title: Characteristics and survival for HIV-associated multicentric Castleman disease in Malawi.
abstract: BACKGROUND
  Clinical reports of multicentric Castleman disease (MCD) from sub-Saharan Africa (SSA) are scarce despite high prevalence of HIV and Kaposi sarcoma-associated herpesvirus (KSHV). Our objective is to describe characteristics and survival for HIV-associated MCD patients in Malawi. To our knowledge, this is the first HIV-associated MCD case series from the region.
  
  
  METHODS
  We describe HIV-positive patients with MCD in Lilongwe, and compare them to HIV-associated lymph node Kaposi sarcoma (KS) and non-Hodgkin lymphoma (NHL) patients treated at our centre. All patients were enrolled into a prospective longitudinal cohort study at a national teaching hospital and cancer referral centre serving half of Malawi's 16 million people. We included adult patients≥18 years of age with HIV-associated MCD (n=6), lymph node KS (n=5) or NHL (n=31) enrolled between 1 June 2013 and 31 January 2015.
  
  
  CONCLUSIONS
  MCD patients had a median age of 42.4 years (range 37.2-51.8). All had diffuse lymphadenopathy and five had hepatosplenomegaly. Concurrent KS was present for one MCD patient, and four had performance status ≥3. MCD patients had lower median haemoglobin (6.4 g/dL, range 3.6-9.3) than KS (11.0 g/dL, range 9.1-12.0, p=0.011) or NHL (11.2 g/dL, range 4.5-15.1, p=0.0007). Median serum albumin was also lower for MCD (2.1 g/dL, range 1.7-3.2) than KS (3.7 g/dL, range 3.2-3.9, p=0.013) or NHL (3.4 g/dL, range 1.8-4.8, p=0.003). All six MCD patients were on antiretroviral therapy (ART) with median CD4 count 208 cells/µL (range 108-1146), and all with HIV RNA <400 copies/mL. Most KS and NHL patients were also on ART, although ART duration was longer for MCD (56.4 months, range 18.2-105.3) than KS (14.2 months, range 6.8-21.9, p=0.039) or NHL (13.8 months, range 0.2-98.8, p=0.017). Survival was poorer for MCD patients than lymph node KS or NHL.
  
  
  CONCLUSIONS
  HIV-associated MCD occurs in Malawi, is diagnosed late and is associated with high mortality. Improvements in awareness, diagnostic facilities, treatment and supportive care are needed to address this likely under-recognized public health problem in SSA.
authors.affiliation: UNC Project-Malawi, Lilongwe, Malawi.|Program in Global Oncology, Lineberger Comprehensive Cancer Center, Chapel Hill, NC, USA.|Department of Medicine, Institute for Global Health and Infectious Diseases, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.|Department of Medicine, University of Malawi College of Medicine, Blantyre, Malawi; gopal@med.unc.edu.
authors.forename: Satish
authors.initials: S
authors.lastname: Gopal
authors.affiliation: UNC Project-Malawi, Lilongwe, Malawi.
authors.forename: N George
authors.initials: NG
authors.lastname: Liomba
authors.affiliation: Division of Hematopathology, Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
authors.forename: Nathan D
authors.initials: ND
authors.lastname: Montgomery
authors.affiliation: UNC Project-Malawi, Lilongwe, Malawi.|Department of Medicine, University of Malawi College of Medicine, Blantyre, Malawi.
authors.forename: Agnes
authors.initials: A
authors.lastname: Moses
authors.affiliation: UNC Project-Malawi, Lilongwe, Malawi.
authors.forename: Bongani
authors.initials: B
authors.lastname: Kaimila
authors.affiliation: Kamuzu Central Hospital, Lilongwe, Malawi.
authors.forename: Richard
authors.initials: R
authors.lastname: Nyasosela
authors.affiliation: UNC Project-Malawi, Lilongwe, Malawi.
authors.forename: Maria
authors.initials: M
authors.lastname: Chikasema
authors.affiliation: United Nations Development Program, Lilongwe, Malawi.
authors.forename: Bal M
authors.initials: BM
authors.lastname: Dhungel
authors.affiliation: UNC Project-Malawi, Lilongwe, Malawi.
authors.forename: Coxcilly
authors.initials: C
authors.lastname: Kampani
authors.affiliation: Department of Microbiology and Immunology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
authors.forename: Marcia K
authors.initials: MK
authors.lastname: Sanders
authors.affiliation: UNC Project-Malawi, Lilongwe, Malawi.
authors.forename: Robert
authors.initials: R
authors.lastname: Krysiak
authors.affiliation: Program in Global Oncology, Lineberger Comprehensive Cancer Center, Chapel Hill, NC, USA.|Department of Medicine, Institute for Global Health and Infectious Diseases, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.|Department of Microbiology and Immunology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
authors.forename: Dirk P
authors.initials: DP
authors.lastname: Dittmer
authors.affiliation: Program in Global Oncology, Lineberger Comprehensive Cancer Center, Chapel Hill, NC, USA.|Division of Hematopathology, Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
authors.forename: Yuri
authors.initials: Y
authors.lastname: Fedoriw
mesh_terms: D000328:Adult; D018791:CD4 Lymphocyte Count; D005871:Castleman Disease / Q000150:complications / Q000401:mortality*; D015331:Cohort Studies; D005260:Female; D015658:HIV Infections / Q000150:complications*; D006801:Humans; D008295:Malawi; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012514:Sarcoma, Kaposi / Q000401:mortality
keywords: HIV; Kaposi sarcoma; Kaposi sarcoma-associated herpesvirus; multicentric Castleman disease; non-Hodgkin lymphoma; sub-Saharan Africa