Risk of hemophagocytic lymphohistiocytosis in adults with fevers of unknown origin: the clinical utility of a new scoring system on early detection

Wang, Hao‐Yuan; Yang, Ching Fen; Chiou, Tzeon Jye; Yang, Sheng; Gau, Jyh Pyng; Yu, Yuan Bin; Chen, Po Min; Hsu, Hui‐Chun; Fung, Chang Phone; Lin, Hui-Wen; Tzeng, Cheng Hwai; Liu, Jin Hwang; Hsiao, Liang Tsai

doi:10.1002/hon.2333

Cited by 9 publications

(8 citation statements)

References 32 publications

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“…Among predisposing conditions, malignancy (especially lymphoma) is a major contributor to HLH/MAS. Investigation for underlying malignancy should be considered in all patients with HLH/MAS, particularly in adults where it occurs in nearly half of cases (7,20,(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37). Although MAS is most recognised and best studied in sJIA and adult-onset Still disease (AOSD), SLE may be a more common cause of HLH/MAS in adults in part due to its higher prevalence (38)(39)(40).…”

Section: Points To Considermentioning

confidence: 99%

The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS)

Shakoory,

Geerlinks,

Wilejto

et al. 2023

Arthritis & Rheumatology

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ObjectiveHaemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life‐threatening systemic hyperinflammatory syndromes that can develop in most inflammatory contexts. They can progress rapidly, and early identification and management are critical for preventing organ failure and mortality. This effort aimed to develop evidence‐based and consensus‐based points to consider to assist clinicians in optimising decision‐making in the early stages of diagnosis, treatment and monitoring of HLH/MAS.MethodsA multinational, multidisciplinary task force of physician experts, including adult and paediatric rheumatologists, haematologist/oncologists, immunologists, infectious disease specialists, intensivists, allied healthcare professionals and patients/parents, formulated relevant research questions and conducted a systematic literature review (SLR). Delphi methodology, informed by SLR results and questionnaires of experts, was used to generate statements aimed at assisting early decision‐making and optimising the initial care of patients with HLH/MAS.ResultsThe task force developed 6 overarching statements and 24 specific points to consider relevant to early recognition of HLH/MAS, diagnostic approaches, initial management and monitoring of HLH/MAS. Major themes included the simultaneous need for prompt syndrome recognition, systematic evaluation of underlying contributors, early intervention targeting both hyperinflammation and likely contributors, careful monitoring for progression/complications and expert multidisciplinary assistance.ConclusionThese 2022 EULAR/American College of Rheumatology points to consider provide up‐to‐date guidance, based on the best available published data and expert opinion. They are meant to help guide the initial evaluation, management and monitoring of patients with HLH/MAS in order to halt disease progression and prevent life‐threatening immunopathology.

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Section: Points To Considermentioning

confidence: 99%

The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS)

Shakoory,

Geerlinks,

Wilejto

et al. 2023

Arthritis & Rheumatology

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“…There is dire need for diagnostic criteria to be revised so as to increase the sensitivity of existing tools. Indeed, there have been studies done recently evaluating other tools of HLH diagnosis offering more sensitivity and specificity, for instance, Bone Marrow score14 or HScore 17…”

Section: Discussionmentioning

confidence: 99%

Cholestatic jaundice: a unique presentation leading to the diagnosis of HLH with Hodgkin lymphoma, HIV and EBV

Shaikh

Kamran

et al. 2018

BMJ Case Reports

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Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of dysregulated immune activity with macrophage activation that can manifest as pancytopenia, coagulopathy and other laboratory abnormalities, usually progressing to multiorgan failure and death. This report documents the rarely reported association between HLH and Hodgkin's lymphoma (HL) with simultaneous HIV and Epstein-Barr virus (EBV) and complete resolution with chemotherapy. The patient initially presented with cholestatic jaundice. He was then found to have HL associated with HLH with coexistent HIV and EBV viraemia. A-Brentuximab-VD regimen for the lymphoma was initiated, resulting in rapid resolution of HLH and ultimately remission of HL. HLH is a syndrome known to have high mortality; thus, early diagnosis and prompt treatment are crucial. Usual presentation includes non-specific symptoms and can easily be overlooked. This case highlights the importance of diagnosing the condition in unusual settings and attempting treatment by targeting the cause of HLH, HL in our case.

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“…Finally, we recently developed a diagnostic tool for use in adult patients with naïve FUO—Bone Marrow Score—which can predict HLH in adults with FUO 11, 26. This scoring system utilizes easily available parameters, including cell blood counts, the presence of peripheral blood leukoerythroblastosis, serum LDH and ferritin levels, and splenomegaly.…”

Section: Discussionmentioning

confidence: 99%

Primary bone marrow lymphoma: A hematological emergency in adults with fever of unknown origin

et al. 2018

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Primary bone marrow lymphoma (PBML) represents non‐Hodgkin lymphoma (NHL) that primarily arises in the bone marrow (BM) without lymphadenopathy. This condition has various definitions and can be masked by prolonged fever, leading to delayed diagnosis. We aimed to identify clinical features and risk indicators of PBML. We enrolled 269 adults with fever of unknown origin (FUO) who underwent a BM study for potential PBML. Thirty patients were diagnosed with PBML (26 and 4 patients in the training and validation cohort, respectively), and 20 patients (67%) showed initial manifestation of hemophagocytic lymphohistiocytosis (HLH). Among PBML patients in the training cohort, their median overall survival is short (8 days), with pneumonia being the most common direct cause of early mortality, followed by life‐threatening HLH. Despite extremely poor prognoses, some B‐cell PBML patients who survived 30 days after BM studies achieved long‐term survival with rituximab‐based treatment. To assist general practitioners in early PBML diagnosis when approaching adults with naïve FUO, we identified several risk indicators, including elevated serum alkaline‐phosphate levels, lowered serum immunoglobulin‐G levels, cytopenia in ≥2 lineages, and peripheral blood leukoerythroblastosis. Our recently published scoring system, which can predict hematological BM disease in FUO adults, showed excellent ability in recognizing PBML early, with high sensitivity and specificity. We conclude that PBML is a specific “clinical” phenotype of NHL; moreover, we have identified diagnostic clues for early identification of FUO adults with underlying PBML, which should be considered a hematological emergency once suspected in any adult with FUO.

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Risk of hemophagocytic lymphohistiocytosis in adults with fevers of unknown origin: the clinical utility of a new scoring system on early detection

Cited by 9 publications

References 32 publications

The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS)

The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS)

Cholestatic jaundice: a unique presentation leading to the diagnosis of HLH with Hodgkin lymphoma, HIV and EBV

Primary bone marrow lymphoma: A hematological emergency in adults with fever of unknown origin

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