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Strauß, Richard; Neureiter, Daniel; Wehler, Markus; Westenburger, B; Kirchner, T.; Hahn, EG

doi:10.1186/cc1559

Cited by 2 publications

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“…[2][3][4] However, while primary HLH has been well characterized in light of the recent discovery of mutations in several genes involved in T-cell cytotoxic activity, it is often challenging to distinguish between true secondary HLH in adults and severe inflammatory conditions, in which features of hemophagocytosis are encountered (e.g., critically ill patients). [5][6][7] Moreover, hypomorphic mutations in PRF1, UNC13D and STBXBP2 have recently been discovered in sporadic cases of HLH in adults. 8 Thus, there may be an overlap between HLH and severe inflammation in some clinical contexts.…”

Section: Introductionmentioning

confidence: 99%

Hemophagocytic syndrome in patients with acute myeloid leukemia undergoing intensive chemotherapy

et al. 2013

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© F e r r a t a S t o r t i F o u n d a t i o nfrequently described as malignancy-or infection-associated hemophagocytic syndrome. [9][10][11] In patients with acute myeloid leukemia (AML), HLH has been occasionally described in case-reports. AML patients may be prone to develop HLH due to their disease-and/or treatment-related impaired immune response and their high susceptibility to severe infections, which act as triggering factors.12 Alerted by several cases of HLH in our department, we sought to determine the frequency and patterns of HLH presentation as well as its impact on prognosis in a series of consecutive AML patients treated with intensive chemotherapy. Methods PatientsBetween January 1, 2006, and December 31, 2010, all consecutive patients with a new diagnosis of AML (except acute promyelocytic leukemia) admitted to our center and eligible for intensive chemotherapy were registered for this study. The diagnostic workup and treatment modalities have been described elsewhere. 13,14 All patients had a central venous catheter placed and were given bacterial digestive tract decontamination, antibiotic therapy for febrile neutropenia (piperacillin-tazobactam/amikacin and imipenem/vancomycin/ciprofloxacin as first-and second-line treatments, respectively) and antifungal prophylaxis with posaconazole. Clinical and biological data were recorded by four of the authors (KD, AS, SB and CR). Biological data, including fibrinogen, C-reactive protein, ferritin and triglyceride levels were assessed at diagnosis and every week thereafter. Bone marrow aspiration was performed routinely at diagnosis, on day 15 of induction chemotherapy (for patients <60 years old), assessment of response (~day 35), in cases of unexpected prolonged cytopenias (> 35 days) or in suspected cases of HLH (i.e, patients receiving antimicrobial treatments for febrile neutropenia, and/or sudden increases in ferritinemia and/or unexpected cytopenias). The presence of features of hemophagocytosis was recorded regardless of clinical presentation. Cytological analysis of MayGrünwald-Giemsa-stained bone marrow smears was performed routinely. Hemophagocytosis was defined by evidence of macrophage-dependent phagocytosis of erythrocytes, leukocytes, platelets and/or their precursors, regardless of the percentage of macrophages (Online Supplementary Figure S1). The findings of the bone marrow aspirates for each patient were discussed between cytologists and physicians during weekly meetings. This study was approved by the Institutional Ethics Committee. Study populationThree hundred and forty-three patients were included. Their characteristics at diagnosis of AML are shown in Table 1. The criteria used to assign patients to the HLH group were hemophagocytosis in the bone marrow with unexplained fever under broad antimicrobial treatment, and/or sudden increases in ferritin and/or unexpected non-blastic pancytopenia. Twenty-nine patients fulfilled these criteria. Three other patients without bone marrow hemophagocytosis had bio-clinical evidenc...

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Section: Introductionmentioning

confidence: 99%

Hemophagocytic syndrome in patients with acute myeloid leukemia undergoing intensive chemotherapy

et al. 2013

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“…Primary HLH is caused by genetic defect, while secondary HLH is mainly triggered by malignant tumors, infections, and immune factors (1,2). The detection of hemophagocytes in the bone marrow is a characteristic of HLH, but not specific, and it is common in other tissues (such as liver, spleen, lymph nodes, etc) of critically ill patients (3). HLH is clinically difficult to diagnose, progresses rapidly, and is susceptible to multi-organ failure, including kidney failure.…”

Section: Introductionmentioning

confidence: 99%

A prediction model for acute kidney injury in adult patients with hemophagocytic lymphohistiocytosis

et al. 2022

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Background and aimsHemophagocytic lymphohistiocytosis is a clinical syndrome resulting from abnormally active immune cells and a cytokine storm, with the accompanying phagocytosis of blood cells. Patients with hemophagocytic lymphohistiocytosis often suffer acute kidney injury during hospitalization, which usually signifies poor prognosis. We would like to establish a prediction model for the occurrence of acute kidney injury in adult patients with hemophagocytic lymphohistiocytosis for risk stratification.MethodWe extracted the electronic medical records of patients diagnosed with hemophagocytic lymphohistiocytosis during hospitalization from January 2009 to July 2019. The observation indicator is the occurrence of acute kidney injury within 28 days of hospitalization. LASSO regression was used to screen variables and modeling was performed by COX regression.ResultsIn the present study, 136 (22.7%) patients suffered from acute kidney injury within 28 days of hospitalization. The prediction model consisted of 11 variables, including vasopressor, mechanical ventilation, disseminated intravascular coagulation, admission heart rate, hemoglobin, baseline cystatin C, phosphorus, total bilirubin, lactic dehydrogenase, prothrombin time, and procalcitonin. The risk of acute kidney injury can be assessed by the sum of the scores of each parameter on the nomogram. For the development and validation groups, the area under the receiver operating characteristic curve was 0.760 and 0.820, and the C-index was 0.743 and 0.810, respectively.ConclusionWe performed a risk prediction model for the development of acute kidney injury in patients with hemophagocytic lymphohistiocytosis, which may help physicians to evaluate the risk of acute kidney injury and prevent its occurrence.

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Cited by 2 publications

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Hemophagocytic syndrome in patients with acute myeloid leukemia undergoing intensive chemotherapy

Hemophagocytic syndrome in patients with acute myeloid leukemia undergoing intensive chemotherapy

A prediction model for acute kidney injury in adult patients with hemophagocytic lymphohistiocytosis

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