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Splenectomy in Hematology. Current Practice and New Perspectives

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Splenectomy in Hematology. Current Practice and New Perspectives Haematologica 1999; 84:431-436 decision making and problem solving Splenectomy in hematology. Current practice and new perspectives UMBERTO BACCARANI,* GIOVANNI TERROSU,* ANNIBALE DONINI,* FRANCESCO ZAJA,° FABRIZIO BRESADOLA,* MICHELE BACCARANI° *Chair and Division of Surgery, Department of Surgery, and °Chair and Division of Hematology, Department of Clinical and Morphologic Research, Udine University Hospital and School of Medicine, Udine, Italy ABSTRACT Background and Objective. Progress and changes in or many years splenectomy was the main point the management of blood diseases, in surgery and in of contact between hematology and surgery. videotechnology stimulate a critical reappraisal of FThe indications for splenectomy in hematology splenectomy in hematology. are numerous, from diagnostic and staging purpos- Design and Methods. We have collected information es to splenic rupture due to infarction, from anemia on the current practice of splenectomy in hematol- or thrombocytopenia to leukemia or lymphoma. ogy in Italy and we have reviewed the results of a These indications were generated over different his- new technique of laparoscopic splenectomy (LS). torical periods. Many of them are soundly based on Results. Current splenectomy practice: the current common sense and practice, but rarely on evidence. practice in Italy is to offer splenectomy as front-line Over time the indications for splenectomy in hema- treatment for hereditary spherocytosis and as sec- tology have undergone some changes,1 depending ond-line for idiopathic thrombocytopenic purpura on modifications and progress in the management (ITP) and hemolytic anemia. Splenectomy is also of malignant and non-malignant blood diseases. offered in selected cases of leukemia and lymphoma Major examples are Hodgkin’s disease (HD) and but is going out of practice for hairy cell leukemia hairy cell leukemia (HCL) where new treatment and Hodgkin’s disease. The number of splenec- strategies and new therapeutic agents have mini- tomies that are performed every year is estimated to 2-5 be higher than 10 per 106 persons (more than 500 mized recourse to splenectomy. The use of splenec- cases per year). Laparoscopic splenectomy (LS): tomy is also influenced by the frequency and the more than 700 cases of LS have been reported so severity of its complications. Late complications far, for thrombocytopenia (470 cases) as well as for mainly involve the immune system and host defence, many other hematologic indications. The procedure being infectious, and advise against splenectomy in carries a mortality of 0.8%, and a complication rate children.6-10 Short term complications are related to of 12%. Time spent in the operating theater ranges the underlying disease and the patient’s clinical and from 1.5 to 4 hours, blood transfusion requirement is hematologic conditions, but also to surgical tech- minimal and the mean post-operative hospital stay is nique and skill. Progress in surgery has made it pos- 3 days. sible to perform splenectomy under videolaparo- Interpretation and Conclusions. Although a prospec- scopic control avoiding laparotomy.11-14 It may, tive comparison is not available, the results of LS therefore, be opportune to reexamine this meeting compare favorably with the results of classic open point between hematology and surgery and work out splenectomy, so that LS is likely to become the tech- evidence-based analysis with the purpose of reaching nique of choice especially when the spleen is small, a consensus and proposing guidelines. This review is as in ITP. LS can also have some advantages in oth- er cases of splenectomy, including splenomegaly for propedeutic to the analysis and stimulatory to the leukemia and lymphoma. These data and sugges- discussion, reporting on the splenectomy policies tions should stimulate and renew a discussion about that are currently practiced in Italy and illustrating splenectomy in hematology, with the purpose of the technique and the results of videolaparoscopic establishing evidence-based guidelines. splenectomy. Data and discussion are limited to ©1999, Ferrata Storti Foundation splenectomy in adults. Splenectomy practice in Italy: an overview Key words: splenectomy, laparoscopy, leukemia, lymphoma, anemia, thrombocytopenia To collect information on the current practice of splenectomy in hematology in adults, we addressed a simple questionnaire to 52 centers of which 41 were Divisions of Hematology and 11 were Divisions of Internal Medicine or Oncology with a known interest in hematology. Centers were located at University or Correspondence: Umberto Baccarani, M.D., Department of Surgery, Udine University Hospital, 33100 Udine, Italy. Phone: international General Hospitals all over Italy. Fourty-seven answers +39-0432-559557 – Fax: international +39-0432-559555. (90%) were received. The questionnaire asked the 432 U. Baccarani et al. respondents to identify the current main indications HCL, where 50% of the institutions have abandoned for and contraindications to splenectomy in chronic splenectomy completely, due to the increasing avail- idiopathic thrombocytopenic purpura (ITP), hemolyt- ability of very effective medical treatments4 and with ic anemia, primary myelofibrosis (MF), non-Hodgk- HD, where staging laparotomy and splenectomy have in’s lymphomas (NHL), chronic lymphocytic leukemia fallen out of use, either because of the improvement (CLL), HCL and HD. In all the answers, a distinction in imaging techniques or because of the increased was made between hereditary spherocytosis (HS) and effectiveness of other treatments.2,3,15 Fear that chronic autoimmune hemolytic anemia (AHA). More- splenectomy can increase the rate of late second over, the answers clarified whether, in any given con- tumors16-18 may also have contributed to the aban- dition, splenectomy was recommended always, some- doning of splenectomy. times, never or exceptionally. Laparoscopic splenectomy All 47 Institutions declared using splenectomy in Splenectomy was traditionally performed through a HS, AHA and chronic ITP (Table 1). In HS all centers midline or left subcostal laparotomy (open splenec- but two advise splenectomy as front-line treatment tomy, or OS). The development of new video-tech- without any major contraindications. In chronic AHA nology combined with advances in surgical instru- and in chronic ITP splenectomy is mainly advised in mentation has allowed the surgeon to enter the peri- case of resistance to treatment or when the course is toneum through multiple tiny incisions of the skin heavily dependent on chronic treatment. Major con- under direct visualization offered by a fiber-optic traindications, as identified by responding centers, scope and a videocamera connected to a TV screen. are advanced age (42% of responding centers for This new laparoscopic technique allows several surgi- AHA, 60% for ITP), cold IgM autoantibodies (9%), cal procedures to be performed, for example chole- HBV or HCV positivity (7%), immunodeficiency or cystectomy, appendectomy, hernia repair, gastric fun- HIV positivity (4%) and systemic autoimmunity (9%). doplication, adrenalectomy.19 In 1992, Carroll et al. in In primary MF with liver/spleen myeloid metapla- Los Angeles were the first to use laparoscopy for sia (Table 1), splenectomy is no longer recommend- splenectomy.11 Since then, several centers worldwide ed in 13/47 centers (28%). Major indications are have adopted this new approach. The abdomen is splenomegaly (68%), anemia (21%) and symptoms insufflated with CO to create a virtual chamber in the (47%). Major contraindications are advanced age 2 peritoneal sac. Special hollow devices, called trocars, (42% of responding centers), a high platelet count are used to introduce the fiber-optic scope and the (39%) and disease acceleration or progression (27%). laparoscopic instruments into the peritoneal cavity. Splenectomy for NHL, CLL and HCL (Table 1) is cur- The operation can now be performed entirely laparo- rently not practised by 2%, 28% and 51% of the cen- scopically. Usually 4 to 5 trocars, located in the upper ters, respectively. Major indications are splenomegaly abdomen, are enough to perform a laparoscopic (41%, 42% and 21%), primary splenic lymphoma or splenectomy (LS). The operation starts with dissec- isolated splenomegaly (67% in NHL), and residual or refractory splenomegaly (20%, 28% and 30%). In HD, splenectomy is out-of-practice in 32/47 cen- ters (68%) (Table 1). In the remaining 15 centers Table 1. A summary of the major indications for splenecto- splenectomy is practised only for the surgical staging my, based on current practice in 47 Italian hematologic of a few, selected early stage cases and sometimes also centers. for the management of an isolated splenomegaly. To summarize, splenectomy is currently indicated Chronic as front-line treatment in HS, irrespective of the HS AHA ITP MF NHL CLL HCL HD degree of anemia, because only two of the 47 centers %%%% %%%% answered that the recommendation for splenectomy depended on Hb level. It is recommended as second- Always 95 0 0 0 0 0 0 0 line treatment in chronic AHA and chronic ITP, when Sometimes 5 100 100 72 98 72 49 32 medical treatment fails or treatment duration and Never or exceptionally 0 0 0 28 2 28 51 68 toxicity are not acceptable. Interestingly, disease sever- Resistance to treatment – 95 79 2 0 8 30 0 ity and duration were not indicated, while five centers Treatment dependence – 6 23 0 0 0 0 0 pointed out that splenectomy can be required to elim- Bleeding 0 0 17 0 0 0 0 0 inate or reduce the anxiety of living with ITP or AHA. Severe thrombocytopenia 0 2 30 8 15 15 4 0 Severe anemia 5 0 0 21 15 19 2 0 In MF, in lymphomas and in chronic lymphoprolifer- Spleen volume 0 0 0 68 41 42 21 0 ative disorders splenectomy is no longer used as front- Residual or refractory – – – 0 20 28 30 0 line treatment. However, it is still recommended in splenomegaly MF when splenomegaly is massive and symptomatic, Isolated splenomegaly – – – – 67 2 0 4 and in NHL and CLL, mainly when spleen volume is Symptoms 0 0 0 47 6 4 2 0 large or the spleen is refractory to treatment, with ane- Pathologic staging – – – – 2 – – 30 mia or thrombocytopenia.
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