologic study has demonstrated ciliary body atrophy on an ischemic basis. The authors used ultrasound biomicroscopy to demonstrate in vivo the hypotrophic condition of the ciliary body in ocular ischemic syndrome.
Abstract: PURPOSE: Amyloidosis refers to a heterogeneous group of disorders associated with deposition of chemically distinct fibril proteins. Isolated orbital amyloidosis is a rare condition and requires systemic examination. The authors report a case of amyloid deposit in the orbit whose systemic investigation has been negative. METHODS: A 64-year-old woman presented to the eye clinic with left-sided orbital mass, mild exophthalmos with downward ocular displacement, and ptosis. The patient presented also visual acuity loss and ocular hypertension. No systemic involvement was noted by systemic workup and it confirmed the primary orbital amyloidosis. RESULTS: The patient was subject to full clinical examination, laboratory examinations, orbital echography, magnetic resonance imaging and total body computed tomography scans, rectal mucosa, and temporal artery biopsies. It was necessary to exclude systemic amyloidosis. The presence of amyloid deposits was confirmed by biopsy of orbital mass. The chemical nature of deposit was characterized using light microscopy, immunohistochemistry, and electron microscopy. CONCLUSIONS: Orbital amyloidosis is a very rare disease. It should be considered in the diagnosis of patients with ptosis and exophthalmos. The treatment usually consists of surgical removal of the amyloid mass and follow-up for a likely local recurrence. In our case, mass excision determined the decrease of exophthalmos and intraocular pressure.
Abstract: Tubulointerstitial nephritis and uveitis syndrome is likely underdiagnosed. A 13-year-old girl with no significant medical history had photophobia, ocular pain, and decreased visual acuity. Bilateral papilledema was present. Renal biopsy revealed interstitial nephritis. Immunosuppressive therapy was added (3 mg/kg/d of cyclosporine). One year later, visual acuity was 20/20 and findings were normal on ocular examination and laboratory tests.
Abstract: PURPOSE: To report a case of pseudotumor cerebri (PTC) following treatment with human recombinant growth hormone (GH). METHODS: A 42-year-old man who developed pseudotumor cerebri 7 years after starting human recombinant GH treatment is presented. RESULTS: The patient's medical history was significant for hypophyseal dwarfism with a serious deficit of GH, hypogonadotropic hypogonadism, and hypothyroidism. In 1996 he started taking GH, testosterone, and L-thyroxine. Fundus examination showed disc edema in the left eye. GH was discontinued, and acetazolamide therapy was initiated. At the 3-month follow-up the acuity without correction was patch and the unilateral papilledema had resolved. CONCLUSIONS: Pseudotumor cerebri or idiopathic intracranial hypertension is an uncommon and complex disorder. The diagnosis is possible when important criteria symptoms and signs are met. Several conditions and risk factors are associated with PTC. The most recently recognized risk factor is GH therapy.
Abstract: Endothelial dysfunction of the optic microcirculation is considered to be the main pathogenetic mechanism in nonarteritic ischemic optic neuropathy. The aim of the present work was to assess whether a clinical improvement is correlated with a reduction in the endothelial activation markers by means of LDL apheresis (LDLA). Three weekly sessions of LDLA were administered in 23 patients affected by nonarteritic ischemic optic neuropathy. Statistically significant reductions were achieved in all parameters: total cholesterol (44.6%), LDL cholesterol (54.6%), fibrinogen (60.9%), von Willebrand factor (38.6%), sE-Selectin (22.6%), sICAM-1 (14%) and sVCAM-1 (15.5%), each of which was correlated with an improvement in the mean deviation of the visual field, although statistical significance for the single parameters was not reached. However, analysis of variance between the mean deviation improvement and the set of parameters taken together yielded highly significant results (p < 0.0001). LDLA was effective in reducing the values of all evaluated endothelial activation markers, and this trend was correlated with an improvement in the visual field.
Abstract: Nonarteritic acute anterior ischemic optic neuropathy (NAION) is a disabling disease which impairs visual function. It is presumed to result from disturbances of microcirculation in the anterior portion of the optic nerve head due to hemodynamic factors derived from excessive blood viscosity, or restriction of the vasal lumen in hypertensive, hypercholesterolemic, diabetic patients. We aimed to determine whether acute reduction of plasma fibrinogen and serum low-density lipoprotein (LDL) cholesterol is effective for treatment of NAION. We recruited 11 patients (7 females, 4 males) with a mean age of 57.2 +/- 19.6 years. All except one of them presented risk factors for atherosclerosis. The mean values of LDL-cholesterol and fibrinogen before treatment were 144 +/- 32 mg/dL and 341 +/- 80 mg/dL, respectively. All were treated with standard therapy (prednisone, salicylate, pentoxiphyllin) and underwent three sessions of LDL-apheresis (HELP system-B Braun) that can reduce plasma LDL-cholesterol and fibrinogen by more than 50% in a very short time. In all patients we observed a drastic reduction of LDL cholesterol and fibrinogen and a clear improvement in the visual functional data. In fact, mean values of corrected vision increased from 3.7/10 +/- 3/10 to 7.9/10 +/- 2.2/10 (P = 0.002) after the third session, while the scotomatous portion of the visual field regressed after the first session, and in 5 patients further regressed after the third session. This improvement had remained stable after 3 months. Thanks to it's effect of antagonizing hemorheologic disorders of the ocular microcirculation, fibrinogen/LDL-apheresis seems to be an efficacious treatment of NAION.
Abstract: Acute anterior ischemic optic neuropathy (AION) is a disabling disease which impairs visual function. Standard treatment is unable to affect the outcome and the visual damage persists. We describe the case of a 64-year-old patient affected by AION, whose only known risk factor was hypercholesterolemia. After a first onset of involvement of the right eye (RE), the patient presented four weeks later with an analogous episode affecting the left eye (LE). Since standard treatment, started at involvement of the RE, had not yielded any beneficial effect, the patient underwent three sessions of LDL apheresis. The scotomatous portion of the visual field reduced even after the first session, there was further improvement after the third, and after six months the condition remained stable. Corrected vision improved from 2/10 to 6/10 after the third session. LDL cholesterol and fibrinogen decresade after the third session from 239 mg/dL to 31 mg/dL and from 289 mg/dL to 92 mg/dL, respectively. In conclusion, thanks to its effect of antagonizing hemorheologic disorders of the ocular microcirculation, LDL apheresis seems to be an efficacious treatment of AION, especially in patients suffering from hypercholesterolemia.
Abstract: In this study, we assessed the Th1/Th2 polarization of the immune response and the involvement of dendritic cells (DC) and Th1 lymphocytes in the pathogenesis of uveitis. Thirty-seven patients with chronic idiopathic uveitis were enrolled: 21 of them had active uveitis and the remaining 16 were in complete remission. Patients with active uveitis were characterized as follows: 5 had intermediate uveitis, 5 panuveitis and the remaining 11 posterior uveitis. Thirteen healthy subjects were also studied as controls. Patients with active uveitis were treated with cyclosporin-A (CsA) associated to low doses of prednisone (PDS) and studied at baseline and after 6 months of therapy. Analysis of cytokine-producing CD3+ lymphocytes revealed a strong Th1 polarization of the immune response in patients with active uveitis. Th1 lymphocytes paralleled serum IL-12 levels and the response to therapy, which greatly reduced both IFN-gamma+/CD3+ lymphocytes and serum IL-12 levels, associated with a general clinical improvement. In vitro studies demonstrated that DC from untreated patients with active uveitis were mature and functionally active. In fact, they showed a higher ability to stimulate cell proliferation of allogeneic T cells in primary mixed lymphocyte reaction (MLR) and produced larger amounts of IL-12 than DC from CsA/PDS-treated patients and those in remission. These results demonstrate that CsA/PDS therapy impairs the capacity of mature DC to secrete IL-12 and inhibits their MLR activity.
Abstract: Retinal abnormalities (RA) are very frequently observed in adult patients with acute myeloid leukemia (AML), but the clinical significance of these findings has not been fully investigated. We examined the fundus oculi in a cohort of 122 adult patients with AML at presentation and analyzed some clinical and biological features to assess whether there was any association with RA. For this purpose, we subdivided the patients into two groups according to the presence or absence of RA (groups 1 and 2, respectively). We considered current laboratory parameters such as white blood cell (WBC) count, hemoglobin (Hb), platelets and serum lactate dehydrogenase (LDH). Moreover, we subdivided the patients into two groups according to age <60 (group A) or > or =60 years (group B) to evaluate a possible association between RA and response to treatment and/or overall survival (OS). In our series, a higher median age and a lower Hb value were associated with group 1 (p = 0.001 and p = 0.04, respectively); the median LDH value was 812 U/l (range 224-5,551) and 607 (range 181-5,244) for groups 1 and 2, respectively (p = 0.02). There was no association between RA and karyotypic alterations. In terms of outcome, in group A (<60 years), 80% patients who achieved complete remission (CR) were in group 2 vs. 13% nonresponders (NR) (p < 0.0001). Median OS of group 2 patients was 49.7 months compared with 7.2 months for those in group 1 (p = 0.002). In group B, 58% patients who achieved CR were in group 1 vs. 15% NR (p < 0.006). Median OS of patients in group 2 was 14.6 months compared with 2.9 months in group 1 (p = 0.02). Our data show that RA are significantly associated with some biological features and with shorter OS in AML patients and this parameter seems to be an effective clinical sign of poor prognosis in terms of CR.
