hemophagocytic lymphohistiocytosis
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Hemophagocytic Lymphohistiocytosis (HLH) is a rare but life-threatening disease that can occur either as a primary condition or as a consequence of a variety of triggers, including infectious diseases. Here we present a case of secondary HLH triggered by systemic Mycobacterium tuberculosis infection in a 59-year-old immunocompromised Hairy Cell Leukemia and previous SARS-CoV2 infected patient. This case report underlines the role of Etoposide-based chemotherapy in treating the severe inflammation that is the defining factor of HLH, suggesting how, even when such therapy is not effective, it may still give the clinicians time to identify the underlying condition and start the appropriate targeted therapy. Moreover, it gives insight on our decision to treat the underlying haematological condition with a BRAF-targeted therapy rather than purine analog-based chemotherapy to reduce the risk of future severe infections.

Abstract Purpose: This study aimed to assess the prognostic value of pretreatment albumin-bilirubin (ALBI) on short-term mortality (30 days) and long-term (≥ 1 year) survivalThe aim of this study was to construct a prognosis model of non-Hodgkin lymphoma-associated secondary hemophagocytic lymphohistiocytosis (NHL-sHLH) patients with hepatic injuries by the combination of ALBI score and clinical parameters.Material and methods: This retrospective study included 168 NHL-sHLH patients with hepatic injuries between February 1, 2014, and February 1, 2020. Multivariable logistic/Cox models and restricted cubic spline models were conducted to evaluate the relationships between the ALBI score and short- and long-term survival. The predictive performance of the ALBI score was assessed and compared using time-dependent receiver operating characteristic (ROC) analysis.Results: Among 168 adult NHL-sHLH patients, 82 (48.8%) patients died within 30 days after admission, and 144 (85.7%) patients died during the follow-up period. Multivariate logistic regression indicated that ALBI grade could be an independent risk factor for predicting the prognosis of patients with 30-day mortality and overall survival (odds ratios [OR]30 days 5.37, 95% confidence interval 2.41-12.64, P < 0.001; hazard ratios [HR]OS 1.52, 95% confidence interval 1.06-2.18, P = 0.023), respectively. The restricted cubic spline curve displayed a linear and positive relationship between the ALBI score and risk of mortality (P for nonlinearity =0.503). Furthermore, receiver operating characteristic (ROC) curve analysis showed that the area under the curve (AUC) for predicting mortality by integrative analysis of the ALBI score and ferritin was significantly improved compared to the ALBI score (AUC 30 days: 0.820 vs 0.693, P = 0.001; AUC1 year: 0.754 vs 0.681, P = 0.043) or ferritin (AUC30 days: 0.820 vs 0.724, P = 0.005; AUC1 year: 0.754 vs 0.658, P = 0.031) alone.Conclusions: These results suggest that the ALBI score could be a useful indicator of 30-day mortality and overall survival (≥1 year) for NHL-sHLH patients with hepatic injuries, and the combination analysis of the ALBI score and ferritin provides incremental prognostic value for clinical use.

ABSTRACT Objective: Hemophagocytic lymphohistiocytosis syndrome (HLHS) is characterized by an immunological hyperactivation of cytotoxic T cells, natural killer cells, and macrophages, leading to the secretion of proinflammatory cytokines. HLHS associated with Visceral Leishmaniasis might be difficult to diagnose once symptoms are similar, resulting in the death of untreated patients. Our aim is to describe a series of cases of Visceral Leishmaniasis with HLHS admitted to a referral hospital for infectious diseases. Case description: All 115 cases of Visceral Leishmaniasis referred to a referral center for pediatric infectious diseases were reviewed to identify the cases of HLHS. Five cases (4.5%) were confirmed with HLHS and they presented fever, splenomegaly, cytopenia, hypertriglyceridemia or hypofibrinogenemia, increased ferritin and hemophagocytosis in the bone marrow. Comments: It important to rule out HLHS in children with infectious diseases that do not respond adequately to treatment or in patients with severe symptoms, especially in leishmaniasis endemic areas.%

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that causes systemic inflammation which can progress to multiorgan failure and death. Symptoms and signs commonly seen in HLH include high fever, hepatosplenomegaly, pancytopenia, and hypertriglyceridemia. This report describes the 8-month clinical course of a 17-year-old male with G6PD deficiency who presented with intermittent high fever of unknown origin for 8 months accompanied by pancytopenia and bilateral lower limb weakness. A pathogenic homozygous missense mutation (c.1081A > T p.(Arg361Trp)) in the PRF1 gene was detected by whole exome sequencing (WES). The brain and the whole spine MRI showed leptomeningeal enhancement at different levels involving both the brain and the spine. Therefore, a diagnosis of familial HLH type 2 with CNS involvement was confirmed. Accordingly, treatment with dexamethasone, cyclosporin, and etoposide in addition to intrathecal methotrexate and hydrocortisone was given. The patient showed a dramatic response with significant neurological improvement of the bilateral lower limb weakness. Genetic analysis has helped the patient’s family with appropriate genetic counselling. This case highlights the importance of immediate treatment with immunosuppressants and the high clinical suspicion of physicians regarding HLH in areas where consanguinity is common.