title: Rapidly progressive Kaposi's Sarcoma in an Iraqi boy received Valproic acid: a case report and review of literature.
abstract: Kaposi'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.
  
  
  
  A 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.
  
  
  
  Our 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.
authors.affiliation: Department of Paediatrics, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto, Nagano, 390-8621, Japan. alkzayerlikaa@yahoo.co.uk.
authors.forename: Lika'a Fasih Y
authors.initials: LF
authors.lastname: Al-Kzayer
authors.affiliation: Department of Emergency Medicine, Dr. G. B. Cross Memorial Hospital, Eastern Health, Clarenville, Newfoundland and Labrador, Canada.
authors.forename: Peter
authors.initials: P
authors.lastname: Keizer
authors.affiliation: Department of Laboratory, Al-Hamdaniya General Hospital, Mosul, Iraq.
authors.forename: Farah T
authors.initials: FT
authors.lastname: Abdulraheem
authors.affiliation: Department of Laboratory Medicine, Shinshu University Hospital, Matsumoto, Nagano, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Sano
authors.affiliation: Japan Chernobyl Foundation (JCF) NPO, Matsumoto, Nagano, Japan.
authors.forename: Minoru
authors.initials: M
authors.lastname: Kamata
authors.affiliation: Department of Paediatrics, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto, Nagano, 390-8621, Japan.|Nagano Children's Hospital, Hematology/Oncology, Matsumoto, Nagano, Japan.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Sakashita
authors.affiliation: Department of Orthopaedic Surgery, Al-Hamdaniya General Hospital, Mosul, Iraq.
authors.forename: Laith A Y
authors.initials: LA
authors.lastname: Habbaba
authors.affiliation: Department of Paediatrics, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto, Nagano, 390-8621, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
mesh_terms: D000927:Anticonvulsants / Q000009:adverse effects*; D002648:Child; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects*; D007493:Iraq; D008297:Male; D012514:Sarcoma, Kaposi / Q000139:chemically induced* / Q000175:diagnosis / Q000276:immunology; D012878:Skin Neoplasms / Q000139:chemically induced* / Q000175:diagnosis / Q000276:immunology; D014635:Valproic Acid / Q000009:adverse effects*
chemical_list: D000927:Anticonvulsants; D007166:Immunosuppressive Agents; D014635:Valproic Acid
keywords: Cerebral palsy (CP); Classic Kaposi sarcoma (CKS); Human herpesvirus-8 (HHV-8); Kaposi’s sarcoma (KS); Valproic acid (VPA)