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

• Published in: PloS one Vol. 11; no. 4; p. e0153335
• Main Authors: El-Mallawany, Nader Kim, Kamiyango, William, Slone, Jeremy S, Villiera, Jimmy, Kovarik, Carrie L, Cox, Carrie M, Dittmer, Dirk P, Ahmed, Saeed, Schutze, Gordon E, Scheurer, Michael E, Kazembe, Peter N, Mehta, Parth S
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 15.04.2016; Public Library of Science (PLoS)
• Subjects: Acquired immune deficiency syndrome; Adolescent; Adolescents; Adults; AIDS; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Antiretroviral drugs; Antiretroviral Therapy, Highly Active; Biology and Life Sciences; Bleomycin; Bleomycin - administration & dosage; Cancer therapies; CD4 antigen; Chemotherapy; Child; Child, Preschool; Children; Children & youth; Clinical trials; Complications and side effects; Cytokines; Dosage and administration; Drug therapy; Edema; Failure analysis; Female; Females; Hematology; Hemoglobin; Highly active antiretroviral therapy; HIV; HIV infections; HIV Infections - complications; HIV Infections - diagnosis; HIV Infections - drug therapy; HIV Infections - epidemiology; HIV-1; Human immunodeficiency virus; Humans; Immunology; Infant; Infections; Infectious diseases; Kaposi's sarcoma; Kaposis sarcoma; Lesions; Lymph; Lymph nodes; Lymphadenopathy; Male; Malignancy; Medical records; Medicine; Medicine and Health Sciences; Nevirapine; Nodules; Observational studies; Oncology; Pathology; Pediatrics; Physicians; Prognosis; Remission; Remission Induction; Retrospective Studies; Risk analysis; Risk factors; Sarcoma; Sarcoma, Kaposi - diagnosis; Sarcoma, Kaposi - drug therapy; Sarcoma, Kaposi - epidemiology; Skin diseases; Society; Socioeconomic Factors; Teenagers; Time Factors; Vincristine; Vincristine - administration & dosage; United States
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0153335

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.