Risks and benefits of splenectomy versus no splenectomy for hereditary spherocytosis - A personal view

British Journal of Haematology

Volumn 145, Issue 6, 2009, Pages 728-732

  Schilling, Robert F ^a  

^a UNIVERSITY OF WISCONSIN SCHOOL OF MEDICINE AND PUBLIC HEALTH   (United States)

Author keywords

Hereditary spherocytosis; Myocardial infarction; Splenectomy; Stroke; Thrombosis

Indexed keywords

ANEMIA; HEREDITARY SPHEROCYTOSIS; HUMAN; MORTALITY; PREVALENCE; PRIORITY JOURNAL; REVIEW; RISK FACTOR; SEX DIFFERENCE; SPLENECTOMY; VASCULAR DISEASE;

ADULT; BACTERIAL INFECTIONS; CHILD; HUMANS; MYOCARDIAL INFARCTION; PATIENT SELECTION; RISK; SPHEROCYTOSIS, HEREDITARY; SPLENECTOMY; TREATMENT OUTCOME;

EID: 66749134596     PISSN: 00071048     EISSN: 13652141     Source Type: Journal    
DOI: 10.1111/j.1365-2141.2009.07694.x     Document Type: Review

References (48)

1. Bader-Meunier, B., Gauthier, F., Archambaud, F., Cynober, T., Miélot, F., Dommergues, J.P., 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. (Pubitemid 32060324)
2. Berne, J.D., Asensio, J.A., Falabella, A. Gomez, H. (1997) Traumatic rupture of the spleen in a patient with hereditary spherocytosis. Journal of Trauma, 42, 323 326. (Pubitemid 27104675)
3. Bertolotti, M., Loria, P., Martella, P., Carulli, L., De Santis, M. Carulli, N. (2000) Bleeding jejunal varices and portal thrombosis in a splenectomized patient with hereditary spherocytosis. Digestive Diseases and Sciences, 45, 373 377. (Pubitemid 30133901)
4. Bolton-Maggs, P.H.B., Stevens, R.F., Dodd, N.J., Lamont, G., Tittensor, P. King, M.J. (2004) Guidelines for the diagnosis and management of hereditary spherocytosis. British Journal of Haematology, 126, 455 474. (Pubitemid 39093088)
5. Bonderman, D., Jakowitsch, J., Adlbrecht, C., Schemper, M., Kyrle, P.A., Schönauer, V., Exner, M., Klepetko, W., Kneussl, M.P., Maurer, G. Lang, I. (2005) Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension. Journal of Thrombosis and Haemostasis, 93, 512 516. (Pubitemid 40392541)
6. Boone, K.E. Watters, D.A.K. (1995) The incidence of malaria after splenectomy in Papua New Guinea. British Medical Journal, 311, 1273.
7. Breuer, C., Janssen, G., Laws, H., Schaper, J., Mayatepek, E., Schroten, H. Tenenbaum, T. (2008) Splenic infarction in a patient hereditary spherocytosis, protein C deficiency and acute infectious mononucleosis. European Journal of Pediatrics, 167, 1449 1452.
8. Cappellini, M.D., Robbiolo, L., Bottasso, B.M., Coppola, R., Fiorelli, G. Mannucci, P.M. (2000) Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. British Journal of Haematology, 111, 467 473. (Pubitemid 32009338)
9. Croom, R.D., III., McMillan, C.W., Orringer, E.P. Sheldon, G.F. (1986) Hereditary spherocytosis. Recent experience and current concepts of pathophysiology. Annals of Surgery, 203, 34 39. (Pubitemid 16183289)
10. Cynober, T., Mohandas, N. Tchernia, G. (1996) Red cell abnormalities in hereditary spherocytosis: relevance to diagnosis and understanding the variable expression of clinical severity. Journal of Laboratory and Clinical Medicine, 128, 259 269. (Pubitemid 27506937)
11. Deodhar, H.A., Marshall, R.J. Barnes, J.N. (1993) Increased risk of sepsis after splenectomy. British Medical Journal, 307, 1408 1409. (Pubitemid 23341675)
12. Eber, S.W., Langendörfer, C.M., Ditzig, M., Reinhardt, D., Stöhr, G., Soldan, W., Schröter, W. Tchernia, G. (1999) Frequency of very late fatal sepsis after splenectomy for hereditary spherocytosis: impact of insufficient antibody response to pneumococcal infection. Annals of Hematology, 78, 524 528.
13. Eraklis, A.J. Filler, R.M. (1972) Splenectomy in childhood: a review of 1413 cases. Journal of Pediatric Surgery, 7, 382 388.
14. Hayag-Barin, J.E., Smith, R. Tucker, F.C., Jr. 1998) Hereditary spherocytosis, thrombocytosis, and chronic pulmonary emboli: a case report and review of the literature. American Journal of Hematology, 57, 82 84. (Pubitemid 28047205)
15. Hirsh, J. Dacie, J.V. (1966) Persistent post-splenectomy thrombocytosis and thromboembolism: a consequence of continuing anaemia. British Journal of Haematology, 12, 44 53.
16. Hoeper, M.M., Niedermeyer, J., Hoffmeyer, F., Flemming, P. Fabel, H. (1999) Pulmonary hypertension after splenectomy? Annals of Internal Medicine, 130, 506 509. (Pubitemid 29135803)
17. Jaïs, X., Ioos, V., Jardim, C., Sitbon, O., Parent, F., Hamid, A., Fadel, E., Dartevelle, P., Simonneau, G. Humbert, M. (2005) Splenectomy and chronic thromboembolic pulmonary hypertension. Thorax, 60, 1031 1034. (Pubitemid 41705472)
18. Jardine, D.L. Laing, A.D. (2004) Delayed pulmonary hypertension following splenectomy for congenital spherocytosis. Internal Medicine Journal, 34, 214 226.
19. Kannel, W.B., Gordon, T., Wolf, P.A. McNamara, P. (1972) Hemoglobin and the risk of cerebral infarction: the Framingham study. Stroke, 3, 409 419.
20. Konradsen, H.B. Henrichsen, J. (1991) Pneumococcal infections in splenectomized children are preventable. Acta Paediatrica Scandinavica, 80, 423 427.
21. Kullberg, B.J., Westendorp, R.G.J., van't Wout, J.W. Meinders, A.E. (1991) Purpura fulminans and symmetrical peripheral gangrene caused by capnocytophaga canimorsus (formerly DF-2) septicemia - a complication of dog bite. Medicine, 70, 287 292.
22. Lion, C., Escande, F. Burdin, J.C. (1996) Capnocytophaga canimorsus infections in human: review of the literature and cases report. European Journal of Epidemiology, 12, 521 533. (Pubitemid 26330141)
23. Manno, C.S. Cohen, A.R. (1989) Splenectomy in mild hereditary spherocytosis: is it worth the risk? American Journal of Pediatric Hematology/Oncology, 11, 300 303. (Pubitemid 19213449)
24. Marchetti, M., Quaglini, S. Barosi, G. (1998) Prophylactic splenectomy and cholecystectomy in mild hereditary spherocytosis: analyzing the decision in different clinical scenarios. Journal of Internal Medicine, 244, 217 226. (Pubitemid 28389658)
25. McGrew, W. Avant, G.R. (1984) Hereditary spherocytosis and portal vein thrombosis. Journal of Clinical Gastroenterology, 6, 381 384.
26. Monge-Argilés, J.A., López-García, M.D., Ortega-Ortega, M.D., Kahn-Mesía, E.I., Pérez-Vicente, J.A., Morales-Ortiz, A. Bautista-Prados, J. (2005) Infarto isquemico cerebral provocado por factor V de Leiden, esferocitosis hereditaria y tabaquismo. Revista de Neurologia, 40, 125 126. (Pubitemid 41473083)
27. Phrommintikul, A., Sukonthasaran, A., Kanjanavanit, R. Nawarawong, W. (2006) Splenectomy: a strong risk factor for pulmonary hypertension in patients with thalassaemia. Heart, 92, 1467 1472. (Pubitemid 44578845)
28. Prabhakaran, K., Jacobs, B., Smaldone, M.C. Franks, M.E. (2007) Stuttering priapism associated with hereditary spherocytosis. The Canadian Journal of Urology, 14, 3702 3704.
29. Rabinstein, A., Tikhomirov, V., Kaluta, A., Gelfmann, N., Iannini, P. Edwards, L. (2000) Recurrent and prolonged fever in asplenic patients with human granulocytic ehrlichiosis. The Quarterly Journal of Medicine, 93, 198 201. (Pubitemid 30128711)
30. Rosner, F., Zarrabi, M.H., Benach, J.L. Habicht, G.S. (1984) Babesiosis in splenectomized adults. American Journal of Medicine, 76, 696 701.
31. Schilling, R.F. (1976) Hereditary spherocytosis: a study of splenectomized persons. Seminars in Hematology, 13, 169 176.
32. Schilling, R.F. (1997) Spherocytosis, splenectomy, strokes, and heart attacks. Lancet, 350, 1677 1678. (Pubitemid 28036834)
33. Schilling, R.F., Gangnon, R.E. Traver, M. (2006) Arteriosclerotic events are less frequent in persons with chronic anemia: evidence from families with hereditary spherocytosis. American Journal of Hematology, 81, 315 317.
34. Schilling, R.F., Gangnon, R.E. Traver, M.I. (2008) Delayed adverse vascular events after splenectomy in hereditary spherocytosis. Journal of Thrombosis and Haemostasis, 6, 1289 1295.
35. Shahidi, N.T. Clatanoff, D.V. (1969) The role of puberty in red-cell production in hereditary haemolytic anaemias. British Journal of Haematology, 17, 335 342.
36. Singer, S.T., Kuypers, F.A., Styles, L., Vichinsky, E.P., Foote, D. Rosenfeld, H. (2006) Pulmonary hypertension in thalassemia: association with platelet activation and hypercoagulable state. American Journal of Hematology, 81, 670 675. (Pubitemid 44306825)
37. Smedema, J.P. Louw, V.J. (2007) Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis. Cardiovascular Journal of South Africa, 18, 84 89. (Pubitemid 46999836)
38. Sparwasser, C., Danz, B. Thon, W.F. (1988) Segmentaler, unilateraler priapismus- ein fallbericht. Der Urologe. Ausgabe A, 27, 266 268.
39. Stoehr, G.A., Sobh, J.N., Luecken, J., Heidemann, K., Mittler, U., Hilgers, R. Eber, S.W. (2006) Near-total splenectomy for hereditary spherocytosis: clinical prospects in relation to disease severity. British Journal of Haematology, 132, 791 793. (Pubitemid 43381643)
40. Stowell, C.P., Gelfand, J.A., Shepard, J.O. Kratz, A. (2007) Case 17-2007 - a 25-year-old woman with relapsing fevers and recent onset dyspnea. New England Journal of Medicine, 356, 2313 2319.
41. Suzuki, Y., Shichishima, T., Mukae, M., Ohsaka, M., Hayama, M., Horie, R., Togano, T., Miyazaki, K., Ichinoe, M., Iwabuchi, K., Fujii, H. Higashihara, M. (2007) Splenic infarction after Epstein-Barr virus infection in a patient with hereditary spherocytosis. International Journal of Hematology, 85, 380 383. (Pubitemid 47457445)
42. Taher, A., Isma'eel, H., Mehio, G., Bignamini, D., Kattamis, A., Rachmilewitz, E.A. Cappellini, M.D. (2006) Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran. Thrombosis and Haemostasis, 96, 488 491. (Pubitemid 44560684)
43. Tracey, E.T. Rice, H.E. (2008) Partial splenectomy for hereditary spherocytosis. Pediatric Clinics of North America, 55, 503 519.
44. Troendle, S.B., Adix, L., Crary, S.E. Buchanan, G.R. (2007) Laboratory markers of thrombosis risk in children with hereditary spherocytosis. Pediatric Blood and Cancer, 49, 781 785. (Pubitemid 47519512)
45. Ustun, C., Kutlar, F., Holley, L., Seigler, M., Burgess, R. Kutlar, A. (2003) Interaction of sickle cell trait with hereditary spherocytosis: splenic infarcts and sequestration. Acta Haematologica, 109, 46 49. (Pubitemid 41754749)
46. Verresen, D., De Backer, W., Van Meerbeeck, J., Neetens, I., Van Marck, E. Vermeire, P. (1991) Spherocytosis and pulmonary hypertension coincidental occurrence or causal relationship? European Respiratory Journal, 4, 629 631.
47. Wang, C.H. Schilling, R.F. (1995) Myocardial infarction and thalassemia trait: an example of heterozygote advantage. American Journal of Hematology, 49, 73 75.
48. White, D.J., Talarico, J., Chang, H.G., Birkhead, G.S., Heimberger, T. Morse, D.L. (1998) Human babesiosis in New York State: review of 139 hospitalized cases and analysis of prognostic factors. Archives of Internal Medicine, 158, 2149 2154. (Pubitemid 28482099)