Invasive sino-orbital mycosis in an aplastic anemia patient caused by Neosartorya laciniosa.
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ABSTRACT:
SUBMITTER: Malejczyk K
PROVIDER: S-EPMC3666822 | biostudies-literature | 2013 Apr
REPOSITORIES: biostudies-literature
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Project description:ObjectiveInvasive sino-orbital fungal infections in immunocompetent patients are a rare clinical entity; the diagnosis and management of which is challenging. We present a large case series of invasive sino-orbital fungal granulomas in patients without pre-existing systemic immunocomprimising condition.DesignRetrospective case series.ParticipantsTwenty cases of invasive sino-orbital/naso-orbital fungal granulomas in immunocompetent individuals.MethodsWe retrospectively analyzed all patients with orbital fungal granuloma who were treated at a tertiary referral eye center in South India between January 2005 and December 2012. Histopathologic confirmation of tissue invasion by fungal elements and presence of granulomatous inflammation was established in all cases included in the study.Main outcome measuresRelief of patient symptoms, resolution/no progression of disease on orbital imaging, ocular, and vision salvage were the treatment outcomes studied.ResultsTwenty patients (11 male and 9 female) were studied. Mean age of patients was 47.4 years ranging from 24-65 years. Aspergillus was the causative fungus in 18 cases while 2 were cases of mucormycosis. Surgical debulking of the orbital disease was performed in 7 patients. Exenteration was performed in 2 patients to prevent spread to the CNS. Medical therapy consisted of oral itraconazole in all patients and intravenous amphotericin B was administered in 2 patients. Average duration of medical therapy required to achieve relief from symptoms was 6-8 months. Recurrences are common and long-term follow-up is essential.ConclusionsOrbital fungal infections are challenging in terms of both diagnosis and treatment. Debulking along with prolonged antifungal therapy seems to be effective in controlling the infection.
| S-EPMC6707179 | biostudies-literature
Project description:A 60-year-old patient with aplastic anemia presented with vesicular varicella-like skin lesions on her face, arms, legs, back, and abdomen. However, diagnosis for herpetic infection was negative. Findings of a skin biopsy led to a tentative histologic diagnosis of toxoplasmosis, and infection with Toxoplasma gondii was confirmed by immunohistochemistry and PCR. Cutaneous toxoplasmosis is a rare finding in immunocompromised patients and might mimic other infectious diseases, and vesicular lesions associated with toxoplasmosis have not been reported previously.
| S-EPMC3666818 | biostudies-literature
Project description:In acquired immune aplastic anemia (AA), pathogenic cytotoxic Th1 cells are activated and expanded, driving an immune response against the hematopoietic stem and progenitor cells (HSPCs) that provokes cell depletion and causes bone marrow failure. However, additional HSPC defects may contribute to hematopoietic failure, reflecting on disease outcomes and response to immunosuppression. Here we derived induced pluripotent stem cells (iPSCs) from peripheral blood (PB) erythroblasts obtained from patients diagnosed with immune AA using non-integrating plasmids to model the disease. Erythroblasts were harvested after hematologic response to immunosuppression was achieved. Patients were screened for germline pathogenic variants in bone marrow failure-related genes and no variant was identified. Reprogramming was equally successful for erythroblasts collected from the three immune AA patients and the three healthy subjects. However, the hematopoietic differentiation potential of AA-iPSCs was significantly reduced both quantitatively and qualitatively as compared to healthy-iPSCs, reliably recapitulating disease: differentiation appeared to be more severely affected in cells from the two patients with partial response as compared to the one patient with complete response. Telomere elongation and the telomerase machinery were preserved during reprogramming and differentiation in all AA-iPSCs. Our results indicate that iPSCs are a reliable platform to model immune AA and recapitulate clinical phenotypes. We propose that the immune attack may cause specific epigenetic changes in the HSPCs that limit adequate proliferation and differentiation.
| S-EPMC9051057 | biostudies-literature
Project description:Case summaryThis report describes an indoor-only cat with a rare form of sino-orbital aspergillosis (SOA) with cervical lymphadenopathy causing local obstruction. Extensive work-up on initial presentation failed to identify the underlying etiology and the diagnosis was not determined until the disease progressed during a prolonged course of glucocorticoid therapy.Relevance and novel informationSOA caused by Aspergillus viridinutans complex is increasingly recognized as a significant cause of mortality in cats in recent years, with most cases reported in Australia, Europe and Asia. Feline SOA carries a poor prognosis owing to its invasive nature and resistance to antifungal therapy. This case demonstrates the importance of clinical awareness of SOA as a differential for cats with chronic nasal signs and exophthalmos in the USA. Moreover, it demonstrates a rare form of presentation and potential difficulty in achieving a correct diagnosis.
| S-EPMC10331201 | biostudies-literature
Project description:Acquired aplastic anemia (AA) is a life-threatening bone marrow failure caused by an autoimmune cytotoxic T lymphocyte attack on hematopoietic stem and progenitor cells. Factors contributing to aberrant autoimmune activation in AA include a deficit of T regulatory cells and high levels of inflammatory cytokines. Several acquired conditions of immune dysregulation and genetic polymorphisms in inflammatory cytokines and human leukocyte antigen genes have been linked to an increased risk of AA. However, AA has not been reported in patients with Mendelian disorders of immune regulation. Here we report a patient with familial common variable immunodeficiency (CVID) caused by a pathogenic variant in NFKB1, who developed AA as an adult. The patient had a difficult clinical course and was unable to tolerate standard AA therapy with cyclosporine A and eltrombopag, with complications attributed in part to the effect of cyclosporine A on NF-κB signaling. Our case suggests a novel link between genetic disorders of immune regulation and AA and highlights the importance of recognizing inherited autoimmunity syndromes in AA patients for the selection of optimal therapy and prognostic counseling.
| S-EPMC7784489 | biostudies-literature
Project description:We report a case of a 51-year old man with a severe aplastic anemia who developed an invasive trichosporonosis to Trichosporon faecale with fungemia and skin lesions during severe neutropenia. The treatment was successful before neutrophil recovery with a combination of voriconazole and liposomal amphotericin B.
| S-EPMC4506992 | biostudies-literature
Project description: Not available
| S-EPMC7369588 | biostudies-literature
Project description:Quality of response to immunosuppressive therapy and long-term outcomes for pediatric severe aplastic anemia remain incompletely characterized. Contemporary evidence to inform treatment of relapsed or refractory severe aplastic anemia for pediatric patients is also limited. The clinical features and outcomes for 314 children treated from 2002 to 2014 with immunosuppressive therapy for acquired severe aplastic anemia were analyzed retrospectively from 25 institutions in the North American Pediatric Aplastic Anemia Consortium. The majority of subjects (n=264) received horse anti-thymocyte globulin (hATG) plus cyclosporine (CyA) with a median 61 months follow up. Following hATG/CyA, 71.2% (95%CI: 65.3,76.6) achieved an objective response. In contrast to adult studies, the quality of response achieved in pediatric patients was high, with 59.8% (95%CI: 53.7,65.8) complete response and 68.2% (95%CI: 62.2,73.8) achieving at least a very good partial response with a platelet count ≥50×109L. At five years post-hATG/CyA, overall survival was 93% (95%CI: 89,96), but event-free survival without subsequent treatment was only 64% (95%CI: 57,69) without a plateau. Twelve of 171 evaluable patients (7%) acquired clonal abnormalities after diagnosis after a median 25.2 months (range: 4.3-71 months) post treatment. Myelodysplastic syndrome or leukemia developed in 6 of 314 (1.9%). For relapsed/refractory disease, treatment with a hematopoietic stem cell transplant had a superior event-free survival compared to second immunosuppressive therapy treatment in a multivariate analysis (HR=0.19, 95%CI: 0.08,0.47; P=0.0003). This study highlights the need for improved therapies to achieve sustained high-quality remission for children with severe aplastic anemia.
| S-EPMC6886407 | biostudies-literature
Project description:Clonal hematopoiesis was investigated in patients with aplastic anemia using next-generation sequencing and single-nucleotide polymorphism (SNP) array-based karyotyping.
| EGAS00001001153 | EGA