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Splenectomy for Splenomegaly and Secondary Hypersplenism

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Splenectomy for Splenomegaly and Secondary Hypersplenism World J. Surg. 9, 437--443, 1985 1985 by the Soci~t~ lnternationale de Chirurgie Splenectomy for Splenomegaly and Secondary Hypersplenism William W. Coon, M.D. Department of Surgery, The University of Michigan Hospitals, Ann Arbor, Michigan, U.S.A. Splenomegaly and secondary hypersplenism may be associ- The separation of hypersplenism into primary ated with acute and chronic infections, autoimmune states, and secondary categories is also imprecise. The portal hypertension or splenic vein thrombosis, and a diseases usually included under "primary" hyper- number of infiltrative and neoplastic conditions involving splenism are those in which the fundamental defect the spleen. Our experience and that of others with these is thought to be related to a congenital or acquired various conditions demonstrates that the decision to per- alteration in cell membrane or structure of form splenectomy should be based on well-defined and hematopoietic cells (idiopathic thrombocytopenic often strictly limited indications. Except for idiopathic purpura, acquired hemolytic anemia, some of the splenomegaly, the presence and severity of secondary congenital hemolytic anemias, etc.). hypersplenism or severely symptomatic splenomegaly This discussion will be confined to selected enti- should be well documented. In each case, the potential for ties usually considered to be associated with "sec- palliation and known mean duration of expected response ondary" hypersplenism in which splenomegaly and must be weighed against the increased morbidity and altered splenic microcirculation may bring about mortality of splenectomy (as compared to operation for splenic sequestration of erythrocytes, neutrophils, "primary" hypersplenism). or platelets. Here, again, the specificity of this terminology is debatable since, in several second- ary hypersplenic conditions (Felty's syndrome, Hypersplenism is a nonspecific term indicating that hairy cell leukemia), altered cell structure may also the spleen may be functioning in a manner having a play a role in the pathophysiologic mechanism of deleterious effect on the blood cells of the host. The cytopenia. usual criteria utilized to indicate the presence of this Secondary hypersplenism has been associated condition are: (a) splenomegaly; (b) a clinically with certain acute and chronic infections, autoim- significant decrease in one or more of the cellular mune states (Felty's syndrome, systemic lupus elements of the blood; (c) the presence of a normal erythematosus), infiltrative and neoplastic condi- or hypercellular bone marrow; and (d) correction or tions involving the spleen (sarcoidosis, Gaucher's improvement of the cytopenia by splenectomy. disease, several myeloproliferative disorders, and However, in clinical application, all of these ele- lymphomas) and occasionally with "congestive ments may not be satisfied. Splenomegaly is not and splenomegaly" brought about by portal hyperten- sion or splenic vein thrombosis. should not be present in idiopathic thrombocy- topenic purpura. A cytopenia present in several of the myeloproliferative disorders may respond to Leukemia splenectomy in spite of the presence of decreased cellular precursors in the bone marrow. Splenectomy has been performed for several types of leukemia for more than 100 years, but its value and limitations have only been defined in the past Reprint requests: William W. Coon, M.D., Section of few years. General Surgery, D2214, Medical Professional Building, Only in the relatively rare hairy cell leukemia is University of Michigan Hospitals, Ann Arbor, Michigan splenectomy the initial treatment of choice. Al- 48109, U.S.A. though this condition was recognized as a distinct 438 World J. Surg. Vol. 9, No. 3, June 1985 entity more than 25 years ago, in the past 10 years operation has been performed primarily for acute other related conditions have been appropriately symptoms and fear of splenic rupture, severe excluded as a result of a better definition of marrow thrombocytopenia, or highly symptomatic spleno- and splenic pathological findings and utilization of megaly [1]. Of 11 patients, 8 were operated on in the the tartrate-resistant acid phosphatase stain which "accelerated phase" of their disease [8] in which is positive in 85-90% of cases. This usually slowly median survival is only 3-6 months. Three died progressive disease is probably a variant form of postoperatively. In 2 patients splenectomy was per- chronic lymphocytic leukemia since the cell of formed as a preliminary to bone marrow transplan- origin appears to be a transformed lymphocyte. tation and both are alive (at 1 and 3 years, respec- Median survival after diagnosis is 3-4 years, but tively). Many reports attributing value to splenec- many patients live for 8-10 years or longer. Re- tomy for this condition have been anecdotal. How- sponses to intensive chemotherapy or to cortico- ever, several recent nonrandomized comparisons of steroids have been poor. Splenectomy is indicated operation versus no operation early in the chronic if the patient develops a hypersplenic complication phase of CGL have shown that splenectomy does such as severe anemia, thrombocytopenia, or not delay progression from the chronic to the blastic granulocytopenia. In our experience with 17 pa- phase and does not prolong survival [9, 10]. In a tients followed at our institution during the past 10 recent prospective trial, similar conclusions were years [1], 16 have had a splenectomy for one of reached [11]. these indications. There have been no operative deaths and only 1 postoperative superficial wound infection. Nine of the 16 splenectomized patients Stage IV Lymphoma are still alive with a median postoperative survival of more than 42 months. Only 2 patients have On rare occasions, patients with far-advanced required postoperative chemotherapy with low- Hodgkin's disease or non-Hodgkin's lymphoma will dose chlorambucil. Thirteen of 16 have achieved a develop severe hypersplenism which is refractory satisfactory hematologic response (hematocrit: 35 to chemotherapy, or a cytopenia interferes with vol % or above; granulocytes: 1,000/mm 3 or more; administration of adequate doses of chemothera- platelets: 100,000/mm 3 or greater) after operation. peutic agents. In our experience with 5 such pa- In a recent analysis of 391 cases [2], patients who tients, 1 died 3 weeks postoperatively with pneu- had a splenectomy survived longer than patients monia. The other 4 achieved a very satisfactory who did not undergo surgery; operative mortality cytologic response with survival from 5 months to rate was 2%. About 80% of patients still achieve an more than 8 years. The entire spectrum of "acceptable hematologic response" (as previously cytopenias were encountered: thrombocytopenia in defined) [2]. In patients who fail to achieve an 1 patient, hemolytic anemia in 1, hemolytic anemia adequate response to splenectomy, low-dose and thrombocytopenia in another, and pancyto- chlorambucil (4 mg/day) has been of value [3]. penia in the fourth. In 3 patients a sustained remis- Recently, bone marrow transplantation [4] or daily sion of hypersplenism was achieved until death administration of alpha interferon [5] have been from lymphoma. The fourth and longest surviving proposed as additional therapeutic modalities. patient had a relapse of a Coombs'-positive hemo- While many patients with chronic lymphocytic lytic anemia 2 years after operation which re- leukemia live for 5-10 years with little or no treat- sponded to steroid therapy and then remained in ment, a small fraction may develop hypersplenic remission until a terminal relapse 81/2 years after complications or massive symptomatic splenomeg- splenectomy. aly, usually relatively late in the course of this disease. Of our 15 patients operated on for one of Sarcoidosis these indications [1], 2 died after operation from persistent thrombocytopenic bleeding. Four pa- Although 6% of patients with sarcoidosis are said to tients with hemolytic anemia, 1 with pancytopenia, have splenomegaly [12], the need for splenectomy and 4 with symptomatic splenomegaly had excellent is extremely uncommon. In the largest reported responses to operation with a mean survival of series, splenectomy was performed for sympto- greater than 4 years. Of 6 patients with thrombo- matic splenomegaly in 7 patients, for infarction or cytopenia, only 2 achieved a satisfactory rise in rupture in 2, for hypersplenism in 5 (all of whom platelet count. Others have reported similar results responded), and for other indications in 2 [13]. In [6, 7]. our personal experience with 8 patients, diagnosis The utilization of splenectomy in the manage- was the indication in 4 individuals who had no prior ment of chronic granulocytic leukemia (CGL) has diagnosis and manifestations compatible with pos- been much more controversial. In our hospital, the sible lymphoma (fever of undetermined origin, W.W. Coon: Splenectomy for Splenomegaly 439 splenomegaly, para-aortic lymphadenopathy, etc.). selection of patients and intensive preoperative One patient had symptomatic splenomegaly. In the preparation are essential. Our experience with a other 3 patients, surgery was performed for hemo- very short postoperative survival in subjects with lytic anemia or neutropenia and all achieved remis- secondary AMM indicates that surgery in this sub- sion after splenectomy. group is of questionable benefit. In patients with chronic anemia requiring frequent transfusion, a source of continued gastrointestinal blood toss Agnogenic Myeloid
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