Peripheral-blood cytopenia, an early indicator of inborn errors of immunity.

Inborn errors of immunity (IEI) are inherited monogenic disorders resulting in defective immune response. Non-infectious presentations are increasingly more apparent. Widely available, cost-effective early indicators are needed. Peripheral-blood cytopenia may be a presenting laboratory feature or an observed secondary phenomenon. This retrospective review of the South African Primary Immunodeficiency Registry (SAPIDR) aimed to assess the haematological indices at presentation and their association with the International Union of Immunological Societies (IUIS) 2019 IEI classification and mortality. Of 396 patients on the SAPIDR, 66% (n = 257) had available haematological results. Sixty percent were males and 85% under 18 years. A majority (53%) had predominantly antibody deficiency. At presentation, infection was prominent (86%) followed by cytopenia (62%). Neutropenia was associated with IUIS III [odds ratio (OR) 3.65, confidence interval (CI) 1.44-9.25], thrombocytopenia with IUIS II (OR 14.39, CI 2.89-71.57), lymphopenia with IUIS I (OR 12.16, CI 2.75-53.73) and pancytopenia with IUSI I (OR 12.24, CI 3.82-39.05) and IUIS II (OR 5.99, CI 2.80-12.76). Cytopenia showed shorter overall survival (OR 2.81, CI 1.288-4.16). Cytopenias that are severe, persistent, unusual and/or recurrent should prompt further investigation for IEI. The full blood count and leucocyte differential may facilitate earlier identification and serve as an adjunct to definitive molecular classification.

Hematological findings in adult patients with SARS CoV-2 infection at Tygerberg Hospital Cape Town South Africa.

Background: Coronavirus disease 2019 (COVID-19) is associated with hematological abnormalities of variable severity. The full blood count (FBC) and leukocyte differential count (DIFF) could facilitate the prediction of disease severity and outcome in COVID-19. This study aimed to assess the hematological parameters in early severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and their correlation with disease outcome. Methods: A retrospective cross-sectional descriptive study was performed. Adults with a FBC and positive SARS-CoV-2 polymerase chain reaction results between March 1, and June 31, 2020 were reviewed. Basic hematological parameters (FBC, DIFF) and human immunodeficiency virus (HIV) status were recorded. Outcome measures were admission to a general ward or intensive care unit (ICU), recovery or death. Results: Six hundred and eighty-five cases median age 51 years, were analyzed. Forty-four percent were males and fourteen percent were HIV-positive with no association between death and/or ICU admission (p = 0.522 and p = 0.830, respectively). Leucocytosis was predictive of ICU admission (odds ratio [OR]: 2.4, confidence interval [CI]: 1.77-3.8186) and neutrophilia, of both mortality (OR: 1.5, CI: 1.0440-2.0899) and ICU admission (OR: 4, CI: 2.5933-6.475). Median lymphocyte count was decreased and d-dimer raised, showing no significant association with outcome. Raised neutrophil-to-lymphocyte-ratio (NLR) was associated with increased odds of mortality (OR: 2.5, CI: 1.3556-3.2503) and ICU admission (OR: 4.8, CI: 2.4307-9.5430) as was monocyte-to-lymphocyte-ratio (MLR) (OR: 2, CI: 1.3132-2.9064) and (OR: 2.3, CI: 1.0608-1.9935), respectively. Hospital admission and older age were significantly associated with mortality (p = 0.0008 and p < 0.0001), respectively. Conclusion: Evidence-based interpretation of routine laboratory parameters, readily available in resource-constrained settings, may identify patients at increased risk of mortality. The FBC, DIFF, NLR, and MLR should form part of the early COVID-19 investigation.