Proposal of a Risk-Stratification Platform to Address Distinct Clinical Features of Pediatric Kaposi Sarcoma in Lilongwe, Malawi

El‐Mallawany, Nader Kim; Kamiyango, William; Villiera, Jimmy; Slone, Jeremy S.; Kovarik, Carrie L.; Campbell, Liane R.; Agrawal, Anurag K.; Dittmer, Dirk P.; Eason, Anthony; Ahmed, Saeed; Schutze, Gordon E.; Scheurer, Michael E.; Kazembe, Peter N.; Mehta, Parth

doi:10.1200/jgo.17.00054

Cited by 16 publications

(57 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Clinical presentation with severe cytopenias is unique and relatively common in childhood KS . Statistically significant associations between clinical presentation with severe thrombocytopenia and elevated KSHV VL and IL‐6 levels were not demonstrated, potentially limited by the small sample size.…”

Section: Discussionmentioning

confidence: 99%

“…The Lilongwe Pediatric KS Staging Classification attempts to stratify four distinct groups with different responses to therapy—mild/moderate cutaneous/oral disease (stage 1), lymphadenopathic KS (stage 2), woody edema (stage 3) and visceral and/or disseminated cutaneous/oral disease (stage 4) . While the limited numbers of patients broken down by stage preclude definitive conclusions regarding associations with KSHV VL, we observed a distinct pattern with all 12 of the patients with detectable KSHV VL categorized as Stages 2 or 4, and 5/6 patients with undetectable KSHV VL being categorized as Stages 1 or 3.…”

Section: Discussionmentioning

confidence: 99%

“…Clinical information was collected using standardized institutional clinical pathways that have been previously described . All patients were treated with chemotherapy plus combination antiretroviral therapy according to Malawian national guidelines . The AIDS Clinical Trial Group TIS staging criteria and Lilongwe Pediatric KS Staging Classification were applied based on established guidelines .…”

Section: Methodsmentioning

confidence: 99%

“…5,6 All patients were treated with chemotherapy plus combination antiretroviral therapy according to Malawian national guidelines. 5,6 The AIDS Clinical Trial Group TIS staging criteria and Lilongwe Pediatric KS Staging Classification were applied based on established guidelines. 6,21,22 The Lilongwe Pediatric KS Staging Classification stratifies four distinct clinical categories based on clinical presentation to address the spectrum of clinical phenotypes specifically seen in childhood KS in KSHV-endemic regions.…”

Section: Methodsmentioning

confidence: 99%

“…Whereas adult Kaposi sarcoma (KS) has been intensely studied since its classification as an AIDS defining malignancy, pediatric KS with or without HIV coinfection has been a neglected disease entity. Although contemporary descriptions of pediatric KS have shed light on the unique clinical features of disease in children, the virologic and biological underpinnings have yet to be explored in children . Caused by KS‐associated herpesvirus (KSHV), also known as human herpesvirus‐8, KS is currently among the most common childhood malignancies in KSHV‐endemic regions including central, eastern and southern Africa .…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

KSHV viral load and Interleukin‐6 in HIV‐associated pediatric Kaposi sarcoma—Exploring the role of lytic activation in driving the unique clinical features seen in endemic regions

El‐Mallawany

Mehta

Kamiyango³

et al. 2018

Intl Journal of Cancer

Self Cite

View full text Add to dashboard Cite

Kaposi sarcoma (KS) is among the most common childhood malignancies in central, eastern, and southern Africa. Although its unique clinical features have been established, biological mechanisms related to the causative agent, KS-associated herpes-virus (KSHV), have yet to be explored in children. We performed a prospective observational pilot study to explore associations between KSHV viral load (VL), human interleukin-6 (IL-6) and IL-10 levels, and clinical characteristics of 25 children with KS in Lilongwe, Malawi from June 2013-August 2015. The median age was 6.4 years. Lymphadenopathy was the most common site of KS involvement (64%), followed by skin and oral mucosa (44% each), woody edema (12%), and pulmonary (8%). Baseline samples for plasma KSHV VL, IL-6 and IL-10 analyses were available for 18/25 patients (72%) at time of KS diagnosis. KSHV VL was detectable at baseline in 12/18 (67%) patients, the median baseline IL-6 level was 8.53 pg/mL (range 4.31-28.33), and the median baseline IL-10 level was 19.53 pg/mL (range 6.91-419.69). Seven (39%) patients presented with an IL-6 level > 10 pg/mL (exceeding twice the upper limit of normal). Detectable KSHV VL was significantly associated with lymphadenopathic KS (p = 0.004), while having undetectable KSHV VL was associated with a higher likelihood of presenting with hyperpigmented skin lesions (p = 0.01). Detectable KSHV VL and elevated IL-6 levels are present in a subset of children with KS. Lytic activation of KSHV and associated elevation in KSHV VL may contribute to the unique clinical manifestations of pediatric KS in KSHV-endemic regions of Africa.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%