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Thromboembolic conditions are a leading cause of mortality, estimated to account for 1 in 4 deaths worldwide in 2010. Over time, the incidence and mortality rates of these conditions have declined in developed countries, but are increasing in developing countries. A delicate balance exists between procoagulant and anticoagulant factors within the vascular system. Numerous acquired or inherited conditions may tip the balance either way, i.e. towards a prothrombotic or prohaemorrhagic state. Acquired thrombotic disorders are the subject of discussion in this issue, the second of a 2-part series on thrombophilia.

Hepatosplenic T-cell lymphoma (HSTCL) is a rare type of Non-Hodgkin Lymphoma (NHL), grouped under the mature or peripheral T-cell lymphomas. It is characterised by extranodal infiltration and proliferation of malignant T-cells within the sinusoids of the liver, sinuses and red pulp of the spleen, and the bone marrow. The tumour cells express CD2 and CD3, but are CD4, CD5 and CD8 negative and express a clonally restricted gamma–delta (or less commonly alpha–beta) T-cell receptor. The disease has an aggressive clinical course associated with a poor prognosis. We highlight and report three patients from South Africa with HSTCL, all of whom had hepatosplenomegaly and cytopaenias, and despite being HIV seronegative and immunocompetent, had a poor outcome, with a mean survival of 7.5 months in the two evaluable patients. This rare entity has not previously been reported from South Africa and as yet needs to be adequately characterised in a population where lymphoma is the most common haematological malignancy in adults, and where approximately two thirds of the adult lymphoma population are HIV seropositive. Keywords: Hepatosplenic T-cell lymphoma, Hepatosplenomegaly, Cytopaenias, Aggressive course, Poor prognosis, South Africa

Acute adult T-cell leukaemia (AATL) is a clinical subtype of adult T-cell leukaemia/lymphoma, a rare type of peripheral T cell lymphoma. It is associated with infection by the retrovirus Human T-Lymphotropic Virus Type-1 (HTLV-1), and is seen most commonly in HTLV-1 endemic areas. The tumour cells have polylobulated nuclei morphologically and are CD3+, CD4+ or CD8+, CD7-, express T-cell receptor , and are CD25+. The disease has an aggressive clinical course associated with marked immunosuppression caused by HTLV-1, and a poor prognosis. We report a rare case of a young male patient who presented with AATL complicated by multiple opportunistic infections including pulmonary tuberculosis and Norwegian scabies. He also had chronic hepatitis B. This constellation of illnesses has not previously been reported from South Africa in a population where another retrovirus, Human Immunodeficiency Virus (HIV), is a far more common occurrence, as are the HIV-related lymphomas.

SummaryIntroduction Peripheral blood smear review (PBSR) is a labour-intensive test, and skilled morphologists are in short supply. It is therefore helpful for laboratories to establish rules for PBSR to improve laboratory efficiency. Previously published guidelines in this regard are useful, but make few recommendations specific to neonates. Neonatal blood is characterized by several peculiarities which would be considered pathological if present in adults. Consequently, smear review rules (SRR) are often triggered in neonates without significant value being added on review. This study aimed to assess and fine-tune the SRR triggered in neonatal samples in order to improve laboratory efficiency. Methods Full blood counts collected from 188 neonatal inpatients of the Chris Hani Baragwanath Academic Hospital in South Africa were retrospectively reviewed, the triggered rules documented, and the value added on PBSR determined. Results Smear review rules were triggered in 148 (78.7%) samples, with significant morphological abnormalities identified in 84 (54.4%), and a false-positive rate of 34.0%. In patients with unhelpful review, the commonest rules triggered were the flags querying the presence of abnormal lymphocytes, blasts or nucleated red blood cells. When one or more of these flags were triggered in the absence of any other SRR, PBSR was always noncontributory. Disregarding these flags in the current cohort would reduce both the review and the false-positive rates by >20% without increasing the false-negative rate. Conclusion False-positive smear review is common in neonates, and minor modifications to SRR can substantially reduce the smear review rate without increasing the false-negative rate.

Background. The HIV epidemic in South Africa (SA) has had a substantial impact on laboratory services, at least partially owing to the well-described propensity to cytopenias in HIV-positive patients. Objectives. ( i ) To formally gauge the impact of HIV infection on the state sector haematology services in SA by determining the HIV seropositivity rate among full blood counts (FBCs) performed at a large academic state sector laboratory; and ( ii ) to document the prevalence of cytopenias among HIV-positive patients in this setting. Methods. Randomly selected FBCs submitted to the National Health Laboratory Service laboratory at Chris Hani Baragwanath Academic Hospital, Johannesburg, were extracted from the laboratory information system (LIS) and retrospectively reviewed. HIV test results and other pertinent information in the LIS were documented, as was the presence of any cytopenias. Results. HIV status was documented in 561 of 1 006 samples (55.8%), with 307 (54.7%) of these being HIV-positive. Of the HIV-positive patients, 63.2% had one or more cytopenia/s. Anaemia was present in 183/307 (59.6%) of the HIV-positive patients, and was severe (haemoglobin

Causes of thrombocytopenia range from laboratory errors to life-threatening pathological conditions. To establish the cause, appropriate laboratory investigation is required.To determine the prevalence and causes of platelet counts100 × 10(9)/L in state health facilities in Johannesburg, South Africa, as well as the quality of the subsequent laboratory work-up in this setting.Full blood counts (FBCs) performed on 7 randomly selected days at the National Health Laboratory Service laboratory at Chris Hani Baragwanath Academic Hospital were retrospectively reviewed. Samples with platelet counts100 × 109/L were identified, and pertinent information was extracted from the laboratory database.Of 4 456 FBCs included, 381 (8.6%) had a platelet count of100 × 10(9)/L. Thrombocytopenia prevalence rates were high in haematology/oncology wards (34.4%), intensive care units (20.5%) and medical wards (18.7%) and among neonatal inpatients (16.5%), and were lowest in outpatient clinics (1-2%). A cause was apparent in ~60% of patients, the commonest causes being chemotherapy and sepsis (each comprising20% of the recognised causes). Spurious thrombocytopenia, disseminated tuberculosis, aplastic anaemia, immune thrombocytopenia and malignant marrow infiltration each accounted for 5 - 10% of the causes, while malaria, thrombotic thrombocytopenic purpura, HIV effect and liver disease were each identified in5% of cases. HIV status was documented in ~70% of the patients, of whom ~50% tested positive. The quality of the laboratory work-up showed differences between specialties within the hospital setting, and was poorest in the primary healthcare clinic sector.Thrombocytopenia is common in hospitalised patients in the Johannesburg academic state sector. Differences in the quality of the laboratory work-up emphasise the need for a standardised approach to thrombocytopenia investigation and increased awareness among clinicians.