Topic:Topic: 19. Aggressive Non-Hodgkin lymphoma - Clinical Background: Background: Secondary Hemophagocytic Lymphohistiocytosis (HLH) is an aggressive life-threatening syndrome leading to uncontrolled immune hyperactivation and end organ failure. Real world data on the outcome of secondary HLH amongst Asians are limited to small case series. Aims: Aims: The purpose of this paper is to describe the patient profiles, aetiologies and survival outcomes of adult patients with secondary HLH in a tertiary hospital in Singapore. Methods: Methods: This is a retrospective medical records review of adult patients with secondary HLH between March 2015 and June 2020. Ethics approval was obtained (DSRB 2020/00833). Survival probabilities over time were estimated using the Kaplan–Meier method and their distributions between malignancy and non-malignancy-associated HLH were compared using log rank test. Hazard ratios (HR), along with 95% confidence intervals (CI), were estimated using Cox proportional hazard models. Results: Results: 36 patients (18 females, 18 males) aged 23 to 80 were followed up for a median 14 months. 16 had underlying aggressive lymphomas (B cell (N=5), T cell (N=5), NK/T cell (N=5), unknown subtype (N=1)). 15 had underlying viral infections (11 Dengue, 2 EBV, 1 parvovirus, 1 unknown). No cause was found in 5 patients. Of the 30 patients who had tissue biopsies performed, 28 (93.3%) demonstrated hemophagocytosis. The mean HSCORE was 234.3 indicating >96% probability for HLH. Treatment: 65% patients without an underlying malignancy required HLH-directed Summary/Conclusion: Summary/Conclusion: Our case series highlight the stark contrast in the overall survival of secondary HLH patients with and without an underlying malignancy. The outcome of malignancy-associated HLH in our study was similar to MD Anderson Cancer Center's experience (25% and 20% overall survival respectively; with a median survival of 1.5 months in both). Prompt recognition amongst physicians likely to encounter HLH, early referral to hematologists, prompt initiation of HLH-directed and supportive therapies and tackling underlying triggers, are key to improving the outcome of secondary HLH.
Aplastic anaemia is a rare clinical syndrome associated with diminished or absent precursors in the bone marrow. Acquired aplastic anaemia secondary to human immunodeficiency virus (HIV) is very rare. We present a 71-year-old woman with severe aplastic anaemia secondary to HIV infection, which was after extensive exclusion of other causes. She achieved undetectable viral load after 5 months of combination antiretroviral therapy but remains profoundly pancytopenic, complicated by recurrent infectious and bleeding complications. HIV infection should be considered in patients with pancytopenia.
The diagnosis of iron deficiency in hospital patients can be difficult in the presence of inflammation. A raised serum transferrin receptor (sTfR) level is useful as a marker of iron deficiency as it is unaffected by inflammation. However, diseases that cause an increase in erythropoietic activity can also result in a raised sTfR level. In South-East Asia, the prevalence of thalassaemia trait is high. As thalassaemia trait is associated with ineffective erythropoiesis and therefore an increase in the sTfR level, we studied the influence of thalassaemia trait on the diagnosis of iron deficiency in hospital patients. Among 431 patients with different combinations of iron deficiency, alpha- and beta-thalassaemia trait, we found that the sTfR level is an excellent diagnostic test for iron deficiency only in patients without thalassaemia trait. alpha-Thalassaemia trait worsened its diagnostic accuracy and beta-thalassaemia trait rendered it a non-diagnostic test. We conclude that in populations with a high prevalence of thalassaemia trait, the sTfR level is not useful in diagnosing iron deficiency unless the patient's thalassaemia status is known.
INTRODUCTION:Iron parameters like serum ferritin and iron saturation are routinely used in diagnosing iron deficiency. However, these tests are influenced by many factors. We aimed to review the accuracy of iron parameters among inpatients in an acute care hospital.MATERIALS AND METHODS:From October 1997 to April 2002, bone marrow aspirate samples from patients on whom concurrent iron studies had been done were analysed. Accuracy of the various iron parameters was analysed using receiver operating characteristic curves.RESULTS:Among 92 bone marrow aspirate samples, 58, 86 and 83 had a concurrent serum ferritin, serum iron and percentage iron saturation done respectively. Serum ferritin is the best marker for predicting the presence of iron deficiency. This is followed by percentage iron saturation and lastly by serum iron. At the most optimal, a serum ferritin of <60 ng/mL has a positive likelihood ratio of 24.35, sensitivity of 69.6%, specificity of 97.1% and positive predictive value of 94.1%. An iron saturation of <7% has a positive likelihood ratio of 21.62, sensitivity of 44.1%, specificity of 98.0% and positive predictive value of 93.8%. Seven out of 50 samples, which had concurrent measurements of iron saturation and serum ferritin, were discordant in indicating the presence of iron deficiency. In 6 cases, the iron saturation was the spurious result.CONCLUSION:A serum ferritin of <60 ng/mL or a percentage iron saturation of <7% is highly predictive of iron deficiency among inpatients in an acute care hospital.