Hemophagocytic lymphohistiocytosis in adults: collaborative analysis of 137 cases of a nationwide German registry
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Hemophagocytic lymphohistiocytosis in adults: collaborative analysis of 137 cases of a nationwide German registry. / Birndt, Sebastian; Schenk, Thomas; Heinevetter, Babett; Brunkhorst, Frank M; Maschmeyer, Georg; Rothmann, Frank; Weber, Thomas; Müller, Markus; Panse, Jens; Penack, Olaf; Schroers, Roland; Braess, Jan; Frickhofen, Norbert; Ehl, Stephan; Janka, Gritta; Lehmberg, Kai; Pletz, Mathias W; Hochhaus, Andreas; Ernst, Thomas; La Rosée, Paul.
In: J CANCER RES CLIN, Vol. 146, No. 4, 04.2020, p. 1065-1077.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Hemophagocytic lymphohistiocytosis in adults: collaborative analysis of 137 cases of a nationwide German registry
AU - Birndt, Sebastian
AU - Schenk, Thomas
AU - Heinevetter, Babett
AU - Brunkhorst, Frank M
AU - Maschmeyer, Georg
AU - Rothmann, Frank
AU - Weber, Thomas
AU - Müller, Markus
AU - Panse, Jens
AU - Penack, Olaf
AU - Schroers, Roland
AU - Braess, Jan
AU - Frickhofen, Norbert
AU - Ehl, Stephan
AU - Janka, Gritta
AU - Lehmberg, Kai
AU - Pletz, Mathias W
AU - Hochhaus, Andreas
AU - Ernst, Thomas
AU - La Rosée, Paul
PY - 2020/4
Y1 - 2020/4
N2 - PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome emerging from a deregulated immune response due to various triggers. In adults, systematic data are sparse, which is why recommendations on diagnosis and management have been adopted from pediatric guidelines. A nationwide clinical registry with associated consulting service as collaborative initiative of HLH-specialized pediatricians and hematologists was initiated to better characterize HLH in adults.METHODS: Patients with proven or suspected HLH were registered by 44 institutions. Both HLH-2004 diagnostic criteria and the HScore (www.saintantoine.aphp.fr/score/) were used to confirm HLH diagnosis. Data referring to underlying disease, treatment, outcome, clinical presentation and laboratory findings were recorded.RESULTS: The study included 137 patients and provides the first systematic data on adult HLH in Germany. Median age was 50 years with a wide range (17-87 years), 87 patients (63.5%) were male. Most common triggering diseases were infections in 61 patients (44.5%) and malignancies in 48 patients (35%). Virtually all patients had elevated ferritin concentrations, and 74% had peak concentrations greater than 10,000 µg/l. At time of analysis, 67 of 131 patients (51%) had died. Patients with malignancy-associated HLH had the shortest median survival (160 days), however no statistically significant difference between subgroups was observed (p = 0.077). Platelets under 20*109/l and low albumin concentrations (< 20 g/l) were associated with poor overall and 30-day survival.CONCLUSION: Close multidisciplinary case consultation and cooperation is mandatory when treating adult HLH patients. Early contact with reference centers is recommended, especially in relapsing or refractory disease.
AB - PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome emerging from a deregulated immune response due to various triggers. In adults, systematic data are sparse, which is why recommendations on diagnosis and management have been adopted from pediatric guidelines. A nationwide clinical registry with associated consulting service as collaborative initiative of HLH-specialized pediatricians and hematologists was initiated to better characterize HLH in adults.METHODS: Patients with proven or suspected HLH were registered by 44 institutions. Both HLH-2004 diagnostic criteria and the HScore (www.saintantoine.aphp.fr/score/) were used to confirm HLH diagnosis. Data referring to underlying disease, treatment, outcome, clinical presentation and laboratory findings were recorded.RESULTS: The study included 137 patients and provides the first systematic data on adult HLH in Germany. Median age was 50 years with a wide range (17-87 years), 87 patients (63.5%) were male. Most common triggering diseases were infections in 61 patients (44.5%) and malignancies in 48 patients (35%). Virtually all patients had elevated ferritin concentrations, and 74% had peak concentrations greater than 10,000 µg/l. At time of analysis, 67 of 131 patients (51%) had died. Patients with malignancy-associated HLH had the shortest median survival (160 days), however no statistically significant difference between subgroups was observed (p = 0.077). Platelets under 20*109/l and low albumin concentrations (< 20 g/l) were associated with poor overall and 30-day survival.CONCLUSION: Close multidisciplinary case consultation and cooperation is mandatory when treating adult HLH patients. Early contact with reference centers is recommended, especially in relapsing or refractory disease.
KW - Adolescent
KW - Adult
KW - Aged
KW - Aged, 80 and over
KW - Female
KW - Germany/epidemiology
KW - Humans
KW - Lymphohistiocytosis, Hemophagocytic/diagnosis
KW - Male
KW - Middle Aged
KW - Prognosis
KW - Registries
KW - Young Adult
U2 - 10.1007/s00432-020-03139-4
DO - 10.1007/s00432-020-03139-4
M3 - SCORING: Journal article
C2 - 32076823
VL - 146
SP - 1065
EP - 1077
JO - J CANCER RES CLIN
JF - J CANCER RES CLIN
SN - 0171-5216
IS - 4
ER -
