Unusual Manifestation of Extraosseous Ewing Sarcoma: Report of 3 Cases.

Ewing sarcoma (ES), described as a diffuse endothelioma of the bone, is divided into two categories: osseous and extraosseous, which mainly affects adolescents. Extraosseous Ewing Sarcomas (EES) are rare tumors originating from soft tissues. Their clinical presentation depends mainly on the primary location of the tumor and are highly chemosensitive and radiosensitive. The purpose of this study was to describe the clinical characteristics and outcomes of 3 children with EES and uncommon presentation treated in our Unit. The diagnosis of EES was confirmed by biopsy and cytogenetic analysis with fluorescence in situ hybridization (FISH). Surgical excision was planned as primary treatment, followed by adjuvant chemotherapy according to EURO-E.W.I.N.G protocol. To date, all patients are alive, 1, 3 and 4 years after completion of treatment, with no signs of recurrence or metastasis.

HIV-1: towards understanding the nature and quantifying the latent reservoir.

The human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) pandemic constitutes one of the greatest public health issues, since 36.9 million people worldwide were living with HIV in 2017 and 940,000 died from AIDS- related illnesses in the same year. One of the main obstacles in the effort to achieve viral eradication or long-term virologic remission is the existence of the HIV-reservoir. Except for resting memory CD4+ T cells there is a plethora of innate immunity cells including macrophages, dendritic cells, follicular T helper cells and NK cells which are now considered to play a role in viral latency and persistence. Hematopoietic precursor cells and progenitor mast cells, astrocytes, fibrocytes, renal and liver epithelial cells could also contribute to the reservoir, but their role remains controversial. Tissue reservoirs, such as the central nervous system (CNS), lymphoid tissue, adipose tissue and the gut-associated-lymphoid-tissue (GALT) are usually referred to as anatomic sanctuaries, where it is difficult to achieve high concentration and efficacy of antiretroviral agents. Accurate quantification of this reservoir is of the utmost importance and multiple assays have been developed for this purpose. The role of several cell populations in viral latency needs to be clarified by further studies. Furthermore, there is an urgent need for new assays, which will accurately measure the size of the reservoir, which plays a key role in predicting the timing of viral rebound upon cessation of antiretroviral treatment, since the currently available ones either overestimate or underestimate the size and have significant limitations. Keywords: HIV-1; cellular reservoirs; tissue reservoirs; quantification.

Contributions of the superoxide dismutase 1 and zinc finger protein 469 (ZNF469) genes to keratoconus.

Keratoconus (KC) is a multifactorial, progressive, degenerative corneal disorder with an incidence of approximately 1 in 2,000 subjects.

Lipid profile of children with acute lymphoblastic leukemia during L-asparaginase treatment.

Background: L-asparaginase is valuable in treating pediatric acute lymphoblastic leukemia (ALL), yet its use has been associated with lipid profile disturbances. Methods: We compared the lipid profile [high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, total cholesterol, triglycerides, apolipoprotein-α1 (Apo-Α1), apolipoprotein-B100 (Αpo-B100), lipoprotein-α (Lp-α), glucose, amylase, and lipase] between newly diagnosed ALL patients, ALL survivors, and healthy controls. We also assessed alterations of the parameters mentioned earlier during induction and consolidation treatment. Results: We recorded significant differences in the lipid profile at diagnosis of children with ALL compared to controls (HDL cholesterol, triglycerides, Apo-A1, and Apo-B100 levels). HDL cholesterol, total cholesterol, and Apo-Α1 levels increased significantly during induction at most time points. Levels of Αpo-B100, triglycerides, and Lp-α exhibited a downward trend. During re-induction, no change was observed. During the treatment of high-risk patients, we found no statistically significant difference for any of the examined variables. Conclusion: To confirm our preliminary results, the role of the administration of L-asparaginase and other medications in the variations in the lipid profile at diagnosis of children with ALL needs to be further elucidated with larger multicentre studies, including more patients from diverse ethnic backgrounds. HIPPOKRATIA 2023, 27 (2):41-47.