Comorbidities Drive Outcomes for Both Malignancy-Associated and Non–Malignancy-Associated Hemophagocytic Syndrome

Johnson, Benny; Giri, Smith; Nunnery, Sara; Wiedower, Eric; Jamy, Omer; Yaghmour, George; Chandler, Jason C.; Martin, Michael

doi:10.1016/j.clml.2016.01.002

Cited by 8 publications

(5 citation statements)

References 28 publications

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“…HLH can occur as initial HLH or concomitantly with malignant disease. The mechanism of malignancy-associated hemophagocytosis is not well understood, and may be associated with cytokine secretion (including interferon-γ and interleukin-6), persistent antigen stimulation by malignant cells, chemotherapy-induced immunosuppression, hematopoietic stem cell transplantation (HSCT), or infection [3].…”

Section: Introductionmentioning

confidence: 99%

Comparison between clinical features and prognosis of malignancy- and non-malignancy–associated pediatric hemophagocytic lymphohistiocytosis

2019

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BackgroundThe differences between the clinical characteristics and survival time in malignancy- and non-malignancy–associated secondary hemophagocytic lymphohistiocytosis (HLH) are unclear. Here, we describe the clinical characteristics, prognostic factors, and survival outcomes of malignancy-associated HLH compared to that of non-malignancy–associated HLH.MethodsWe retrospectively analyzed 91 pediatric patients with HLH (age < 14 years) at the Affiliated Hospital of Qingdao University Pediatric Department between January 2005 and October 2016. The patients were divided into the malignancy-associated group (n = 22) and non-malignancy–associated group (n = 69, also considered the control group). The clinical features were compared using the Mann–Whitney U and χ2 tests. The overall survival time was compared using log rank and Mann–Whitney U tests.ResultsHemoglobin (HGB; p = 0.004), alanine aminotransferase (ALT; p = 0.002), and aspartate aminotransferase (AST; p = 0.001) levels in the malignancy-associated group differed from that in the non-malignancy–associated group. The mean survival times were 26.9 ± 3.82 months (malignancy-associated HLH) and 35.03 ± 2.19 months (non-malignancy–associated HLH). The overall survival time between the two groups was not statistically significantly different (p = 0.055). Univariate analysis showed that disseminated intravascular coagulation (DIC) score > 5 (p = 0.001), albumin < 25 g/L (p = 0.000), HGB < 60 g/L (p = 0.001), and platelet count (PLT) < 30 × 109/L (p = 0.042) correlated with prognosis. Multivariate Cox analysis showed that albumin < 25 g/L (p = 0.017), HGB < 60 g/L (p = 0.027), and bone marrow hemophagocytosis (p = 0.034) correlated with worse prognosis.ConclusionsPatients with non-malignancy–associated HLH do not have better survival, although their prognosis is relatively better in clinical practice. A higher DIC score at diagnosis and lower albumin, HGB, and PLT levels are negative prognostic factors in malignancy-associated HLH.

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Section: Introductionmentioning

confidence: 99%

Comparison between clinical features and prognosis of malignancy- and non-malignancy–associated pediatric hemophagocytic lymphohistiocytosis

2019

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“…Currently, R-CHOP is the standard-of-care first-line chemotherapy regimen for DLBCL-HLH, whereas in refractory cases, intensive chemotherapy regimens are suggested [ 9 ]. Chemotherapy may not salvage these patients because of severe multiorgan failure at presentation leading to an overall mortality of > 90% [ 10 ]. In such cases, cytokine adsorption might bridge the patient until chemotherapy contributes to reduce HLH disease activity.…”

Section: Discussionmentioning

confidence: 99%

Cytokine adsorption therapy in lymphoma-associated hemophagocytic lymphohistiocytosis and allogeneic stem cell transplantation

et al. 2021

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Lymphoma-associated Hemophagocytic lymphohistiocytosis (HLH) represents a severe complication of disease progression, mediated through cytokine release from the lymphoma cells. Cytokine adsorption may contribute as a supportive treatment to stabilize organ function by reduction of cytokine levels. So far, no experiences of cytokine adsorption and simultaneous stem cell transplantation were published. We report the case of a patient with aggressive lymphoma secondary to chronic lymphocytic leukemia with rapidly progressive HLH (Richter’s transformation) upon conditioning chemotherapy prior to allogeneic stem cell transplantation (ASCT). Continuous hemodiafiltration was initiated in the treatment of shock with acute renal failure, lactacidosis and need for high-dose catecholamine therapy, integrating an additional cytokine-adsorbing filter (CytoSorb®) to reduce cytokine levels. This was followed by scheduled allogenic stem cell transplantation. We observed a marked decrease in interleukin-6 plasma levels, associated with a reduced need for vasopressor therapy and organ function stabilization. Hematopoietic engraftment was present at day 14 post-ASCT, leading to disease-free discharge at day 100 post-transplantation. Cytokine adsorption may serve as a safe adjunct to HLH/sepsis treatment during allogeneic stem cell transplantation. Clinical studies are required to make future treatment recommendations.

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“…In contrast, acquired, or secondary forms of HLH most commonly occur due to extreme immunologic activation, which can be triggered by infections, autoimmune disorders, and malignancy, with leukemia and lymphoma being the most common associated neoplasms [ 7 , 8 ]. Retrospective analysis of 276 adult patients hospitalized for secondary HLH looking at novel predictors of mortality, showed that malignancy-associated secondary HLH, older age, and worse renal function were associated with worse outcomes, and that outcomes were predicated on the extent of organ dysfunction at diagnosis [ 9 ]. The onset of secondary HLH has also been reported to occur during pregnancy, typically after the second trimester [ 10 - 12 ].…”

Section: Introductionmentioning

confidence: 99%

A Case of Recurrent Pregnancy-Induced Adult Onset Familial Hemophagocytic Lymphohistiocytosis

Wang

Ramsingh

et al. 2018

World J Oncol

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Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disease primarily of children, characterized by a severe hyperinflammatory state. We describe a case of adult onset familial HLH with a novel exon 19, c.1607G>T (p.Arg536Leu) heterozygous mutation of the UNC13D gene in a 40-year-old woman who developed HLH during her first and second pregnancies, both episodes occurring during the first trimester. Our patient was treated successfully both times with HLH-94 protocol following spontaneous abortions and is currently in the process of getting a bone marrow transplant. We also discuss pregnancy as a potential trigger for late onset familial HLH.

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Comorbidities Drive Outcomes for Both Malignancy-Associated and Non–Malignancy-Associated Hemophagocytic Syndrome

Cited by 8 publications

References 28 publications

Comparison between clinical features and prognosis of malignancy- and non-malignancy–associated pediatric hemophagocytic lymphohistiocytosis

Comparison between clinical features and prognosis of malignancy- and non-malignancy–associated pediatric hemophagocytic lymphohistiocytosis

Cytokine adsorption therapy in lymphoma-associated hemophagocytic lymphohistiocytosis and allogeneic stem cell transplantation

A Case of Recurrent Pregnancy-Induced Adult Onset Familial Hemophagocytic Lymphohistiocytosis

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