Skip to main content
Hauptrubriken
CME Facharzt-Training Webinare Zeitschriften Bücher e.Medpedia Podcast
Service
Jobbörse Info & Hilfe
Fachgebiete
Anästhesiologie Allgemeinmedizin Arbeitsmedizin Augenheilkunde Chirurgie Dermatologie Gynäkologie und Geburtshilfe HNO Innere Medizin Kardiologie Neurologie Onkologie und Hämatologie Orthopädie und Unfallchirurgie Pädiatrie Pathologie Psychiatrie Radiologie Rechtsmedizin Urologie Zahnmedizin
Themen
Typ-2-Diabetes und Adipositas Klimawandel und Gesundheit Neues aus dem Markt COVID-19 Praxis und Beruf Seltene Erkrankungen GOÄ & EBM Panorama
Sammlungen
Kasuistiken Algorithmen & Infografiken Blickdiagnosen Arthropedia FlapFinder (für Dermatochirurgen) Videos Kongressberichterstattung Medizin für Apothekerinnen und Apotheker Für Ärztinnen und Ärzte in Weiterbildung Für Medizinstudierende

Live-Webinar "Urologie und Sexualmedizin in der Praxis"

Häufige urologisch-sexualmedizinische Themen in der Praxis sind das Thema des MMW-Webinars am 5. Juni von 17.30 bis 19 Uhr. Konkret geht es um die Frage, wann bei Harnwegsinfektionen auf Antibiotika verzichtet werden kann, und um ein Update zur Therapie von Libido- und Potenzstörungen des Mannes. Der dritte Vortrag behandelt sexuell übertragbare Krankheiten. Stellen Sie Ihre Fragen an die Experten und sammeln Sie 2 CME-Punkte. Jetzt gleich anmelden!
Erweiterte Suche
Anmelden
nach oben
Medical Oncology
Erschienen in: Medical Oncology 4/2013

01.12.2013 | Review Article

Hemophagocytic lymphohistiocytosis (HLH): a review of literature

verfasst von: Rohtesh S. Mehta, Roy E. Smith

Erschienen in: Medical Oncology | Ausgabe 4/2013

Einloggen, um Zugang zu erhalten

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease in children and an exceptionally rare occurrence in adults. It is categorized broadly into primary (familial) or the secondary types; the latter being associated most commonly with an underlying malignancy. HLH carries a high rate of mortality, and the treatment itself is associated with significant morbidity and risk of mortality. A high degree of suspicion for the diagnosis, early treatment and aggressive supportive care is critical for management. We present a comprehensive review of literature describing the clinical features, diagnosis, management and outcome of HLH.
Literatur
1.
Henter JI, Arico M, Egeler RM, Elinder G, Favara BE, Filipovich AH, et al. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. Med Pediatr Oncol. 1997;28:342–7.CrossRefPubMed
2.
Clementi R, Emmi L, Maccario R, Liotta F, Moretta L, Danesino C, et al. Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Blood. 2002;100:2266–7.CrossRefPubMed
3.
Nagafuji K, Nonami A, Kumano T, Kikushige Y, Yoshimoto G, Takenaka K, et al. Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis. Haematologica. 2007;92:978–81.CrossRefPubMed
4.
Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology (Oxford). 2008;47:1686–91.CrossRef
5.
Kuzmanova SI. The macrophage activation syndrome: a new entity, a potentially fatal complication of rheumatic disorders. Folia Med (Plovdiv). 2005;47:21–5.
6.
Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124–31.CrossRefPubMed
7.
Karras A, Thervet E, Legendre C. Hemophagocytic syndrome in renal transplant recipients: report of 17 cases and review of literature. Transplantation. 2004;77:238–43.CrossRefPubMed
8.
Taniai N, Akimaru K, Kawano Y, Mizuguchi Y, Shimizu T, Takahashi T, et al. Hemophagocytic syndrome after living-donor liver transplantation for fulminant liver failure: a case report. Hepatogastroenterology. 2005;52:923–6.PubMed
9.
Boelens JJ, Lazo G, Gaiser JF, Wulffraat NM. Epstein-Barr virus-associated haemophagocytic lympho-histiocytosis after stem cell transplantation. Bone Marrow Transpl. 2006;38:709–10.CrossRef
10.
11.
Coffey AJ, Brooksbank RA, Brandau O, Oohashi T, Howell GR, Bye JM, et al. Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene. Nat Genet. 1998;20:129–35.CrossRefPubMed
12.
Menasche G, Pastural E, Feldmann J, Certain S, Ersoy F, Dupuis S, et al. Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome. Nat Genet. 2000;25:173–6.CrossRefPubMed
13.
Barrat FJ, Auloge L, Pastural E, Lagelouse RD, Vilmer E, Cant AJ, et al. Genetic and physical mapping of the Chediak-Higashi syndrome on chromosome 1q42-43. Am J Hum Genet. 1996;59:625–32.PubMedCentralPubMed
14.
Dell’Angelica EC, Shotelersuk V, Aguilar RC, Gahl WA, Bonifacino JS. Altered trafficking of lysosomal proteins in Hermansky-Pudlak syndrome due to mutations in the beta 3A subunit of the AP-3 adaptor. Mol Cell. 1999;3:11–21.CrossRefPubMed
15.
Pachlopnik Schmid J, Canioni D, Moshous D, Touzot F, Mahlaoui N, Hauck F, et al. Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency). Blood. 2011;117:1522–9.CrossRefPubMed
16.
Kaplan J, De Domenico I, Ward DM. Chediak-Higashi syndrome. Curr Opin Hematol. 2008;15:22–9.CrossRefPubMed
17.
Rubin CM, Burke BA, McKenna RW, McClain KL, White JG, Nesbit ME Jr, et al. The accelerated phase of Chediak-Higashi syndrome. An expression of the virus-associated hemophagocytic syndrome? Cancer. 1985;56:524–30.CrossRefPubMed
18.
Griscelli C, Durandy A, Guy-Grand D, Daguillard F, Herzog C, Prunieras M. A syndrome associating partial albinism and immunodeficiency. Am J Med. 1978;65:691–702.CrossRefPubMed
19.
Mancini AJ, Chan LS, Paller AS. Partial albinism with immunodeficiency: Griscelli syndrome: report of a case and review of the literature. J Am Acad Dermatol. 1998;38:295–300.CrossRefPubMed
20.
Wei ML. Hermansky-Pudlak syndrome: a disease of protein trafficking and organelle function. Pigment Cell Res. 2006;19:19–42.CrossRefPubMed
21.
Enders A, Zieger B, Schwarz K, Yoshimi A, Speckmann C, Knoepfle EM, et al. Lethal hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type II. Blood. 2006;108:81–7.CrossRefPubMed
22.
Henter JI, Elinder G, Soder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand. 1991;80:428–35.CrossRefPubMed
23.
Chang CS, Wang CH, Su IJ, Chen YC, Shen MC. Hematophagic histiocytosis: a clinicopathologic analysis of 23 cases with special reference to the association with peripheral T-cell lymphoma. J Formos Med Assoc. 1994;93:421–8.PubMed
24.
Veerakul G, Sanpakit K, Tanphaichitr VS, Mahasandana C, Jirarattanasopa N. Secondary hemophagocytic lymphohistiocytosis in children: an analysis of etiology and outcome. J Med Assoc Thai. 2002;85(Suppl 2):S530–41.PubMed
25.
Han AR, Lee HR, Park BB, Hwang IG, Park S, Lee SC, et al. Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome. Ann Hematol. 2007;86:493–8.CrossRefPubMed
26.
Celkan T, Berrak S, Kazanci E, Ozyurek E, Unal S, Ucar C, et al. Malignancy-associated hemophagocytic lymphohistiocytosis in pediatric cases: a multicenter study from Turkey. Turk J Pediatr. 2009;51:207–13.PubMed
27.
Osugi Y, Hara J, Tagawa S, Takai K, Hosoi G, Matsuda Y, et al. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood. 1997;89:4100–3.PubMed
28.
Takada H, Ohga S, Mizuno Y, Nomura A, Hara T. Increased IL-16 levels in hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol. 2004;26:567–73.CrossRefPubMed
29.
Takada H, Nomura A, Ohga S, Hara T. Interleukin-18 in hemophagocytic lymphohistiocytosis. Leuk Lymphoma. 2001;42:21–8.CrossRefPubMed
30.
Takada H, Takahata Y, Nomura A, Ohga S, Mizuno Y, Hara T. Increased serum levels of interferon-gamma-inducible protein 10 and monokine induced by gamma interferon in patients with haemophagocytic lymphohistiocytosis. Clin Exp Immunol. 2003;133:448–53.CrossRefPubMedCentralPubMed
31.
Trottestam H, Horne A, Arico M, Egeler RM, Filipovich AH, Gadner H, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood. 2011;118:4577–84.CrossRefPubMedCentralPubMed
32.
Okamoto M, Yamaguchi H, Isobe Y, Yokose N, Mizuki T, Tajika K, et al. Analysis of triglyceride value in the diagnosis and treatment response of secondary hemophagocytic syndrome. Intern Med. 2009;48:775–81.CrossRefPubMed
33.
Horne A, Trottestam H, Arico M, Egeler RM, Filipovich AH, Gadner H, et al. Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis. Br J Haematol. 2008;140:327–35.CrossRefPubMed
34.
Hallahan AR, Carpenter PA, O’Gorman-Hughes DW, Vowels MR, Marshall GM. Haemophagocytic lymphohistiocytosis in children. J Paediatr Child Health. 1999;35:55–9.CrossRefPubMed
35.
Henter JI, Elinder G. Cerebromeningeal haemophagocytic lymphohistiocytosis. Lancet. 1992;339:104–7.CrossRefPubMed
36.
Tong H, Ren Y, Liu H, Xiao F, Mai W, Meng H, et al. Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome: comparison of T-cell lymphoma with and without hemophagocytic syndrome. Leuk Lymphoma. 2008;49:81–7.CrossRefPubMed
37.
Risdall RJ, McKenna RW, Nesbit ME, Krivit W, Balfour HH Jr, Simmons RL, et al. Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer. 1979;44:993–1002.CrossRefPubMed
38.
Takahashi N, Miura I, Chubachi A, Miura AB, Nakamura S. A clinicopathological study of 20 patients with T/natural killer (NK)-cell lymphoma-associated hemophagocytic syndrome with special reference to nasal and nasal-type NK/T-cell lymphoma. Int J Hematol. 2001;74:303–8.CrossRefPubMed
39.
Shin HJ, Chung JS, Lee JJ, Sohn SK, Choi YJ, Kim YK, et al. Treatment outcomes with CHOP chemotherapy in adult patients with hemophagocytic lymphohistiocytosis. J Korean Med Sci. 2008;23:439–44.CrossRefPubMedCentralPubMed
40.
Fitzgerald NE, MacClain KL. Imaging characteristics of hemophagocytic lymphohistiocytosis. Pediatr Radiol. 2003;33:392–401.CrossRefPubMed
41.
Zhang K, Filipovich AH, Johnson J, Marsh RA, Villanueva J. Hemophagocytic lymphohistiocytosis, familial. In: Pagon RA, Bird TD, Dolan CR, Stephens K, Adam MP, editors. SourceGeneReviews™ [Internet]. Seattle, WA: University of Washington, Seattle; 1993–2006 [updated 2010 Sep 07].
42.
Ambruso DR, Hays T, Zwartjes WJ, Tubergen DG, Favara BE. Successful treatment of lymphohistiocytic reticulosis with phagocytosis with epipodophyllotoxin VP 16-213. Cancer. 1980;45:2516–20.CrossRefPubMed
43.
Janka GE. Familial hemophagocytic lymphohistiocytosis: therapy in the German experience. Pediatr Hematol Oncol. 1989;6:227–31.CrossRefPubMed
44.
Henter JI, Elinder G, Finkel Y, Soder O. Successful induction with chemotherapy including teniposide in familial erythrophagocytic lymphohistiocytosis. Lancet. 1986;2:1402.CrossRefPubMed
45.
Stephan JL, Donadieu J, Ledeist F, Blanche S, Griscelli C, Fischer A. Treatment of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins, steroids, and cyclosporin A. Blood. 1993;82:2319–23.PubMed
46.
Thompson PA, Allen CE, Horton T, Jones JY, Vinks AA, McClain KL. Severe neurologic side effects in patients being treated for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2009;52:621–5.CrossRefPubMedCentralPubMed
47.
Marsh RA, Allen CE, McClain KL, Weinstein JL, Kanter J, Skiles J, et al. Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab. Pediatr Blood Cancer. 2013;60:101–9.
48.
Strout MP, Seropian S, Berliner N. Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis. Nat Rev Clin Oncol. 2010;7:415–20.CrossRefPubMed
49.
Mahlaoui N, Ouachee-Chardin M, de Saint Basile G, Neven B, Picard C, Blanche S, et al. Immunotherapy of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins: a single-center retrospective report of 38 patients. Pediatrics. 2007;120:e622–8.CrossRefPubMed
50.
Verbsky JW, Grossman WJ. Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives. Ann Med. 2006;38:20–31.CrossRefPubMed
51.
Henter JI, Samuelsson-Horne A, Aricò M, Egeler RM, Elinder G, Filipovich AH, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002;100:2367–73.
52.
Yoon HS, Im HJ, Moon HN, Lee JH, Kim HJ, Yoo KH, et al. The outcome of hematopoietic stem cell transplantation in Korean children with hemophagocytic lymphohistiocytosis. Pediatr Transpl. 2010;14:735–40.CrossRef
53.
Ohga S, Kudo K, Ishii E, Honjo S, Morimoto A, Osugi Y, et al. Hematopoietic stem cell transplantation for familial hemophagocytic lymphohistiocytosis and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Japan. Pediatr Blood Cancer. 2010;54:299–306.PubMed
54.
Sparber-Sauer M, Honig M, Schulz AS, zur Stadt U, Schutz C, Debatin KM, et al. Patients with early relapse of primary hemophagocytic syndromes or with persistent CNS involvement may benefit from immediate hematopoietic stem cell transplantation. Bone Marrow Transpl. 2009;44:333–8.CrossRef
55.
Marsh RA, Jordan MB, Filipovich AH. Reduced-intensity conditioning haematopoietic cell transplantation for haemophagocytic lymphohistiocytosis: an important step forward. Br J Haematol. 2011;154:556–63.CrossRefPubMedCentralPubMed
56.
Imashuku S, Teramura T, Tauchi H, Ishida Y, Otoh Y, Sawada M, et al. Longitudinal follow-up of patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Haematologica. 2004;89:183–8.PubMed
57.
Imashuku S, Hibi S, Todo S, Sako M, Inoue M, Kawa K, et al. Allogeneic hematopoietic stem cell transplantation for patients with hemophagocytic syndrome (HPS) in Japan. Bone Marrow Transpl. 1999;23:569–72.CrossRef
58.
Yu JT, Hwang WL, Wang RC, Teng CL. Reduced intensity conditioning allogeneic hematopoietic stem cell transplant could be beneficial to angioimmunoblastic T-cell lymphoma patients with hemophagocytic lymphohistiocytosis. Ann Hematol. 2012;91:805–7.CrossRefPubMed
59.
Wang S, Degar BA, Zieske A, Shafi NQ, Rose MG. Hemophagocytosis exacerbated by G-CSF/GM-CSF treatment in a patient with myelodysplasia. Am J Hematol. 2004;77:391–6.CrossRefPubMed
60.
Metadaten
Titel
Hemophagocytic lymphohistiocytosis (HLH): a review of literature
verfasst von
Rohtesh S. Mehta
Roy E. Smith
Publikationsdatum
01.12.2013
Verlag
Springer US
Erschienen in
Medical Oncology / Ausgabe 4/2013
Print ISSN: 1357-0560
Elektronische ISSN: 1559-131X
DOI
https://doi.org/10.1007/s12032-013-0740-3

Weitere Artikel der Ausgabe 4/2013

Medical Oncology 4/2013 Zur Ausgabe

Original Paper

Comparison of serious adverse reactions between thalidomide and lenalidomide: analysis in the French Pharmacovigilance database

Original Paper

Investigation on XRCC1 genetic polymorphism and its relationship with breast cancer risk factors in Chinese women

Original Paper

Mepilex Lite dressings for managing acute radiation dermatitis in nasopharyngeal carcinoma patients: a systematic controlled clinical trial

Short Communication

Clinical implications of the tumor-infiltrating lymphocyte subsets in colorectal cancer

Original Paper

High level of p37δ-mRNA relative to p110δ-mRNA in neuroblastoma tumors correlates with poor patient survival

Review Article

Protein kinase C (PKC) as a drug target in chronic lymphocytic leukemia

Neu im Fachgebiet Onkologie

Bei seelischem Stress sind Checkpoint-Hemmer weniger wirksam

03.06.2024 NSCLC Nachrichten

Wie stark Menschen mit fortgeschrittenem NSCLC von einer Therapie mit Immun-Checkpoint-Hemmern profitieren, hängt offenbar auch davon ab, wie sehr die Diagnose ihre psychische Verfassung erschüttert

Antikörper mobilisiert Neutrophile gegen Krebs

03.06.2024 Onkologische Immuntherapie Nachrichten

Ein bispezifischer Antikörper formiert gezielt eine Armee neutrophiler Granulozyten gegen Krebszellen. An den Antikörper gekoppeltes TNF-alpha soll die Zellen zudem tief in solide Tumoren hineinführen.

Erhebliches Risiko für Kehlkopfkrebs bei mäßiger Dysplasie

29.05.2024 Larynxkarzinom Nachrichten

Fast ein Viertel der Personen mit mäßig dysplastischen Stimmlippenläsionen entwickelt einen Kehlkopftumor. Solche Personen benötigen daher eine besonders enge ärztliche Überwachung.

15% bedauern gewählte Blasenkrebs-Therapie

29.05.2024 Urothelkarzinom Nachrichten

Ob Patienten und Patientinnen mit neu diagnostiziertem Blasenkrebs ein Jahr später Bedauern über die Therapieentscheidung empfinden, wird einer Studie aus England zufolge von der Radikalität und dem Erfolg des Eingriffs beeinflusst.

Update Onkologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen
Bildnachweise