title: Clinical Factors Associated with Long-Term Complete Remission versus Poor Response to Chemotherapy in HIV-Infected Children and Adolescents with Kaposi Sarcoma Receiving Bleomycin and Vincristine: A Retrospective Observational Study.
abstract: Kaposi sarcoma (KS) is the most common HIV-associated malignancy in children and adolescents in Africa. Pediatric KS is distinct from adult disease. We evaluated the clinical characteristics associated with long-term outcomes. We performed a retrospective observational analysis of 70 HIV-infected children and adolescents with KS less than 18 years of age diagnosed between 8/2010 and 6/2013 in Lilongwe, Malawi. Local first-line treatment included bleomycin and vincristine plus nevirapine-based highly active anti-retroviral therapy (HAART). Median age was 8.6 years (range 1.7-17.9); there were 35 females (50%). Most common sites of presentation were: lymph node (74%), skin (59%), subcutaneous nodules (33%), oral (27%), woody edema (24%), and visceral (16%). Eighteen (26%) presented with lymphadenopathy only. Severe CD4 suppression occurred in 28%. At time of KS diagnosis, 49% were already on HAART. Overall, 28% presented with a platelet count < 100 x 109/L and 37% with hemoglobin < 8 g/dL. The 2-year event-free (EFS) and overall survival (OS) were 46% and 58% respectively (median follow-up 29 months, range 15-50). Multivariable analysis of risk of death and failure to achieve EFS demonstrated that visceral disease (odds ratios [OR] 19.08 and 11.61, 95% CI 2.22-163.90 and 1.60-83.95 respectively) and presenting with more than 20 skin/oral lesions (OR 9.57 and 22.90, 95% CI 1.01-90.99 and 1.00-524.13 respectively) were independent risk factors for both. Woody edema was associated with failure to achieve EFS (OR 7.80, 95% CI 1.84-33.08) but not death. Univariable analysis revealed that lymph node involvement was favorable for EFS (OR 0.28, 95% CI 0.08-0.99), while T1 TIS staging criteria, presence of cytopenias, and severe immune suppression were not associated with increased mortality. Long-term complete remission is achievable in pediatric KS, however outcomes vary according to clinical presentation. Based on clinical heterogeneity, treatment according to risk-stratification is necessary to improve overall outcomes.
authors.affiliation: Department of Pediatrics, Division of Hematology, Oncology, and Hematopoietic Stem Cell Transplantation, New York Medical College, Valhalla, New York, United States of America.|Department of Pediatrics, Section of Hematology and Oncology, Baylor College of Medicine, Houston, Texas, United States of America.
authors.forename: Nader Kim
authors.initials: NK
authors.lastname: El-Mallawany
authors.affiliation: Baylor College of Medicine Children's Foundation Malawi, Lilongwe, Malawi.
authors.forename: William
authors.initials: W
authors.lastname: Kamiyango
authors.affiliation: Department of Pediatrics, Section of Hematology and Oncology, Baylor College of Medicine, Houston, Texas, United States of America.|Texas Children's Cancer and Hematology Centers, Houston, Texas, United States of America.
authors.forename: Jeremy S
authors.initials: JS
authors.lastname: Slone
authors.affiliation: Baylor College of Medicine Children's Foundation Malawi, Lilongwe, Malawi.
authors.forename: Jimmy
authors.initials: J
authors.lastname: Villiera
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
authors.forename: Carrie L
authors.initials: CL
authors.lastname: Kovarik
authors.affiliation: Department of Pediatrics, Loyola University Chicago, Stritch School of Medicine, Maywood, Illinois, United States of America.
authors.forename: Carrie M
authors.initials: CM
authors.lastname: Cox
authors.affiliation: Department of Microbiology and Immunology, Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, North Carolina, United States of America.
authors.forename: Dirk P
authors.initials: DP
authors.lastname: Dittmer
authors.affiliation: Baylor College of Medicine Children's Foundation Malawi, Lilongwe, Malawi.|Department of Pediatrics, Baylor College of Medicine, Houston, Texas, United States of America.
authors.forename: Saeed
authors.initials: S
authors.lastname: Ahmed
authors.affiliation: Department of Pediatrics, Baylor College of Medicine, Houston, Texas, United States of America.
authors.forename: Gordon E
authors.initials: GE
authors.lastname: Schutze
authors.affiliation: Department of Pediatrics, Section of Hematology and Oncology, Baylor College of Medicine, Houston, Texas, United States of America.|Texas Children's Cancer and Hematology Centers, Houston, Texas, United States of America.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Scheurer
authors.affiliation: Baylor College of Medicine Children's Foundation Malawi, Lilongwe, Malawi.
authors.forename: Peter N
authors.initials: PN
authors.lastname: Kazembe
authors.affiliation: Department of Pediatrics, Section of Hematology and Oncology, Baylor College of Medicine, Houston, Texas, United States of America.|Texas Children's Cancer and Hematology Centers, Houston, Texas, United States of America.
authors.forename: Parth S
authors.initials: PS
authors.lastname: Mehta
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D023241:Antiretroviral Therapy, Highly Active; D001761:Bleomycin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D005260:Female; D015658:HIV Infections / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D015497:HIV-1; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D012074:Remission Induction; D012189:Retrospective Studies; D012514:Sarcoma, Kaposi / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D012959:Socioeconomic Factors; D013997:Time Factors; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D001761:Bleomycin; D014750:Vincristine