Contents lists available at ScienceDirect Cancer Epidemiology journal homepage: www.elsevier.com/locate/canep Incidence of haematological malignancies, Eastern Cape Province; South Africa, 2004–2013 Diana Oelofse a,b, ⁎ , Ilse Truter a,c a Department of Pharmacy, Nelson Mandela University, PO Box 77000, Port Elizabeth, 6031, South Africa b National Health Laboratory Service, Haematopathology, PO Box 467, Port Elizabeth, 6000, South Africa c Drug Utilization Research Unit (DURU), South Africa ARTICLE INFO Keywords: Haematological malignancies Incidence South Africa Urbanisation Leukaemia Rare cancer ABSTRACT Background: The incidence of haematological malignancies in Africa’s rapidly urbanising populations is in- sufficiently explored. Reliable population-based cancer statistics, however, continues to be a scarce resource in Africa and tends to be urban biased with limited rural coverage. In addition, many haematological malignancies are regarded as rare cancers, a sub-group that often affects the young disproportionately and require advanced diagnostic services and facilities able to deliver costly sophisticated treatments. This study provides a first at- tempt to estimate the incidence of haematological malignancies among the Eastern Cape Province population of South Africa. Method: Multiple public- and private sector data archives and resources were utilised to optimise the identifi- cation of incident cases, including clinical records; bone marrow; cytology; histology; flow cytometry and cy- togenetic records. Crude incidence, age-and gender-standardised rates are presented and comparison made with existing national data and select data from other economically developed countries and global institutions. Results: A total of 3603 incident cases were identified between 2004 and 2013. Mature lymphoid malignancies accounted for approximately 60% (n = 2153), myeloma/plasma cell neoplasms 13% (n = 465), acute leukaemia 17% (n = 596), chronic myeloid leukaemia 4% (n = 155) and other myeloproliferative neoplasms 6% (n = 234) when stratified according to conventional groups. Most subtypes increase with age, with male excess. Conclusion: Haematological malignancies in the Eastern Cape Province show disparities in gender and pa- thology-specific incidence patterns. The present study suggest that haematological malignancies are not un- common in this region and the incidence rate of at least one rare subtype, APL, is comparable with some European populations. 1. Introduction Haematological malignancies (HMs) have been described in Africa and Southern Africa for over a century [1–4]. Early references to cancer from the previous century bear witness to longstanding and widespread interest in cancer prevalence. The narrative provides clues to changing patterns in cancer incidence. Some changes have been exposed as ar- tefacts relating to improvements in diagnostic and registration prac- tices. Some deviations represent real changes, for example the surge in lymphomas in the era of the HIV/AIDS pandemic [5]. The African continent, however, remains under-represented in global cancer sta- tistics, with urban bias, limited rural coverage and limited reliable population-based incidence data [6]. South Africa is described by the United Nations as a middle-income country, with indigenous populations akin to those in other developing countries urbanising at a fast pace [7]. The Eastern Cape Province (ECP) is regarded as one of the least urbanised regions, with vast and sparsely populated rural areas and is known for its large number of remaining traditional dwellings [8]. Although ECP has two highly de- veloped metropolitan areas, the rural population continues to engage in traditional lifestyles, some through choice, but many through necessity imposed by widespread poverty and under-development [8]. Cancer surveillance in ECP entered the local and international arena in 1955 when high prevalence was detected for eosophageal cancer. https://doi.org/10.1016/j.canep.2018.01.016 Received 14 September 2017; Received in revised form 19 December 2017; Accepted 26 January 2018 ⁎ Corresponding author at: Department of Pharmacy, Nelson Mandela University, PO Box 77000, Port Elizabeth, 6031, South Africa. Tel.: +27 41 3956111. E-mail addresses: diana.oelofse@nhls.ac.za, s213521962@mandela.ac.za (D. Oelofse), ilse.truter@mandela.ac.za (I. Truter). Abbreviations: ALL, acute lymphoblastic leukaemia; AML, acute myeloid leukaemia; APL, acute promyelocytic leukaemia; ASR, age standardised incidence rates; CI, confidence interval; CML, chronic myeloid leukaemia; ECP, Eastern Cape Province; HMs, haematological malignancies; HL, hodgkin lymphoma; ICD-O-3, International Classification of Disease for Oncology; IR, incidence rate; LPD, lymphoproliferative disorders; MGUS, monoclonal gammopathy of undetermined significance; MLUS, monoclonal B-cell lymphocytosis; NHL, non-hodgkin lymphoma; NPCR, National Program for Cancer Registries; RARECARE, surveillance of rare cancers in Europe Cancer Epidemiology 53 (2018) 166–171 1877-7821/ © 2018 Elsevier Ltd. All rights reserved. T