Rapidly progressive Kaposi’s Sarcoma in an Iraqi boy received Valproic acid: a case report and review of literature

Abstract

BackgroundKaposi’s sarcoma (KS), an endothelial neoplasm, is associated with human herpes virus (HHV) -8 infection. KS has four clinical sub-types: Mediterranean/classic, African/endemic, human immunodeficiency virus (HIV) -associated/epidemic, and transplantation-related/iatrogenic. Immunosuppression is an important cofactor in KS process. Classic KS (CKS) is exceedingly rare in children and when occurs, it is much more disseminated than adults. The epidemic, HIV-associated and the iatrogenic forms of childhood KS are a result of a profound and acquired T-cell deficiency. To our knowledge, this is the first paediatric KS case report from Iraq. Our patient was showing an unusual aggressive course of the disease while receiving Valproic acid (VPA) of the potential immune-suppressive effect.Case presentationA six-year-old Iraqi boy, who had cerebral palsy (CP) and epilepsy since the age of 9-months, had received VPA to control his seizures. He developed skin discoloration followed by nodules that disseminated proximally from the lower extremities to the groin, face, ears and oral cavity, and then he died from severe respiratory distress after 110 days from the disease evolution. KS diagnosis was proved by a skin biopsy. As the patient was of Arab-Asian ethnicity and was HIV-seronegative status, accordingly, his condition best fitted the classic form of KS. However, recent studies showed the link of VPA with the reactivation of HHV-8. Moreover, accumulated experimental and clinical data elucidated that VPA induces T-cell suppression. Given that there was a lack of facilities to perform the laboratory immunological diagnostic tests in Iraq, the VPA-induced effect on immunity in our case (iatrogenic KS) could not be evaluated.ConclusionsOur report demonstrates a rare, rapidly progressing paediatric KS case and highlights the possible role of the 5-years’ administration of VPA and its challenging effect on cellular immunity based on recent studies. Thus, VPA could have promoted the development of the KS in our patient. This report also recalls the need of paediatricians to consider KS especially when the skin lesion appears at the child’s foot even in countries outside the geographical map of the disease.Electronic supplementary materialThe online version of this article (doi:10.1186/s12887-016-0653-3) contains supplementary material, which is available to authorized users.

Asymmetrically enlarged parietal foramina in a rare case of Goldenhar syndrome with a possible etiopathogenesis

“…The Eastern province had reported the second highest frequency of birth defects, including GS next to the capital Al-Riyadh, together they accounted for about half of cases of birth defects in the country [ 14 ]. Taken together, the Eastern province is close to Basra; a city in the South of Iraq, which was heavily bombarded with depleted uranium (DU) since the first Gulf War (GW) 1991 [ 15 ]. Knowing that DU has a radioactive half-life measured in billions of years and toxic effects through air, water, and soil contamination, thus, spreading environmental pollution is an inevitable consequence.…”

Towards Better Understanding of KSHV Life Cycle: from Transcription and Posttranscriptional Regulations to Pathogenesis

“…Various host and experimental factors trigger virus reactivation from latently infected cells. To investigate the mechanism underlying the switch between the latent and reactivation states, several chemical reagents, including 12-O-tetradecanoyl-phorbol-13-acetate (TPA) (Miller et al 1997), sodium butyrate, valproic acid, ionomycin, and 5-azacytidine, have been widely used (Al-Kzayer et al 2016; Lukac et al 1999b; Shin et al 2014). While KSHV infection is necessary for the development of KSHV-associated diseases, it is not sufficient to induce tumorigenesis (Andrei and Snoeck 2015; Ganem 2010).…”

Perfil clínico e epidemiológico das alterações bucais em portadores do HIV

“…Pesquisadores tem observado uma redução da prevalência de manifestações bucais por infecções oportunistas com início da terapia antirretroviral combinada 9,10 . Esse dado vantajoso tem ocorrido devido a uma recuperação parcial das funções do sistema imunológico após supressão da viremia e redução da destruição celular causada pelo HIV, além de um possível efeito antiapoptose de algumas drogas 9 .…”