2024

Survival in primary hemophagocytic lymphohistiocytosis, 2016 to 2021: etoposide is better than its reputation
Böhm S, Wustrau K, Pachlopnik Schmid J, Prader S, Ahlman M, Yacobovich J, Beier R, Speckmann C, Behnisch W, Ifversen M, Jordan M, Marsh R, Naumann-Bartsch N, Mauz-Koerholz C, Honig M, Schulz A, Malinowska I, Hines M, Nichols K, Gil-Herrera J, Talano J, Crooks B, Formankova R, Jorch N, Bakhtiar S, Kühnle I, Streiter M, Nathrath M, Russo A, Duerken M, Lang P, Lindemans C, Henter J, Lehmberg K, Ehl S
BLOOD. 2024;143(10):872-881.

2023

Immunodeficiency with susceptibility to lymphoma with complex genotype affecting energy metabolism (FBP1, ACAD9) and vesicle trafficking (RAB27A)
Brauer N, Maruta Y, Lisci M, Strege K, Oschlies I, Nakamura H, Böhm S, Lehmberg K, Brandhoff L, Ehl S, Parvaneh N, Klapper W, Fukuda M, Griffiths G, Hennies H, Niehues T, Ammann S
FRONT IMMUNOL. 2023;14:1151166.

Development of a centralised triage centre for children with cancer and blood disorders in response to the humanitarian crisis in Ukraine
Salek M, Mueller A, Alanbousi I, Cepowska Z, Dutkiewicz M, Earl J, Evseev D, Kizyma R, Kliuchkivska K, Kolodrubiec J, Matczak K, Nogovitsyna Y, Oszer A, Pogorelyy M, Raciborska A, Rasul S, Sokolowski I, Sopilnyak A, Vinitsky A, Wlodarski M, Wobst N, Yakimkova T, Rodriguez-Galindo C, Wise P, Mlynarski W, Agulnik A
LANCET ONCOL. 2023;24(12):1315-1318.

MAP kinase activating death domain deficiency is a novel cause of impaired lymphocyte cytotoxicity
Schütze K, Groß M, Cornils K, Wustrau K, Schneppenheim S, Lenhartz H, Korenke G, Janka G, Ledig S, Müller I, Ehl S, Lehmberg K
BLOOD ADV. 2023;7(8):1531-1535.

Prospective Newborn Screening for SCID in Germany: A First Analysis by the Pediatric Immunology Working Group (API)
Speckmann C, Nennstiel U, Hönig M, Albert M, Ghosh S, Schuetz C, Brockow I, Hörster F, Niehues T, Ehl S, Wahn V, Borte S, Lehmberg K, Baumann U, Beier R, Krüger R, Bakhtiar S, Kuehl J, Klemann C, Kontny U, Holzer U, Meinhardt A, Morbach H, Naumann-Bartsch N, Rothoeft T, Kreins A, Davies E, Schneider D, Bernuth H, Klingebiel T, Hoffmann G, Schulz A, Hauck F
J CLIN IMMUNOL. 2023;43(5):965-978.

2022

Global effort to evacuate Ukrainian children with cancer and blood disorders who have been affected by war
Agulnik A, Kizyma R, Salek M, Wlodarski M, Pogorelyy M, Oszer A, Yakimkova T, Nogovitsyna Y, Dutkiewicz M, Dalle J, Dirksen U, Eggert A, Fernández-Teijeiro A, Greiner J, Kraal K, Mueller A, Sramkova L, Zecca M, Wise P, Mlynarski W
LANCET HAEMATOL. 2022;9(9):e645-e647.

Reduced-Intensity/Reduced-Toxicity Conditioning Approaches Are Tolerated in XIAP Deficiency but Patients Fare Poorly with Acute GVHD
Arnold D, Nofal R, Wakefield C, Lehmberg K, Wustrau K, Albert M, Morris E, Heimall J, Bunin N, Kumar A, Jordan M, Cole T, Choo S, Brettig T, Speckmann C, Ehl S, Salamonowicz M, Wahlstrom J, Rao K, Booth C, Worth A, Marsh R
J CLIN IMMUNOL. 2022;42(1):36-45.

Rubella vaccine-induced granulomas are a novel phenotype with incomplete penetrance of genetic defects in cytotoxicity
Groß M, Speckmann C, May A, Gajardo-Carrasco T, Wustrau K, Maier S, Panning M, Huzly D, Agaimy A, Bryceson Y, Choo S, Chow C, Dückers G, Fasth A, Fraitag S, Gräwe K, Haxelmans S, Holzinger D, Hudowenz O, Hübschen J, Khurana C, Kienle K, Klifa R, Korn K, Kutzner H, Lämmermann T, Ledig S, Lipsker D, Meeths M, Naumann-Bartsch N, Rascon J, Schänzer A, Seidl M, Tesi B, Vauloup-Fellous C, Vollmer-Kary B, Warnatz K, Wehr C, Neven B, Vargas P, Sepulveda F, Lehmberg K, Schmitt-Graeff A, Ehl S
J ALLERGY CLIN IMMUN. 2022;149(1):388-399.e4.

Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults
Hines M, von Bahr Greenwood T, Beutel G, Beutel K, Hays J, Horne A, Janka G, Jordan M, van Laar J, Lachmann G, Lehmberg K, Machowicz R, Miettunen P, La Rosée P, Shakoory B, Zinter M, Henter J
CRIT CARE MED. 2022;50(5):860-872.

Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT
Machowicz R, Suarez F, Wiktor-Jedrzejczak W, Eikema D, de Wreede L, Blok H, Isaksson C, Einsele H, Poiré X, van Dorp S, Nikolousis E, Johansson J, Kobbe G, Zecca M, Arnold R, Gerbitz A, Finke J, Díez-Martín J, Bonifazi F, McQuaker G, Lenhoff S, Rohrlich P, Theobald M, Ljungman P, Collin M, Albert M, Ehninger G, Carlson K, Halaburda K, Lehmberg K, Schönland S, Yakoub-Agha I, Gennery A, Lankester A, Kröger N
BONE MARROW TRANSPL. 2022;57(5):817-823.

Essential medicines for childhood cancer in Europe: a pan-European, systematic analysis by SIOPE
Otth M, Brack E, Kearns P, Kozhaeva O, Ocokoljic M, Schoot R, Vassal G
LANCET ONCOL. 2022;23(12):1537-1546.

2021

Is neutralization of IFN-γ sufficient to control inflammation in HLH?
Ehl S, von Bahr Greenwood T, Bergsten E, Fischer A, Henter J, Hines M, Lehmberg K, Janka G, Moshous D, Nichols K
PEDIATR BLOOD CANCER. 2021;68(3):e28886.

Chronisch-rezidivierende Gonarthritis und Autoimmunneutropenie
Fröhlich A, Lehmberg K, Pagel J, Peldschus K, Schoof B, Schulze-Sturm U, Speth F
arthritis + rheuma. 2021;41(6):387-392.

HSCT is effective in patients with PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome
Laberko A, Burlakov V, Maier S, Abinun M, Skinner R, Kozlova A, Suri D, Lehmberg K, Müller I, Balashov D, Novichkova G, Holzinger D, Gennery A, Shcherbina A
J ALLERGY CLIN IMMUN. 2021;148(1):250-255.e1.

Primary haemophagocytic lymphohistiocytosis (Chédiak-Higashi Syndrome) triggered by acute SARS-CoV-2 infection in a six-week-old infant
Lange M, Linden T, Müller H, Flasskuehler M, Koester H, Lehmberg K, Ledig S, Ehl S, Heep A, Beske F
BRIT J HAEMATOL. 2021;195(2):198-200.

Case Report: Hemophagocytic Lymphohistiocytosis and Non-Tuberculous Mycobacteriosis Caused by a Novel GATA2 Variant
Mika T, Vangala D, Eckhardt M, La Rosée P, Lange C, Lehmberg K, Wohlschläger C, Biskup S, Fuchs I, Mann J, Ehl S, Warnatz K, Schroers R
FRONT IMMUNOL. 2021;12:.

Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations
Thalhammer J, Kindle G, Nieters A, Rusch S, Seppänen M, Fischer A, Grimbacher B, Edgar D, Buckland M, Mahlaoui N, Ehl S
J ALLERGY CLIN IMMUN. 2021;148(5):1332-1341.e5.

Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia
Zielen S, Duecker R, Woelke S, Donath H, Bakhtiar S, Buecker A, Kreyenberg H, Huenecke S, Bader P, Mahlaoui N, Ehl S, El-Helou S, Pietrucha B, Plebani A, van der Flier M, van Aerde K, Kilic S, Reda S, Kostyuchenko L, McDermott E, Galal N, Pignata C, Pérez J, Laws H, Niehues T, Kutukculer N, Seidel M, Marques L, Ciznar P, Edgar J, Soler-Palacín P, von Bernuth H, Krueger R, Meyts I, Baumann U, Kanariou M, Grimbacher B, Hauck F, Graf D, Granado L, Prader S, Reisli I, Slatter M, Rodríguez-Gallego C, Arkwright P, Bethune C, Deripapa E, Sharapova S, Lehmberg K, Davies E, Schuetz C, Kindle G, Schubert R
J CLIN IMMUNOL. 2021;41(8):1878-1892.

2020

Functional flow cytometry of monocytes for routine diagnosis of innate primary immunodeficiencies
Ammann S, Fuchs S, Martin-Martin L, Castro C, Spielberger B, Klemann C, Elling R, Heeg M, Speckmann C, Hainmann I, Kaiser-Labusch P, Horneff G, Thalhammer J, Bredius R, Stadt U, Lehmberg K, Fuchs I, von Spee-Mayer C, Henneke P, Ehl S
J ALLERGY CLIN IMMUN. 2020;145(1):434-437.e4.

Stem cell transplantation for children with hemophagocytic lymphohistiocytosis: results from the HLH-2004 study
Bergsten E, Horne A, Hed Myrberg I, Aricó M, Astigarraga I, Ishii E, Janka G, Ladisch S, Lehmberg K, McClain K, Minkov M, Nanduri V, Rosso D, Sieni E, Winiarski J, Henter J
BLOOD ADV. 2020;4(15):3754-3766.

Hemophagocytic lymphohistiocytosis in adults: collaborative analysis of 137 cases of a nationwide German registry
Birndt S, Schenk T, Heinevetter B, Brunkhorst F, Maschmeyer G, Rothmann F, Weber T, Müller M, Panse J, Penack O, Schroers R, Braess J, Frickhofen N, Ehl S, Janka G, Lehmberg K, Pletz M, Hochhaus A, Ernst T, La Rosée P
J CANCER RES CLIN. 2020;146(4):1065-1077.

Neuroinflammatory Disease as an Isolated Manifestation of Hemophagocytic Lymphohistiocytosis
Blincoe A, Heeg M, Campbell P, Hines M, Khojah A, Klein-Gitelman M, Talano J, Speckmann C, Touzot F, Lankester A, Legger G, Rivière J, Garcia-Prat M, Alonso L, Putti M, Lehmberg K, Maier S, El Chazli Y, Elmaksoud M, Astigarraga I, Kurjane N, Bulina I, Kenina V, Bryceson Y, Rascon J, Lortie A, Goldstein G, Booth C, Worth A, Wassmer E, Schmitt E, Warren J, Bednarski J, Ali S, Chiang K, Krueger J, Henry M, Holland S, Marsh R, Ehl S, Haddad E
J CLIN IMMUNOL. 2020;40(6):901-916.

Targeted busulfan-based reduced-intensity conditioning and HLA-matched HSCT cure hemophagocytic lymphohistiocytosis
Felber M, Steward C, Kentouche K, Fasth A, Wynn R, Zeilhofer U, Haunerdinger V, Volkmer B, Prader S, Gruhn B, Ehl S, Lehmberg K, Müller D, Gennery A, Albert M, Hauck F, Rao K, Veys P, Hassan M, Lankester A, Schmid J, Hauri-Hohl M, Güngör T
BLOOD ADV. 2020;4(9):1998-2010.

Tuberculosis-Associated HLH in an 8-Month-Old Infant: A Case Report and Review
Hauch H, Skrzypek S, Woessmann W, Lehmberg K, Ehl S, Speckmann C, Schneck E, Koerholz D, Jux C, Neuhäuser C
FRONT PEDIATR. 2020;8:.

Distinct molecular response patterns of activating STAT3 mutations associate with penetrance of lymphoproliferation and autoimmunity
Jägle S, Heeg M, Grün S, Rensing-Ehl A, Maccari M, Klemann C, Jones N, Lehmberg K, Bettoni C, Warnatz K, Grimbacher B, Biebl A, Schauer U, Hague R, Neth O, Mauracher A, Pachlopnik Schmid J, Fabre A, Kostyuchenko L, Führer M, Lorenz M, Schwarz K, Rohr J, Ehl S
CLIN IMMUNOL. 2020;210:108316.

Incidence of SCID in Germany from 2014 to 2015 an ESPED* Survey on Behalf of the API*** Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland (German Paediatric Surveillance Unit) ** Arbeitsgemeinschaft Pädiatrische Immunologie
Shai S, Perez-Becker R, Andres O, Bakhtiar S, Bauman U, von Bernuth H, Classen C, Dückers G, El-Helou S, Gangfuß A, Ghosh S, Grimbacher B, Hauck F, Hoenig M, Husain R, Kindle G, Kipfmueller F, Klemann C, Krüger R, Lainka E, Lehmberg K, Lohrmann F, Morbach H, Naumann-Bartsch N, Oommen P, Schulz A, Seidemann K, Speckmann C, Sykora K, von Kries R, Niehues T
J CLIN IMMUNOL. 2020;40(5):708-717.

Ulceration in Prolidase Deficiency: Successful Treatment with Anticoagulants
Süßmuth K, Metze D, Muresan A, Lehmberg K, Zur Stadt U, Speckmann C, Park J, Marquardt T, Oji V, Goerge T
ACTA DERM-VENEREOL. 2020;100(1):adv00002.

Cell Versus Cytokine - Directed Therapies for Hemophagocytic Lymphohistiocytosis (HLH) in Inborn Errors of Immunity
Wegehaupt O, Wustrau K, Lehmberg K, Ehl S
FRONT IMMUNOL. 2020;11:.

Risk factors for mixed chimerism in children with hemophagocytic lymphohistiocytosis after reduced toxicity conditioning
Wustrau K, Greil J, Sykora K, Albert M, Burkhardt B, Lang P, Meisel R, Wössmann W, Beier R, Schulz A, Bader P, Chada M, Kühl J, Schlegel P, Speckmann C, Gruhn B, Seidel M, Wawer A, Ozga A, Janka G, Ehl S, Müller I, Lehmberg K
PEDIATR BLOOD CANCER. 2020;67(9):e28523.

2019

Zinkmangel als Ursache eines „Dekubitus“ beim Frühgeborenen?
Asselborn J, Thayssen M, Lehmberg K, Apostolidou S, Singer D
Z GEBURTSH NEONATOL. 2019;223(6):369-372.

A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis
Chellapandian D, Hines M, Zhang R, Jeng M, van den Bos C, Santa-María López V, Lehmberg K, Sieni E, Wang Y, Nakano T, Williams J, Fustino N, Astigarraga I, Dunkel I, Abla O, van Halteren A, Pei D, Cheng C, Weitzman S, Sung L, Nichols K
CANCER-AM CANCER SOC. 2019;125(6):963-971.

The German National Registry of Primary Immunodeficiencies (2012-2017)
El-Helou S, Biegner A, Bode S, Ehl S, Heeg M, Maccari M, Ritterbusch H, Speckmann C, Rusch S, Scheible R, Warnatz K, Atschekzei F, Beider R, Ernst D, Gerschmann S, Jablonka A, Mielke G, Schmidt R, Schürmann G, Sogkas G, Baumann U, Klemann C, Viemann D, von Bernuth H, Krüger R, Hanitsch L, Scheibenbogen C, Wittke K, Albert M, Eichinger A, Hauck F, Klein C, Rack-Hoch A, Sollinger F, Avila A, Borte M, Borte S, Fasshauer M, Hauenherm A, Kellner N, Müller A, Ülzen A, Bader P, Bakhtiar S, Lee J, Heß U, Schubert R, Wölke S, Zielen S, Ghosh S, Laws H, Neubert J, Oommen P, Hönig M, Schulz A, Steinmann S, Schwarz K, Dückers G, Lamers B, Langemeyer V, Niehues T, Shai S, Graf D, Müglich C, Schmalzing M, Schwaneck E, Tony H, Dirks J, Haase G, Liese J, Morbach H, Foell D, Hellige A, Wittkowski H, Masjosthusmann K, Geberzahn L, Hedrich C, Müller C, Rösen-Wolff A, Roesler J, Zimmermann A, Behrends U, Rieber N, Schauer U, Handgretinger R, Holzer U, Henes J, Kanz L, Boesecke C, Rockstroh J, Schwarze-Zander C, Wasmuth J, Dilloo D, Hülsmann B, Schönberger S, Schreiber S, Zeuner R, Ankermann T, von Bismarck P, Huppertz H, Kaiser-Labusch P, Greil J, Jakoby D, Kulozik A, Metzler M, Naumann-Bartsch N, Sobik B, Graf N, Heine S, Kobbe R, Lehmberg K, Müller I, Herrmann F, Horneff G, Klein A, Peitz J, Schmidt N, Bielack S, Groß-Wieltsch U, Classen C, Klasen J, Deutz P, Kamitz D, Lassay L, Tenbrock K, Wagner N, Bernbeck B, Brummel B, Lara-Villacanas E, Münstermann E, Schneider D, Tietsch N, Westkemper M, Weiß M, Kramm C, Kühnle I, Kullmann S, Girschick H, Specker C, Vinnemeier-Laubenthal E, Haenicke H, Schweigerer L, Müller T, Stiefel M, Belohradsky B, Soetedjo V, Kindle G, Grimbacher B
FRONT IMMUNOL. 2019;10:1272.

Haematopoietic Stem Cell Transplantation for Primary Haemophagocytic Lymphohistiocytosis
Lehmberg K, Moshous D, Booth C
FRONT PEDIATR. 2019;7:435.

Risikofaktoren für einen gemischten Chimerismus nach Stammzelltransplantation mit Treosulfan- oder Melphalanbasierter Konditionierung bei Kindern und Jugendlichen mit primärer Hämophagozytischer Lymphohistiozytose
Wustrau K, Albert M, Bader P, Beier R, Burkhardt B, Chada M, Greil J, Gruhn B, Kühl J, Lang P, Meisel R, Schulz A, Seidel M, Speckmann C, Sykora K, Wawer A, Wößmann W, Ozga A, Janka-Schaub G, Ehl S, Müller I, Lehmberg K
2019. Monatsschrift Kinderheilkunde > Ausgabe 7/2018 . 646-647.

2018

Recommendations for the Use of Etoposide-Based Therapy and Bone Marrow Transplantation for the Treatment of HLH: Consensus Statements by the HLH Steering Committee of the Histiocyte Society
Ehl S, Astigarraga I, von Bahr Greenwood T, Hines M, Horne A, Ishii E, Janka G, Jordan M, La Rosée P, Lehmberg K, Machowicz R, Nichols K, Sieni E, Wang Z, Henter J
J ALLER CL IMM-PRACT. 2018;6(5):1508-1517.

T-cell gene therapy for perforin deficiency corrects cytotoxicity defects and prevents hemophagocytic lymphohistiocytosis manifestations
Ghosh S, Carmo M, Calero-Garcia M, Ricciardelli I, Bustamante Ogando J, Blundell M, Schambach A, Ashton-Rickardt P, Booth C, Ehl S, Lehmberg K, Thrasher A, Gaspar H
J ALLERGY CLIN IMMUN. 2018;142(3):904-913.

Is an infectious trigger always required for primary hemophagocytic lymphohistiocytosis? Lessons from in utero and neonatal disease
Heeg M, Ammann S, Klemann C, Panning M, Falcone V, Hengel H, Lehmberg K, Zur Stadt U, Wustrau K, Janka G, Ehl S
PEDIATR BLOOD CANCER. 2018;65(11):e27344.

Hämophagozytische Lymphohistiozytose
Lehmberg K
2018. Referenz Hämatologie. Kreuzer K (eds.). Georg Thieme Verlag KG, .

Histiozytäre Erkrankungen
Lehmberg K
2018. Pädiatrische Hämatologie und Onkologie. Niemeyer C, Eggert A (eds.). 2. ed. Springer, .

Neuroimaging findings in children with hereditary hemophagocytic lymphohistiocytosis – report from the German reference center
Löbel U, Wustrau K, Müller I, Janka-Schaub G, Fiehler J, Lehmberg K
2018. NeuroRAD 2018 - 53. Jahrestagung der Deutschen Gesellschaft für Neuroradiologe e.V.. .

Treatment dilemmas in asymptomatic children with primary hemophagocytic lymphohistiocytosis
Lucchini G, Marsh R, Gilmour K, Worth A, Nademi Z, Rao A, Booth C, Amrolia P, Silva J, Chiesa R, Wynn R, Lehmberg K, Astigarraga I, Güngör T, Stary J, Moshous D, Ifversen M, Zinn D, Jordan M, Kumar A, Yasumi T, Veys P, Rao K
BLOOD. 2018;132(19):2088-2096.

Autoinflammatory mutation in NLRC4 reveals a leucine-rich repeat (LRR)-LRR oligomerization interface
Moghaddas F, Zeng P, Zhang Y, Schützle H, Brenner S, Hofmann S, Berner R, Zhao Y, Lu B, Chen X, Zhang L, Cheng S, Winkler S, Lehmberg K, Canna S, Czabotar P, Wicks I, De Nardo D, Hedrich C, Zeng H, Masters S
J ALLERGY CLIN IMMUN. 2018;142(6):1956-1967.e6.

Risk factors for mixed chimerism after stem cell transplantation with treosulfan or melphalan based conditioning regimens in children and adolescents with primary hemophagocytic lymphohistocytosis
Wustrau K, Albert M, Bader P, Beier R, Burkhardt B, Chada M, Greil J, Gruhn B, Kühl J, Lang P, Meisel R, Schulz A, Seidel M, Speckmann C, Sykora K, Wawer A, Wößmann W, Ozga A, Ehl S, Müller I, Lehmberg K
2018. Bone Marrow Transplantation (2018) Abstracts Collection. .

2017

Primary and secondary hemophagocytic lymphohistiocytosis have different patterns of T-cell activation, differentiation and repertoire
Ammann S, Lehmberg K, Zur Stadt U, Janka-Schaub G, Rensing-Ehl A, Klemann C, Heeg M, Bode S, Fuchs I, Ehl S
EUR J IMMUNOL. 2017;47(2):364-373.

Effective Immunological Guidance of Genetic Analyses Including Exome Sequencing in Patients Evaluated for Hemophagocytic Lymphohistiocytosis
Ammann S, Lehmberg K, Zur Stadt U, Klemann C, Bode S, Speckmann C, Janka G, Wustrau K, Rakhmanov M, Fuchs I, Hennies H, Ehl S
J CLIN IMMUNOL. 2017;37(8):770-780.

Confirmed efficacy of etoposide and dexamethasone in HLH Treatment: long-term results of the cooperative HLH-2004 study
Bergsten E, Horne A, Aricó M, Astigarraga I, Egeler R, Filipovich A, Ishii E, Janka G, Ladisch S, Lehmberg K, McClain K, Minkov M, Montgomery S, Nanduri V, Rosso D, Henter J
BLOOD. 2017;130(25):2728-2738.

Differences in Granule Morphology yet Equally Impaired Exocytosis among Cytotoxic T Cells and NK Cells from Chediak-Higashi Syndrome Patients
Chiang S, Wood S, Tesi B, Akar H, Al-Herz W, Ammann S, Belen F, Caliskan U, Kaya Z, Lehmberg K, Patiroglu T, Tokgoz H, Ünüvar A, Introne W, Henter J, Nordenskjöld M, Ljunggren H, Meeths M, Ehl S, Krzewski K, Bryceson Y
FRONT IMMUNOL. 2017;8:426.

Hemophagocytic lymphohistiocytosis as presenting manifestation of profound combined immunodeficiency due to an ORAI1 mutation
Klemann C, Ammann S, Heizmann M, Fuchs S, Bode S, Heeg M, Fuchs H, Lehmberg K, Zur Stadt U, Roll C, Vraetz T, Speckmann C, Lorenz M, Schwarz K, Rohr J, Feske S, Ehl S
J ALLERGY CLIN IMMUN. 2017;140(6):1721-1724.

Evolution of disease activity and biomarkers on and off rapamycin in 28 patients with autoimmune lymphoproliferative syndrome
Klemann C, Esquivel M, Magerus-Chatinet A, Lorenz M, Fuchs I, Neveux N, Castelle M, Rohr J, Bettoni da Cunha C, Ebinger M, Kobbe R, Kremens B, Kollert F, Gambineri E, Lehmberg K, Seidel M, Siepermann K, Voelker T, Schuster V, Goldacker S, Schwarz K, Speckmann C, Picard C, Fischer A, Rieux-Laucat F, Ehl S, Rensing-Ehl A, Neven B
HAEMATOLOGICA. 2017;102(2):e52-e56.

Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series
Lehmberg K, Hassenpflug W, Klaassen I, Hillebrand G, Oyen F, Budde U, Singer D, Schneppenheim R
Z GEBURTSH NEONATOL. 2017;221(1):39-42.

Development and Initial Validation of the Macrophage Activation Syndrome/Primary Hemophagocytic Lymphohistiocytosis Score, a Diagnostic Tool that Differentiates Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome
Minoia F, Bovis F, Davì S, Insalaco A, Lehmberg K, Shenoi S, Weitzman S, Espada G, Gao Y, Anton J, Kitoh T, Kasapcopur O, Sanner H, Merino R, Astigarraga I, Alessio M, Jeng M, Chasnyk V, Nichols K, Huasong Z, Li C, Micalizzi C, Ruperto N, Martini A, Cron R, Ravelli A, Horne A
J PEDIATR-US. 2017;189:72-78.e3.

Mutations in EXTL3 Cause Neuro-immuno-skeletal Dysplasia Syndrome
Oud M, Tuijnenburg P, Hempel M, van Vlies N, Ren Z, Ferdinandusse S, Jansen M, Santer R, Johannsen J, Bacchelli C, Alders M, Li R, Davies R, Dupuis L, Cale C, Wanders R, Pals S, Ocaka L, James C, Müller I, Lehmberg K, Strom T, Engels H, Williams H, Beales P, Roepman R, Dias P, Brunner H, Cobben J, Hall C, Hartley T, Le Quesne Stabej P, Mendoza-Londono R, Davies E, de Sousa S, Lessel D, Arts H, Kuijpers T
AM J HUM GENET. 2017;100(2):281-296.

2016

Mutations in AP3D1 associated with immunodeficiency and seizures define a new type of Hermansky-Pudlak syndrome
Ammann S, Schulz A, Krägeloh-Mann I, Dieckmann N, Niethammer K, Fuchs S, Eckl K, Plank R, Werner R, Altmüller J, Thiele H, Nürnberg P, Bank J, Strauss A, von Bernuth H, Zur Stadt U, Grieve S, Griffiths G, Lehmberg K, Hennies H, Ehl S
BLOOD. 2016;127(8):997-1006.

Unterarmgangrän bei "normaler" Gerinnung.
Blohm M, Lehmberg K, Schrum J, Helmke K, Ridderbusch I, Schneppenheim R, Singer D
Z GEBURTSH NEONATOL. 2016;220:133-134.

The minimum required level of donor chimerism in hereditary hemophagocytic lymphohistiocytosis
Hartz B, Marsh R, Rao K, Henter J, Jordan M, Filipovich L, Bader P, Beier R, Burkhardt B, Meisel R, Schulz A, Winkler B, Albert M, Greil J, Karasu G, Woessmann W, Corbaciologlu S, Gruhn B, Holter W, Kühl J, Lang P, Seidel M, Veys P, Löfstedt A, Ammann S, Ehl S, Janka G, Müller I, Lehmberg K
BLOOD. 2016;127(25):3281-90.

Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens E, De Benedetti F, Filipovic A, Grom A, Henter J, Ilowite N, Jordan M, Khubchandani R, Kitoh T, Lehmberg K, Lovell D, Miettunen P, Nichols K, Ozen S, Pachlopnik Schmid J, Ramanan A, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner H, Martini A, Ruperto N, Cron R
BMJ OPEN. 2016;2(1):e000161.

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative
Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens E, De Benedetti F, Filipovic L, Grom A, Henter J, Ilowite N, Jordan M, Khubchandani R, Kitoh T, Lehmberg K, Lovell D, Miettunen P, Nichols K, Ozen S, Pachlopnik Schmid J, Ramanan A, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner H, Martini A, Ruperto N, Cron R
ANN RHEUM DIS. 2016;75(3):481-9.

EZH2 mutation in an adolescent with Weaver syndrome developing acute myeloid leukemia and secondary hemophagocytic lymphohistiocytosis
Usemann J, Ernst T, Schäfer V, Lehmberg K, Seeger K
AM J MED GENET A. 2016;170(5):1274-7.

2015

The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis
Bode S, Ammann S, Al-Herz W, Bataneant M, Dvorak C, Gehring S, Gennery A, Gilmour K, Gonzalez-Granado L, Groß-Wieltsch U, Ifversen M, Lingman-Framme J, Matthes-Martin S, Mesters R, Meyts I, van Montfrans J, Pachlopnik Schmid J, Pai S, Soler-Palacin P, Schuermann U, Schuster V, Seidel M, Speckmann C, Stepensky P, Sykora K, Tesi B, Vraetz T, Waruiru C, Bryceson Y, Moshous D, Lehmberg K, Jordan M, Ehl S
HAEMATOLOGICA. 2015;100(7):978-88.

Novel Patient with Late-Onset Familial Hemophagocytic Lymphohistiocytosis with STXBP2 Mutations Presenting with Autoimmune Hepatitis, Neurological Manifestations and Infections Associated with Hypogammaglobulinemia
Esmaeilzadeh H, Bemanian M, Nabavi M, Arshi S, Fallahpour M, Fuchs I, Zur Stadt U, Warnatz K, Ammann S, Ehl S, Lehmberg K, Rezaei N
J CLIN IMMUNOL. 2015;35(1):22-25.

Out of the haemophagocytic lymphohistiocytosis niche
Lehmberg K
LANCET HAEMATOL. 2015;2(12):e508-9.

Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies
Lehmberg K, Nichols K, Henter J, Girschikofsky M, Greenwood T, Jordan M, Kumar A, Minkov M, La Rosée P, Weitzman S
HAEMATOLOGICA. 2015;100(8):997-1004.

Malignancy-associated haemophagocytic lymphohistiocytosis in children and adolescents
Lehmberg K, Sprekels B, Nichols K, Woessmann W, Müller I, Suttorp M, Bernig T, Beutel K, Bode S, Kentouche K, Kolb R, Längler A, Minkov M, Schilling F, Schmid I, Vieth S, Ehl S, Zur Stadt U, Janka G
BRIT J HAEMATOL. 2015;170(4):539-49.

2014

Hemophagocytic lymphohistiocytosis in imported pediatric visceral leishmaniasis in a nonendemic area
Bode S, Bogdan C, Beutel K, Behnisch W, Greiner J, Henning S, Jorch N, Jankofsky M, Jakob M, Schmid I, Veelken N, Vraetz T, Janka G, Ehl S, Lehmberg K
J PEDIATR-US. 2014;165(1):147-153.e1.

Hemophagocytic syndromes - An update
Janka-Schaub G, Lehmberg K
BLOOD REV. 2014;28(4):135-142.

Treosulfan-based conditioning regimen for children and adolescents with hemophagocytic lymphohistiocytosis
Lehmberg K, Albert M, Beier R, Beutel K, Gruhn B, Kröger N, Meisel R, Schulz A, Stachel D, Woessmann W, Janka G, Müller I
HAEMATOLOGICA. 2014;99(1):180-4.

Determination of an appropriate cut-off value for ferritin in the diagnosis of hemophagocytic lymphohistiocytosis
Lehmberg K, McClain K, Janka-Schaub G, Allen C
PEDIATR BLOOD CANCER. 2014.

Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic Arthritis: a multinational, multicenter study of 362 patients
Minoia F, Davì S, Horne A, Demirkaya E, Bovis F, Li C, Lehmberg K, Weitzman S, Insalaco A, Wouters C, Shenoi S, Espada G, Ozen S, Anton J, Khubchandani R, Russo R, Pal P, Kasapcopur O, Miettunen P, Maritsi D, Merino R, Shakoory B, Alessio M, Chasnyk V, Sanner H, Gao Y, Huasong Z, Kitoh T, Avcin T, Fischbach M, Frosch M, Grom A, Huber A, Jelusic M, Sawhney S, Uziel Y, Ruperto N, Martini A, Cron R, Ravelli A
ARTHRITIS RHEUMATOL. 2014;66(11):3160-9.

2013

Fulminant Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
Bohne S, Kentouche K, Petersen I, Fritzenwanger M, Pletz M, Lehmberg K, Paul L, Guntinas-Lichius O
LARYNGOSCOPE. 2013;123(2):362-365.

Syntaxin binding mechanism and disease-causing mutations in Munc18-2
Hackmann Y, Graham S, Ehl S, Höning S, Lehmberg K, Aricò M, Owen D, Griffiths G
P NATL ACAD SCI USA. 2013;110(47):E4482-91.

Hemophagocytic lymphohistiocytosis - pathogenesis and treatment
Janka-Schaub G, Lehmberg K
HEMATOL-AM SOC HEMAT. 2013;2013:605-11.

The risk of hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type 2
Jessen B, Bode S, Ammann S, Chakravorty S, Davies G, Diestelhorst J, Frei-Jones M, Gahl W, Gochuico B, Griese M, Griffiths G, Janka-Schaub G, Klein C, Kögl T, Kurnik K, Lehmberg K, Maul-Pavicic A, Mumford A, Pace D, Parvaneh N, Rezaei N, de Saint Basile G, Schmitt-Graeff A, Schwarz K, Karasu G, Zieger B, zur Stadt U, Aichele P, Ehl S
BLOOD. 2013;121(15):2943-51.

Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis.
Lehmberg K, Ehl S
BRIT J HAEMATOL. 2013;160(3):275-287.

Administration of recombinant erythropoietin alone does not improve the phenotype in iron refractory iron deficiency anemia patients.
Lehmberg K, Grosse R, Muckenthaler M, Altamura S, Nielsen P, Schmid H, Graubner U, Oyen F, Zeller W, Schneppenheim R, Janka-Schaub G
ANN HEMATOL. 2013;92(3):387-394.

Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis
Lehmberg K, Pink I, zu Eulenburg C, Beutel K, Maul-Pavicic A, Janka-Schaub G
J PEDIATR-US. 2013;162(6):1245-51.

Allogeneic hematopoietic cell transplantation for XIAP deficiency: an international survey reveals poor outcomes.
Marsh R, Rao K, Satwani P, Lehmberg K, Müller I, Li D, Kim M, Fischer A, Latour S, Sedlacek P, Barlogis V, Hamamoto K, Kanegane H, Milanovich S, Margolis D, Dimmock D, Casper J, Douglas D, Amrolia P, Veys P, Kumar A, Jordan M, Bleesing J, Filipovich A
BLOOD. 2013;121(6):877-883.

Sequential decisions on FAS sequencing guided by biomarkers in patients with lymphoproliferation and autoimmune cytopenia
Rensing-Ehl A, Janda A, Lorenz M, Gladstone B, Fuchs I, Abinun M, Albert M, Butler K, Cant A, Cseh A, Ebinger M, Goldacker S, Hambleton S, Hebart H, Houet L, Kentouche K, Kühnle I, Lehmberg K, Mejstrikova E, Niemeyer C, Minkov M, Neth O, Dückers G, Owens S, Rösler J, Schilling F, Schuster V, Seidel M, Smisek P, Sukova M, Svec P, Wiesel T, Gathmann B, Schwarz K, Vach W, Ehl S, Speckmann C
HAEMATOLOGICA. 2013;98(12):1948-55.

X-linked inhibitor of apoptosis (XIAP) deficiency: the spectrum of presenting manifestations beyond hemophagocytic lymphohistiocytosis
Speckmann C, Lehmberg K, Albert M, Damgaard R, Fritsch M, Gyrd-Hansen M, Rensing-Ehl A, Vraetz T, Grimbacher B, Salzer U, Fuchs I, Ufheil H, Belohradsky B, Hassan A, Cale C, Elawad M, Strahm B, Schibli S, Lauten M, Kohl M, Meerpohl J, Rodeck B, Kolb R, Eberl W, Soerensen J, von Bernuth H, Lorenz M, Schwarz K, zur Stadt U, Ehl S
CLIN IMMUNOL. 2013;149(1):133-41.

Persistent defective membrane trafficking in epithelial cells of patients with familial hemophagocytic lymphohistiocytosis type 5 due to STXBP2/MUNC18-2 mutations
Stepensky P, Bartram J, Barth T, Lehmberg K, Walther P, Amann K, Philips A, Beringer O, zur Stadt U, Schulz A, Amrolia P, Weintraub M, Debatin K, Hoenig M, Posovszky C
PEDIATR BLOOD CANCER. 2013;60(7):1215-22.

Clinical phenotype and functional analysis of a rare XIAP/BIRC4 mutation
Vieth S, Ammann S, Schwarz K, Härtel C, Schultz C, Lehmberg K, Lauten M
KLIN PADIATR. 2013;225(6):343-6.

2012

Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis.
Bode S, Lehmberg K, Maul-Pavicic A, Vraetz T, Janka-Schaub G, Zur Stadt U, Ehl S
ARTHRITIS RES THER. 2012;14(3):213.

A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes.
Bryceson Y, Pende D, Maul-Pavicic A, Gilmour K, Ufheil H, Vraetz T, Chiang S, Marcenaro S, Meazza R, Bondzio I, Walshe D, Janka-Schaub G, Lehmberg K, Beutel K, Zur Stadt U, Binder N, Arico M, Moretta L, Henter J, Ehl S
BLOOD. 2012;119(12):2754-2763.

Distinct mutations in STXBP2 are associated with variable clinical presentations in patients with familial hemophagocytic lymphohistiocytosis type 5 (FHL5)
Pagel J, Beutel K, Lehmberg K, Koch F, Maul-Pavicic A, Rohlfs A, Al-Jefri A, Beier R, Lilian B, Ehlert K, Gross-Wieltsch U, Jorch N, Kremens B, Pekrun A, Sparber-Sauer M, Mejstrikova E, Wawer A, Ehl S, Zur Stadt U, Janka-Schaub G
BLOOD. 2012;119(25):6016-6024.

Risk factors for early death in children with haemophagocytic lymphohistiocytosis.
Trottestam H, Berglöf E, Horne A, Onelöv E, Beutel K, Lehmberg K, Sieni E, Silfverberg T, Aricò M, Janka-Schaub G, Henter J
ACTA PAEDIATR. 2012;101(3):313-318.

2011

Subtle differences in CTL cytotoxicity determine susceptibility to hemophagocytic lymphohistiocytosis in mice and humans with Chediak-Higashi syndrome.
Jessen B, Maul-Pavicic A, Ufheil H, Vraetz T, Enders A, Lehmberg K, Längler A, Gross-Wieltsch U, Bay A, Kaya Z, Bryceson Y, Koscielniak E, Badawy S, Davies G, Hufnagel M, Schmitt-Graeff A, Aichele P, Zur Stadt U, Schwarz K, Ehl S
BLOOD. 2011;118(17):4620-4629.

2009

Pancytopenia in a 4-year-old boy. 22q11.2 microdeletion syndrome.
Lehmberg K, Rohr J, Schneppenheim R, Sommerfeld K, Janka-Schaub G
ACTA PAEDIATR. 2009;98(5):769-770, 912-914.

2007

From neonates to adolescents--the diagnostic significance of pitted erythrocytes in hyposplenic and asplenic children.
Lehmberg K, Steinhausen B, Janka-Schaub G
KLIN PADIATR. 2007;219(6):339-342.

2005

Resistance to cytarabine and gemcitabine and in vitro selection of transduced cells after retroviral expression of cytidine deaminase in human hematopoietic progenitor cells.
Bardenheuer W, Lehmberg K, Rattmann I, Brueckner A, Schneider A, Sorg U, Seeber S, Moritz T, Flasshove M
LEUKEMIA. 2005;19(12):2281-2288.

Letzte Aktualisierung aus dem FIS: 21.12.2024 - 03:11 Uhr