2015
DOI: 10.1111/bjh.13462
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Malignancy‐associated haemophagocytic lymphohistiocytosis in children and adolescents

Abstract: SummaryHaemophagocytic lymphohistiocytosis (HLH) in the context of malignancy is mainly considered a challenge of adult haematology. While this association is also observed in children, little is known regarding inciting factors, appropriate treatment and prognosis. We retrospectively analysed 29 paediatric and adolescent patients for presenting features, type of neoplasm or preceding chemotherapy, treatment and outcome. Haemophagocytic lymphohistiocytosis was considered triggered by the malignancy (M-HLH) in … Show more

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Cited by 134 publications
(124 citation statements)
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“…Allen and McClain hypothesized that the syndrome results from complex interactions of host genetics and extrinsic immune challenges [7]. Studies have shown that immune defects in genes affecting regulation of granule-dependent lymphocyte activity as well as intracellular granule trafficking, mutations in T cell functioning (CD27), and cytokine production, that is, interferon (IFN)-gamma and interleukin (IL)-6, by malignant cells play a key role in HLH evolution [5, 8]. Similar genes and inflammatory responses may become affected during neoplasm therapy, inciting an iatrogenic HLH in adults [911].…”
Section: Discussionmentioning
confidence: 99%
“…Allen and McClain hypothesized that the syndrome results from complex interactions of host genetics and extrinsic immune challenges [7]. Studies have shown that immune defects in genes affecting regulation of granule-dependent lymphocyte activity as well as intracellular granule trafficking, mutations in T cell functioning (CD27), and cytokine production, that is, interferon (IFN)-gamma and interleukin (IL)-6, by malignant cells play a key role in HLH evolution [5, 8]. Similar genes and inflammatory responses may become affected during neoplasm therapy, inciting an iatrogenic HLH in adults [911].…”
Section: Discussionmentioning
confidence: 99%
“…There are a number of cases associated with lymphoma and leukaemia, most frequently T cell receptor gamma-delta bearing T cell lymphomas. These appear to be rare in children, but this occurrence is rather frequent in adults [6]. HLH can also be associated to an underlying rheumatic disease with sJIA being by far the most frequent one.…”
Section: Introductionmentioning
confidence: 99%
“…(r=0.7 p=0.035) Our findings indicate that patients with paediatric ALL receive a significant iron burden during therapy, placing them at risk of later morbidity and thatserum ferritin is not a reliable indicator of iron status in this population. Hyperferritinaemia has a wide differential aside from iron overload, including infection, inflammation, and haemophagocytic lymphohistiocytosis (HLH) (Lehmberg, et al, 2015). Extreme hyperferritinaemia in the context of ALL is not specific for a diagnosis of HLH although ALL is a recognised cause of secondary HLH.…”
mentioning
confidence: 99%