Malignancy‐associated haemophagocytic lymphohistiocytosis in children and adolescents

Summary Haemophagocytic 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 p...

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Veröffentlicht in:British journal of haematology 2015-08, Vol.170 (4), p.539-549
Hauptverfasser: Lehmberg, Kai, Sprekels, Björn, Nichols, Kim E., Woessmann, Wilhelm, Müller, Ingo, Suttorp, Meinolf, Bernig, Toralf, Beutel, Karin, Bode, Sebastian F. N., Kentouche, Karim, Kolb, Reinhard, Längler, Alfred, Minkov, Milen, Schilling, Freimut H., Schmid, Irene, Vieth, Simon, Ehl, Stephan, Zur Stadt, Udo, Janka, Gritta E.
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container_end_page 549
container_issue 4
container_start_page 539
container_title British journal of haematology
container_volume 170
creator Lehmberg, Kai
Sprekels, Björn
Nichols, Kim E.
Woessmann, Wilhelm
Müller, Ingo
Suttorp, Meinolf
Bernig, Toralf
Beutel, Karin
Bode, Sebastian F. N.
Kentouche, Karim
Kolb, Reinhard
Längler, Alfred
Minkov, Milen
Schilling, Freimut H.
Schmid, Irene
Vieth, Simon
Ehl, Stephan
Zur Stadt, Udo
Janka, Gritta E.
description Summary Haemophagocytic 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 21 patients, most of whom had T‐ (n = 12) or B‐cell neoplasms (n = 7), with Epstein–Barr virus as a co‐trigger in five patients. In eight patients, HLH occurred during chemotherapy (Ch‐HLH) for malignancy, mainly acute leukaemias (n = 7); an infectious trigger was found in seven. In M‐ and Ch‐HLH, median overall survival was 1·2 and 0·9 years, and the 6 month survival rates were 67% and 63%, respectively. Seven of 11 deceased M‐HLH patients exhibited active malignancy and HLH at the time of death, while only two out of five deceased Ch‐HLH patients had evidence of active HLH. To overcome HLH, malignancy‐ and HLH‐directed treatments were administered in the M‐HLH cohort; however, it was not possible to determine superiority of one approach over the other. For Ch‐HLH, treatment ranged from postponement of chemotherapy to the use of etoposide‐containing regimens.
doi_str_mv 10.1111/bjh.13462
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N. ; Kentouche, Karim ; Kolb, Reinhard ; Längler, Alfred ; Minkov, Milen ; Schilling, Freimut H. ; Schmid, Irene ; Vieth, Simon ; Ehl, Stephan ; Zur Stadt, Udo ; Janka, Gritta E.</creator><creatorcontrib>Lehmberg, Kai ; Sprekels, Björn ; Nichols, Kim E. ; Woessmann, Wilhelm ; Müller, Ingo ; Suttorp, Meinolf ; Bernig, Toralf ; Beutel, Karin ; Bode, Sebastian F. N. ; Kentouche, Karim ; Kolb, Reinhard ; Längler, Alfred ; Minkov, Milen ; Schilling, Freimut H. ; Schmid, Irene ; Vieth, Simon ; Ehl, Stephan ; Zur Stadt, Udo ; Janka, Gritta E.</creatorcontrib><description>Summary Haemophagocytic 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 21 patients, most of whom had T‐ (n = 12) or B‐cell neoplasms (n = 7), with Epstein–Barr virus as a co‐trigger in five patients. In eight patients, HLH occurred during chemotherapy (Ch‐HLH) for malignancy, mainly acute leukaemias (n = 7); an infectious trigger was found in seven. In M‐ and Ch‐HLH, median overall survival was 1·2 and 0·9 years, and the 6 month survival rates were 67% and 63%, respectively. Seven of 11 deceased M‐HLH patients exhibited active malignancy and HLH at the time of death, while only two out of five deceased Ch‐HLH patients had evidence of active HLH. To overcome HLH, malignancy‐ and HLH‐directed treatments were administered in the M‐HLH cohort; however, it was not possible to determine superiority of one approach over the other. 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N.</au><au>Kentouche, Karim</au><au>Kolb, Reinhard</au><au>Längler, Alfred</au><au>Minkov, Milen</au><au>Schilling, Freimut H.</au><au>Schmid, Irene</au><au>Vieth, Simon</au><au>Ehl, Stephan</au><au>Zur Stadt, Udo</au><au>Janka, Gritta E.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Malignancy‐associated haemophagocytic lymphohistiocytosis in children and adolescents</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>2015-08</date><risdate>2015</risdate><volume>170</volume><issue>4</issue><spage>539</spage><epage>549</epage><pages>539-549</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><abstract>Summary Haemophagocytic 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 21 patients, most of whom had T‐ (n = 12) or B‐cell neoplasms (n = 7), with Epstein–Barr virus as a co‐trigger in five patients. In eight patients, HLH occurred during chemotherapy (Ch‐HLH) for malignancy, mainly acute leukaemias (n = 7); an infectious trigger was found in seven. In M‐ and Ch‐HLH, median overall survival was 1·2 and 0·9 years, and the 6 month survival rates were 67% and 63%, respectively. Seven of 11 deceased M‐HLH patients exhibited active malignancy and HLH at the time of death, while only two out of five deceased Ch‐HLH patients had evidence of active HLH. To overcome HLH, malignancy‐ and HLH‐directed treatments were administered in the M‐HLH cohort; however, it was not possible to determine superiority of one approach over the other. For Ch‐HLH, treatment ranged from postponement of chemotherapy to the use of etoposide‐containing regimens.</abstract><cop>England</cop><pmid>25940575</pmid><doi>10.1111/bjh.13462</doi><tpages>11</tpages><oa>free_for_read</oa></addata></record>
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subjects Acute Disease
Adolescent
Adult
anaplastic large cell lymphoma
Child
Child, Preschool
Disease-Free Survival
Epstein-Barr Virus Infections - complications
Epstein-Barr Virus Infections - drug therapy
Epstein-Barr Virus Infections - mortality
Female
hemophagocytic syndrome
Hodgkin lymphoma
Humans
Infant
Infant, Newborn
Leukemia - drug therapy
Leukemia - mortality
Lymphohistiocytosis, Hemophagocytic - etiology
Lymphohistiocytosis, Hemophagocytic - mortality
Lymphohistiocytosis, Hemophagocytic - therapy
macrophage activation syndrome
Male
Retrospective Studies
Survival Rate
T‐cell lymphoma
title Malignancy‐associated haemophagocytic lymphohistiocytosis in children and adolescents
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