Recommendations regarding splenectomy in hereditary hemolytic anemias

被引:155
作者
Iolascon, Achille [1 ,2 ]
Andolfo, Immacolata [1 ,2 ]
Barcellini, Wilma [3 ]
Corcione, Francesco [4 ]
Garcon, Loic [5 ]
De Franceschi, Lucia [6 ,7 ]
Pignata, Claudio [8 ]
Graziadei, Giovanna [9 ]
Pospisilova, Dagmar [10 ,11 ]
Rees, David C. [12 ]
de Montalembert, Mariane [13 ]
Rivella, Stefano [14 ,15 ]
Gambale, Antonella [1 ,2 ]
Russo, Roberta [1 ,2 ]
Ribeiro, Leticia [16 ]
Jules-Vives-Corrons [17 ]
Martinez, Patricia Aguilar [18 ]
Kattamis, Antonis [19 ]
Gulbis, Beatrice [20 ]
Cappellini, Maria Domenica [9 ]
Roberts, Irene [21 ]
Tamary, Hannah [22 ]
机构
[1] Univ Federico II Naples, Dept Mol Med & Med Biotechnol, Naples, Italy
[2] CEINGE Biotecnol Avanzate, Naples, Italy
[3] Osped Maggiore Policlin, IRCCS Ca Granda Fdn, Oncohematol Unit, Milan, Italy
[4] Monaldi Hosp AORN Colli, Dept Gen Surg, Milan, Italy
[5] CHU Amiens Picardie, Serv Hematol Biol, Amiens, France
[6] Univ Verona, Dept Med, Verona, Italy
[7] AOUI Verona, Vernaison, France
[8] Univ II Naples Federico, Dept Translat Med Sci, Naples, Italy
[9] Univ Milan, Osped Maggiore Policlin, Fdn IRCCS Ca Granda, Dept Clin Sci & Commun Hlth, Milan, Italy
[10] Palacky Univ Olomouc, Fac Med & Dent, Dept Pediat, Olomouc, Czech Republic
[11] Univ Hosp Olomouc, Olomouc, Czech Republic
[12] Kings Coll London, Sch Med, Kings Coll Hosp, Dept Paediat Haematol, London, England
[13] Hop Necker Enfants Malad, Pediat Dept, Paris, France
[14] Weill Cornell Med Coll, Childrens Blood & Canc Fdn Labs, Div Hematol Oncol, Dept Pediat, New York, NY USA
[15] Childrens Hosp Philadelphia, Div Hematol, Dept Pediat, Philadelphia, PA 19104 USA
[16] Hosp & Univ Ctr Coimbra CHUC, Hematol Serv, Coimbra, Portugal
[17] Hosp Clin Barcelona, Red Cell Pathol Unit, Barcelona, Spain
[18] Hosp St Eloi, CHRU Montpellier, Lab Hematol, Montpellier, France
[19] Univ Athens, Dept Pediat 1, Athens, Greece
[20] Hosp Erasme, ULB, Dept Clin Chem, Brussels, Belgium
[21] Univ Oxford, John Radcliffe Hosp, Childrens Hosp, Dept Pediat, Oxford, England
[22] Tel Aviv Univ, Sackler Fac Med, Schneider Childrens Med Ctr Israel, Pediat Hematol Unit, Tel Aviv, Israel
关键词
PYRUVATE-KINASE DEFICIENCY; SICKLE-CELL-DISEASE; BONE-MARROW-TRANSPLANTATION; AMINO-ACID SUBSTITUTIONS; SPLENIC VEIN-THROMBOSIS; LONG-TERM RISKS; PULMONARY-HYPERTENSION; LAPAROSCOPIC SPLENECTOMY; THROMBOEMBOLIC DISEASE; SUBTOTAL SPLENECTOMY;
D O I
10.3324/haematol.2016.161166
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short-and long-term infectious and thrombotic complications. In light of the priorities identified by the European Hematology Association Roadmap we generated specific recommendations for each disorder, except thalassemia syndromes for which there are other, recent guidelines. Our recommendations are intended to enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and the possible consequences. As no randomized clinical trials, case control or cohort studies regarding splenectomy in these disorders were found in the literature, recommendations for each disease were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children.
引用
收藏
页码:1304 / 1313
页数:10
相关论文
共 107 条
[1]  
Al-Jafar Hassan A, 2013, BMJ Case Rep, V2013, DOI 10.1136/bcr-2013-201061
[2]   Laparoscopic splenectomy for massive splenomegaly in benign hematological diseases [J].
Al-Mulhim, Abdulrahman Saleh .
SURGICAL ENDOSCOPY AND OTHER INTERVENTIONAL TECHNIQUES, 2012, 26 (11) :3186-3189
[3]   Indications and complications of splenectomy for children with sickle cell disease [J].
Al-Salem, Ahmed H. .
JOURNAL OF PEDIATRIC SURGERY, 2006, 41 (11) :1909-1915
[4]   Dehydrated hereditary stomatocytosis linked to gain-of-function mutations in mechanically activated PIEZO1 ion channels [J].
Albuisson, Juliette ;
Murthy, Swetha E. ;
Bandell, Michael ;
Coste, Bertrand ;
Louis-dit-Picard, Helene ;
Mathur, Jayanti ;
Feneant-Thibault, Madeleine ;
Tertian, Gerard ;
de Jaureguiberry, Jean-Pierre ;
Syfuss, Pierre-Yves ;
Cahalan, Stuart ;
Garcon, Loic ;
Toutain, Fabienne ;
Rohrlich, Pierre Simon ;
Delaunay, Jean ;
Picard, Veronique ;
Jeunemaitre, Xavier ;
Patapoutian, Ardem .
NATURE COMMUNICATIONS, 2013, 4
[5]   Laparoscopic splenectomy and/or cholecystectomy for children with sickle cell disease [J].
Alwabari, Abdulla ;
Parida, Lalit ;
Al-Salem, Ahmed Hassan .
PEDIATRIC SURGERY INTERNATIONAL, 2009, 25 (05) :417-421
[6]   New insights on hereditary erythrocyte membrane defects [J].
Andolfo, Immacolata ;
Russo, Roberta ;
Gambale, Antonella ;
Iolascon, Achille .
HAEMATOLOGICA, 2016, 101 (11) :1284-1294
[7]   Novel Gardos channel mutations linked to dehydrated hereditary stomatocytosis (xerocytosis) [J].
Andolfo, Immacolata ;
Russo, Roberta ;
Manna, Francesco ;
Shmukler, Boris E. ;
Gambale, Antonella ;
Vitiello, Giuseppina ;
De Rosa, Gianluca ;
Brugnara, Carlo ;
Alper, Seth L. ;
Snyder, L. Michael ;
Iolascon, Achille .
AMERICAN JOURNAL OF HEMATOLOGY, 2015, 90 (10) :921-926
[8]   Multiple clinical forms of dehydrated hereditary stomatocytosis arise from mutations in PIEZO1 [J].
Andolfo, Immacolata ;
Alper, Seth L. ;
De Franceschi, Lucia ;
Auriemma, Carla ;
Russo, Roberta ;
De Falco, Luigia ;
Vallefuoco, Fara ;
Esposito, Maria Rosaria ;
Vandorpe, David H. ;
Shmukler, Boris E. ;
Narayan, Rupa ;
Montanaro, Donatella ;
D'Armiento, Maria ;
Vetro, Annalisa ;
Limongelli, Ivan ;
Zuffardi, Orsetta ;
Glader, Bertil E. ;
Schrier, Stanley L. ;
Brugnara, Carlo ;
Stewart, Gordon W. ;
Delaunay, Jean ;
Iolascon, Achille .
BLOOD, 2013, 121 (19) :3925-3935
[9]   Pulmonary arterial hypertension in previously splenectomized patients with β-thalassemic disorders [J].
Atichartakarn, V ;
Likittanasombat, K ;
Chuncharunee, S ;
Chandanamattha, P ;
Worapongpaiboon, S ;
Angchaisuksiri, P ;
Aryurachai, K .
INTERNATIONAL JOURNAL OF HEMATOLOGY, 2003, 78 (02) :139-145
[10]   In vivo platelet activation and hyperaggregation in hemoglobin E/β-thalassemia:: A consequence of splenectomy [J].
Atichartakarn, V ;
Angchaisuksiri, P ;
Aryurachai, K ;
Chuncharunee, S ;
Thakkinstian, A .
INTERNATIONAL JOURNAL OF HEMATOLOGY, 2003, 77 (03) :299-303