Research Articles (Haematological Pathology)

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An approach to the diagnosis and management of multiple myeloma
(South African Medical Association, 2019-09-30) Fazel, F.; Bassa, F.
ENGLISH ABSTRACT: Multiple myeloma (MM) is a plasma cell dyscrasia that accounts for ~10% of haematological malignancies. It is a disease of the elderly, with a slight male predominance. Almost all cases of MM are preceded by an asymptomatic, premalignant phase known as monoclonal gammopathy of undetermined significance (MGUS). The clinical presentation of MM may be nonspecific, with the most common presenting symptoms being fatigue, bone pain and anaemia. The diagnostic criteria for MM were revised in 2014 to include 3 specific biomarkers of malignancy that are associated with an increased risk of target organ damage. This has resulted in a paradigm shift in the management of MM. The introduction of immunomodulatory agents and proteasome inhibitors has significantly improved the survival of patients with MM. Autologous stem cell transplantation remains the standard of care in younger, fit patients, where there is also a clear role for maintenance chemotherapy. Transplant-ineligible patients benefit from a prolonged induction therapy, and the role of maintenance therapy in this setting is still unclear. Despite major advances in therapy, MM remains an incurable malignant condition and novel agents such as monoclonal antibodies play an important role, especially in the elderly and patients who have relapsed.
The value of histopathology of the placenta in a tertiary referral hospital in South Africa
(Health and Medical Publishing Group, 2019) Malusi, Z.; Schubert, P. T.; Theron, G. B.; Wright, C. A.
Background. Unexplained intrauterine death (IUD) remains the most common cause of perinatal death in babies of <1 000 g in South Africa (SA). Information from examination of the placenta subsequent to an adverse perinatal outcome is often underutilised and placental histology can contribute to determining the cause of perinatal death and other adverse outcomes in many instances. Objectives. To correlate placental histopathology with the clinical indication for submission and to demonstrate the value of placental histopathology in understanding adverse perinatal outcomes. Methods. We reviewed 2 years’ singleton placental histology reports at a tertiary academic hospital in the Western Cape, SA. All samples were from placentas of >24 weeks’ gestation. Results. The total sample (N=822) comprised 60.9% live-birth placentas and 39.1% IUD placentas. In the IUD group, the cause of death was clinically unexplained in 55.9% of cases. Histopathology identified in this group included chorioamnionitis (CA) (34.5%), maternal vascular malperfusion (32.1%), abruptio placentae (31.5%), delayed villous maturation (17.8%) and toxoplasmosis, other agents, rubella, cytomegalovirus and herpes simplex (TORCH) infections (6.1%), most commonly syphilis. No pathology was found in only 2% of IUD cases. Among live births, preterm labour accounted for 41.9% of placental submissions, of which the cause was unknown in 46.2% of cases. Clinically indicated and histologically defined CA was poorly correlated. Conclusion. This study demonstrates the value of placental histopathology in cases of adverse perinatal outcome.
Adult T-cell leukaemia / lymphoma in an adolescent patient : expect the unexpected
(AOSIS, 2020-05-25) Abdullah, Ibtisam; Nell, Erica-Mari; Chapanduka, Zivanai C.
ENGLISH ABSTRACT: This case study explores a clinicopathological presentation of Adult T-cell leukaemia/lymphoma (ATLL) at Tygerberg Hospital; a disease associated with adulthood noted in an adolescent patient. Adult T-cell leukaemia–lymphoma oncogenesis develops through a multistep process with an accumulation of mutations. Infection through human T-lymphotropic virus type 1 (HTLV-1) is the first step of a multistep process resulting in eventual clonal proliferation of mature T-cells. There is a long latency period of 20–50 years from the time of infection with HTLV-1 to the development of symptoms of ATLL; thus, ATLL is a malignancy associated with adulthood. The median age of diagnosis is 58, ranging from the third to ninth decade of life. This is an ideal learning case as it highlights the importance of recognising ATLL in children and young adults in our population.
The impact of physician education regarding the importance of providing complete clinical information on the request forms of thrombophilia-screen tests at Tygerberg hospital in South Africa
(Public Library of Science, 2020-08-06) Abdullah, Ibtisam; Jafta, Andrea D.; Chapanduka, Zivanai C.
Background: Thrombophilia-screen tests are specialised haemostasis tests that are affected by numerous unique patient variables including the presence of acute thrombosis, the concomitant use of medication and patient demographics. Complete information on the request form is therefore crucial for the haematological pathologist to make patient-specific interpretation of patients’ results. Objectives: To assess the completeness of thrombophilia-screen test request forms and determine the impact of provision of incomplete information, on the interpretive comments generated by reporting haematological pathologists. To assess the impact of an educational session given to clinicians on the importance of providing all the relevant information on the request forms. Method: Two retrospective audits, each covering 3 months, were performed to evaluate the completeness of demographic and clinical information on thrombophilia-screen request forms and its impact on the quality of the interpretive comments before and after an educational intervention. Results: One hundred and seventy-one request forms were included in the first audit and 146 in the second audit. The first audit revealed that all 171 thrombophilia-screen request forms had complete patient demographic information but none had clinical information. Haematological pathologists only made generic comments which could not be applied to a specific patient. The second audit, conducted after a physician educational session, did not reveal any improvement in the clinical information provision by the test-ordering physicians. This was reportedly due to the lack of space on the request form. The interpretive comments therefore remained generic and not patient-specific. Conclusion: Physicians’ failure to provide relevant clinical information made it impossible for pathologists to make patient-specific interpretation of the results. A single physician education session did not change the practice, reportedly due to the inappropriate design of the test request form. Further studies are required to investigate the impact of an improved request form and the planned electronic test requesting.
Guidelines for lupus anticoagulant testing in South Africa
(The Society of Medical Laboratory Technologists of South Africa, 2020) Bailly, J.; Louw, S.; De Koker, A.; Potgieter, J. J. C.; Coetzee, M. J.; Chapanduka, Z. C.; Opie, J. J.
ENGLISH ABSTRACT: The lupus anticoagulant (LA) refers to the prolongation of certain coagulation tests due to the action of heterogenous autoantibodies. However, the LA is a misnomer since it is associated with thrombosis in vivo, and most commonly is detected as an incidental, transient laboratory finding associated with conditions such as autoimmune diseases, infections and even in healthy individuals. Repeatedly positive LA testing in the setting of thrombotic and/or obstetric complications is required to diagnose the antiphospholipid syndrome (APS). This review intends to provide clarity on LA testing in the South African context and to provide a national guideline in order to standardise LA testing, interpretation and reporting of results.

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