Abstract

IntroductionClinical 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.MethodsWe 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.Results and discussionMCD 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.ConclusionsHIV-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.

Highlights

  • 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)

  • HIV-associated multicentric Castleman disease (MCD) is a lymphoproliferative disorder caused by Kaposi sarcomaassociated herpesvirus (KSHV) [1Á5]

  • Unlike Kaposi sarcoma (KS), MCD incidence may be increasing in the antiretroviral therapy (ART) era [6]

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Summary

Introduction

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. We included adult patients ]18 years of age with HIV-associated MCD (n 06), lymph node KS (n05) or NHL (n 031) enrolled between 1 June 2013 and 31 January 2015. MCD patients had lower median haemoglobin (6.4 g/dL, range 3.6Á9.3) than KS (11.0 g/dL, range 9.1Á12.0, p00.011) or NHL (11.2 g/dL, range 4.5Á15.1, p 00.0007). All six MCD patients were on antiretroviral therapy (ART) with median CD4 count 208 cells/mL (range 108Á1146), and all with HIV RNA B400 copies/mL. HIV-associated multicentric Castleman disease (MCD) is a lymphoproliferative disorder caused by Kaposi sarcomaassociated herpesvirus (KSHV) [1Á5]. Patients can be critically ill, and often died within two years until recent therapies became available

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