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.

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PloS one, ISSN: 1932-6203, Vol: 11, Issue: 4, Page: e0153335

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10.1371/journal.pone.0153335; 10.1371/journal.pone.0153335.t001; 10.1371/journal.pone.0153335.g003; 10.1371/journal.pone.0153335.g001; 10.1371/journal.pone.0153335.t003; 10.1371/journal.pone.0153335.g002; 10.1371/journal.pone.0153335.t002
PMC4833299; 4833299
Nader Kim El-Mallawany; William Kamiyango; Jeremy S. Slone; Jimmy Villiera; Carrie L. Kovarik; Carrie M. Cox; Dirk P. Dittmer; Saeed Ahmed; Gordon E. Schutze; Michael E. Scheurer; Peter N. Kazembe; Parth S. Mehta; Shou-Jiang Gao Show More Hide
Public Library of Science (PLoS); Touro Scholar; Figshare
Biochemistry, Genetics and Molecular Biology; Agricultural and Biological Sciences; Medicine; Adolescent; Antineoplastic Combined Chemotherapy Protocols; Antiretroviral Therapy; Highly Active; Bleomycin; Child; Preschool; Female; HIV Infections; HIV-1; Humans; Infant; Male; Prognosis; Remission Induction; Retrospective Studies; Sarcoma; Kaposi; Socioeconomic Factors; Time Factors; Vincristine; Antiretroviral Therapy, Highly Active; Child, Preschool; Sarcoma, Kaposi; Hematology; Oncology; Pediatrics; Biotechnology; Immunology; Cancer; 110309 Infectious Diseases; 18 years; Retrospective Observational Study Kaposi sarcoma; presentation; risk factors; HAART; Poor Response; Median age; Pediatric KS; T 1 TIS; EFS; Kaposi Sarcoma; Clinical Factors Associated; Univariable analysis; lymph node involvement; adolescent; OS; KS diagnosis; Severe CD 4 suppression; adult disease; CI; odds ratios; failure; outcome; Woody edema; platelet count; 8.6 years
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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.