Skip to main content
SpringerLink
Log in

Splenektomie bei Kindern und Jugendlichen mit hämatologischen Erkrankungen

  • Living reference work entry
  • First Online:
Kinderchirurgie

Part of the book series: Springer Reference Medizin ((SRM))

  • 349 Accesses

Zusammenfassung

Die Milz hat eine zentrale immunologische Funktion im allgemeinen Kontext der B-Zellentwicklung, der Antigenpräsentation und der damit verbundenen Reifung des Immunsystems, die zudem insbesondere bei der Abwehr bekapselter Bakterien zum Tragen kommt. Darüber hinaus ist die Milz der zentrale Abbauort für gealterte, intrinsisch defekte, durch Antikörper opsonierte oder durch andere externe Einflüsse veränderte Blutzellen. Aus diesen Funktionen ergibt sich einerseits die Bedeutung der vollständigen oder partiellen Splenektomie für hämatologische Erkrankungen sowie andererseits das damit verbundene potenzielle Risiko für Spätfolgen. In diesem Kapitel werden zunächst die potenziellen, mit einer Splenektomie assoziierten Risiken und Komplikationen dargestellt und Empfehlungen zur Prophylaxe der Postsplenektomie-Infektionen diskutiert. Anschließend werden Vorgehensweisen zur Splenektomie aus hämatologischer Sicht skizziert, bevor in Einzelabschnitten eine Darstellung spezifischer Krankheitsbilder einschließlich der Rolle der vollständigen oder partiellen bzw. subtotalen Splenektomie in der Behandlung von Patienten mit diesen Erkrankungen erfolgt.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

Literatur

  • Alizai NK, Richards EM, Stringer MD (2010) Is cholecystectomy really an indication for concomitant splenectomy in mild hereditary spherocytosis? Arch Dis Child 95:596–599

    Article  PubMed  Google Scholar 

  • Andolfo I, Russo R, Gambale A, Iolascon A (2016) New insights on hereditary erythrocyte membrane defects. Haematologica 101:1284–1294

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Aydinok Y, Bayraktaroglu S, Yildiz D, Alper H (2011) Myocardial iron loading in patients with thalassemia major in Turkey and the potential role of splenectomy in myocardial siderosis. J Pediatr Hematol Oncol 33:374–378

    Article  CAS  PubMed  Google Scholar 

  • Bader-Meunier B, Gauthier F, Archambaud F, Cynober T, Mielot F, Dommergues JP, Warszawski J, Mohandas N, Tchernia G (2001) Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis. Blood 97:399–403

    Article  CAS  PubMed  Google Scholar 

  • Beutler E, Gelbart T (2000) Estimating the prevalence of pyruvate kinase deficiency from the gene frequency in the general white population. Blood 95:3585–3588

    PubMed  CAS  Google Scholar 

  • Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ (2004) Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol 126:455–474

    Article  CAS  PubMed  Google Scholar 

  • Brousse V, Buffet P, Rees D (2014) The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol 166:165–176

    Article  PubMed  Google Scholar 

  • Cappellini MD, Fiorelli G (2008) Glucose-6-phosphate dehydrogenase deficiency. Lancet 371:64–74

    Article  CAS  PubMed  Google Scholar 

  • Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V (2014) Guidelines for the management of transfusion dependent thalassemia (TDT). Thalassemia International Federation, Nicosia

    Google Scholar 

  • Cario H, Kohne E (2016) AWMF S1-Leitlinie: Thalassämien. AWMF online http://www.awmf.org/leitlinien/detail/ll/025-017.html. Zugegriffen am 15.04.2018.

  • Cooper N (2017) State of the art – how I manage immune thrombocytopenia. Br J Haematol 177:39–54

    Article  PubMed  Google Scholar 

  • Davies JM, Lewis MP, Wimperis J, Rafi I, Ladhani S, Bolton-Maggs PH (2011) Review of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen: prepared on behalf of the British committee for standards in haematology by a working party of the haemato-oncology task force. Br J Haematol 155:308–317

    Article  CAS  PubMed  Google Scholar 

  • Deutsche Gesellschaft für Infektiologi (DGI) e.V. (2017) Asplenie-Net. Prävention bei Splenektomie und Asplenie. https://asplenie-net.org/

  • Eber S (2006) Angeborene Erythrozytenmembrandefekte. In: Gadener H, Gaedicke G, Niemeyer C (Hrsg) Pädiatrische Hämatologie und Onkologie. Springer Medizin Verlag, Heidelberg, S 123–138

    Chapter  Google Scholar 

  • Eber S, Andres O (2016) S1-Leitlinie: Hereditäre Sphärozytose. AWMF online http://www.awmf.org/leitlinien/detail/ll/025-087.html

  • Eber S, Lux SE (2004) Hereditary spherocytosis – defects in proteins that connect the membrane skeleton to the lipid bilayer. Semin Hematol 41:118–141

    Article  CAS  PubMed  Google Scholar 

  • Engelhardt M, Eber S, Germing U, Heimpel H, Kern WV, Schmugge M (2013) Prävention von Infektionen und Thrombosen nach Splenektomie oder funktioneller Asplenie. Onkopedia Online https://www.onkopedia.com/de/onkopedia/guidelines/praevention-von-infektionen-und-thrombosen-nach-splenektomie-oder-funktioneller-asplenie/@@view/html/index.html

  • Englum BR, Rothman J, Leonard S, Reiter A, Thornburg C, Brindle M, Wright N, Heeney MM, Jason Smithers C, Brown RL et al (2016) Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia. J Pediatr Surg 51:122–127

    Article  PubMed  Google Scholar 

  • Guizzetti L (2016) Total versus partial splenectomy in pediatric hereditary spherocytosis: a systematic review and meta-analysis. Pediatr Blood Cancer 63:1713–1722

    Article  PubMed  Google Scholar 

  • Hamilton JW, Jones FG, McMullin MF (2004) Glucose-6-phosphate dehydrogenase Guadalajara – a case of chronic non-spherocytic haemolytic anaemia responding to splenectomy and the role of splenectomy in this disorder. Hematology 9:307–309

    Article  CAS  PubMed  Google Scholar 

  • Harteveld CL, Higgs DR (2010) Alpha-thalassaemia. Orphanet J Rare Dis 5:13

    Article  PubMed  PubMed Central  Google Scholar 

  • Heimpel H, Anselstetter V, Chrobak L, Denecke J, Einsiedler B, Gallmeier K, Griesshammer A, Marquardt T, Janka-Schaub G, Kron M et al (2003) Congenital dyserythropoietic anemia type II: epidemiology, clinical appearance, and prognosis based on long-term observation. Blood 102:4576–4581

    Article  CAS  PubMed  Google Scholar 

  • Hill QA, Stamps R, Massey E, Grainger JD, Provan D, Hill A (2017) The diagnosis and management of primary autoimmune haemolytic anaemia. Br J Haematol 176:395–411

    Article  PubMed  Google Scholar 

  • Iolascon A, Heimpel H, Wahlin A, Tamary H (2013) Congenital dyserythropoietic anemias: molecular insights and diagnostic approach. Blood 122:2162–2166

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Iolascon A, Andolfo I, Barcellini W, Corcione F, Garcon L, De Franceschi L, Pignata C, Graziadei G, Pospisilova D, Rees DC et al (2017) Recommendations regarding splenectomy in hereditary hemolytic anemias. Haematologica 102:1304–1313

    Article  PubMed  PubMed Central  Google Scholar 

  • Krauth MT, Lechner K, Neugebauer EA, Pabinger I (2008) The postoperative splenic/portal vein thrombosis after splenectomy and its prevention – an unresolved issue. Haematologica 93:1227–1232

    Article  PubMed  Google Scholar 

  • Kristinsson SY, Gridley G, Hoover RN, Check D, Landgren O (2014) Long-term risks after splenectomy among 8,149 cancer-free American veterans: a cohort study with up to 27 years follow-up. Haematologica 99:392–398

    Article  PubMed  PubMed Central  Google Scholar 

  • Kung C, Hixon J, Kosinski PA, Cianchetta G, Histen G, Chen Y, Hill C, Gross S, Si Y, Johnson K et al (2017) AG-348 enhances pyruvate kinase activity in red blood cells from patients with pyruvate kinase deficiency. Blood 130:1347–1356

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Lobitz S, Cario H, Grosse R, Jarisch A, Kulozik AE, Kunz J (2014) AWMF S2k-Leitlinie 025/016: Sichelzellkrankheit. AWMF online http://www.awmf.org/leitlinien/detail/ll/025-016.html

  • Marchetti M, Quaglini S, Barosi G (1998) Prophylactic splenectomy and cholecystectomy in mild hereditary spherocytosis: analyzing the decision in different clinical scenarios. J Intern Med 244:217–226

    Article  CAS  PubMed  Google Scholar 

  • Miano M (2016) How I manage Evans syndrome and AIHA cases in children. Br J Haematol 172:524–534

    Article  PubMed  Google Scholar 

  • Morris CR, Vichinsky EP (2010) Pulmonary hypertension in thalassemia. Ann N Y Acad Sci 1202:205–213

    Article  CAS  PubMed  Google Scholar 

  • Mouttalib S, Rice HE, Snyder D, Levens JS, Reiter A, Soler P, Rothman JA, Thornburg CD (2012) Evaluation of partial and total splenectomy in children with sickle cell disease using an Internet-based registry. Pediatr Blood Cancer 59:100–104

    Article  PubMed  PubMed Central  Google Scholar 

  • Piel FB, Weatherall DJ (2014) The alpha-thalassemias. N Engl J Med 371:1908–1916

    Article  CAS  PubMed  Google Scholar 

  • Pincez T, Guitton C, Gauthier F, de Lambert G, Picard V, Feneant-Thibault M, Turhan A, Mohandas N, Tchernia G, Garcon L (2016) Long-term follow-up of subtotal splenectomy for hereditary spherocytosis: a single-center study. Blood 127:1616–1618

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Rao VK (2015) Approaches to managing autoimmune cytopenias in novel immunological disorders with genetic underpinnings like autoimmune lymphoproliferative syndrome. Front Pediatr 3:65

    Article  PubMed  PubMed Central  Google Scholar 

  • Rogulski R, Adamowicz-Salach A, Matysiak M, Piotrowski D, Gogolewski M, Piotrowska A, Roik D, Kaminski A (2016) Laparoscopic splenectomy for hereditary spherocytosis-preliminary report. Eur J Haematol 96:637–642

    Article  PubMed  Google Scholar 

  • Rund D, Rachmilewitz E (2005) Beta-thalassemia. N Engl J Med 353:1135–1146

    Article  CAS  PubMed  Google Scholar 

  • Russo R, Gambale A, Langella C, Andolfo I, Unal S, Iolascon A (2014) Retrospective cohort study of 205 cases with congenital dyserythropoietic anemia type II: definition of clinical and molecular spectrum and identification of new diagnostic scores. Am J Hematol 89:E169–E175

    Article  PubMed  Google Scholar 

  • Schilling RF, Gangnon RE, Traver MI (2008) Delayed adverse vascular events after splenectomy in hereditary spherocytosis. J Thromb Haemost 6:1289–1295

    Article  CAS  PubMed  Google Scholar 

  • Slater BJ, Chan FP, Davis K, Dutta S (2010) Institutional experience with laparoscopic partial splenectomy for hereditary spherocytosis. J Pediatr Surg 45:1682–1686

    Article  PubMed  Google Scholar 

  • Stewart GW, Amess JA, Eber SW, Kingswood C, Lane PA, Smith BD, Mentzer WC (1996) Thrombo-embolic disease after splenectomy for hereditary stomatocytosis. Br J Haematol 93:303–310

    Article  CAS  PubMed  Google Scholar 

  • Stoehr GA, Sobh JN, Luecken J, Heidemann K, Mittler U, Hilgers R, Eber SW (2006) Near-total splenectomy for hereditary spherocytosis: clinical prospects in relation to disease severity. Br J Haematol 132:791–793

    Article  PubMed  Google Scholar 

  • Strasser BM, Holschneider AM (1986) Die Milz: Funktion, Erkrankungen, Chirurgie und Replantation. Hippocrates Verlag, Stuttgart

    Google Scholar 

  • Stuart MJ, Nagel RL (2004) Sickle-cell disease. Lancet 364:1343–1360

    Article  PubMed  Google Scholar 

  • Taher A, Tyan PI (2014) The Spleen. In: Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V (Hrsg) Guidelines for the management of transfusion dependent thalassemia (TDT). Thalassemia International Federation, Nicosia, S 126–132

    Google Scholar 

  • Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, Saned MS, El-Chafic AH, Fasulo MR, Cappellini MD (2010) Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood 115:1886–1892

    Article  CAS  PubMed  Google Scholar 

  • Taher AT, Musallam KM, Cappellini MD, Weatherall DJ (2011) Optimal management of beta thalassaemia intermedia. Br J Haematol 152:512–523

    Article  CAS  PubMed  Google Scholar 

  • Taher A, Vichinsky E, Musallam KM, Cappellini MD, Viprakasit V (2013) Splenectomy. In: Taher A, Vichinsky E, Musallam KA, Cappellini MD, Viprakasit V (Hrsg) Guidelines for the management of non transfusion dependent thalassemia. Thalassemia International Federation, Nicosia, S 19–26

    Google Scholar 

  • Thomsen RW, Schoonen WM, Farkas DK, Riis A, Fryzek JP, Sorensen HT (2010) Risk of venous thromboembolism in splenectomized patients compared with the general population and appendectomized patients: a 10-year nationwide cohort study. J Thromb Haemost 8:1413–1416

    Article  CAS  PubMed  Google Scholar 

  • Williamson D (1993) The unstable haemoglobins. Blood Rev 7:146–163

    Article  CAS  PubMed  Google Scholar 

  • Zanella A, Fermo E, Bianchi P, Valentini G (2005) Red cell pyruvate kinase deficiency: molecular and clinical aspects. Br J Haematol 130:11–25

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Ulm, Ulm, Deutschland

    Holger Cario

Authors
  1. Holger Cario
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Holger Cario .

Editor information

Editors and Affiliations

  1. Kinderchirurgische Klinik und Poliklinik, Klinikum der Universität München Kinderchirurgische Klinik und Poliklinik, München, Bayern, Germany

    Dietrich von Schweinitz

  2. Abt. Kinderchirurgie, Medizinische Hochschule Hannover (MHH) Kinderklinik, Hannover, Germany

    Benno Ure

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Cario, H. (2018). Splenektomie bei Kindern und Jugendlichen mit hämatologischen Erkrankungen. In: von Schweinitz, D., Ure, B. (eds) Kinderchirurgie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-53390-1_7-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-662-53390-1_7-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-53390-1

  • Online ISBN: 978-3-662-53390-1

  • eBook Packages: Springer Referenz Medizin

Publish with us

Policies and ethics

Search

Navigation