Malignancy-associated haemophagocytic lymphohistiocytosis in children and adolescents.
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.
Paediatric Haematology and Oncology, University Medical Centre Hamburg Eppendorf, Hamburg, Germany.
Kai
K
Lehmberg
Paediatric Haematology and Oncology, University Medical Centre Hamburg Eppendorf, Hamburg, Germany.
Björn
B
Sprekels
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
Kim E
KE
Nichols
Paediatric Haematology and Oncology, University Hospital Giessen, Giessen, Germany.
Wilhelm
W
Woessmann
Paediatric Haematology and Oncology, University Medical Centre Hamburg Eppendorf, Hamburg, Germany.
Ingo
I
Müller
Paediatric Haematology and Oncology, University Hospital Dresden, Dresden, Germany.
Meinolf
M
Suttorp
Paediatric Haematology and Oncology, University Hospital Halle, Halle, Germany.
Toralf
T
Bernig
Paediatric Haematology and Oncology, Clinical Centre Schwabing and München Rechts der Isar, Technical University Munich, Munich, Germany.
Karin
K
Beutel
Centre of Chronic Immunodeficiency, University of Freiburg, Freiburg, Germany.
Sebastian F N
SF
Bode
Paediatric Haematology and Oncology, University Hospital Jena, Jena, Germany.
Karim
K
Kentouche
Paediatric Haematology and Oncology, Elisabeth-Hospital Oldenburg, Oldenburg, Germany.
Reinhard
R
Kolb
Gemeinschaftskrankenhaus Herdecke, University of Witten/Herdecke, Herdecke, Germany.
Alfred
A
Längler
Paediatric Haematology and Oncology, Rudolfstiftung Hospital Vienna, Vienna, Austria.
Milen
M
Minkov
Paediatric Oncology and Haematology, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Freimut H
FH
Schilling
Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University Munich, Munich, Germany.
Irene
I
Schmid
Paediatric Haematology and Oncology, University Hospital Kiel, Kiel, Germany.
Simon
S
Vieth
Centre of Chronic Immunodeficiency, University of Freiburg, Freiburg, Germany.
Stephan
S
Ehl
Paediatric Haematology and Oncology, University Medical Centre Hamburg Eppendorf, Hamburg, Germany.
Udo
U
Zur Stadt
Paediatric Haematology and Oncology, University Medical Centre Hamburg Eppendorf, Hamburg, Germany.
Gritta E
GE
Janka
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000188:drug therapy / Q000401:mortality; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007938:Leukemia / Q000188:drug therapy / Q000401:mortality; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology / Q000401:mortality / Q000628:therapy; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate
Hodgkin lymphoma; T-cell lymphoma; anaplastic large cell lymphoma; hemophagocytic syndrome; macrophage activation syndrome