Hypersplenism; report of a case of intramedullary histiocytic reticulosis showing hypersplenism.
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BACKGROUND AND OBJECTIVES: Hypersplenism is frequently seen in patients with cirrhosis. However, it is unclear why some patients with cirrhosis develop marked hypersplenism and others do not. Additionally, the implications of severe hypersplenism are unknown. Therefore, we conducted a study to evaluate the predictors and implications of severe hypersplenism in patients with cirrhosis. SUBJECTS AND METHODS: All subjects with cirrhosis who were referred to Indiana University over a 53-month period for liver transplantation were studied. Severe hypersplenism was defined as platelet count < 75,000 per mm3 and/or white blood cell count < 2,000 per mm3 in the presence of splenomegaly. The outcomes of interest were development of spontaneous bacterial peritonitis (SBP), variceal bleeding, and death. Patients were observed until death, transplantation, or study closure. RESULTS: The study group comprised 329 subjects with cirrhosis and their median follow-up time was 450 days (0.25-42 months). The prevalence of severe hypersplenism was 33%. Decompensated liver disease [odds ratio (OR), 2.0; 95% confidence interval (CI), 1.1-3.7] and a history of alcohol consumption (OR 2.3; 95% CI, 1.4-3.8) were independent predictors of severe hypersplenism. The presence of severe hypersplenism independently predicted the development of variceal bleeding [hazard ratio (HR) 4.1; 95% CI, 1.7-10], SBP (HR 8.0; 95% CI, 3.1-20.5), and death (HR 2.0; 95% CI 1.2-3.4). CONCLUSIONS: This study suggests that severe hypersplenism is an independent risk factor for developing variceal bleeding, SBP, and death in patients with cirrhosis. If these observations are confirmed, severe hypersplenism can be considered as an indication for prophylactic measures against variceal bleeding and SBP.
OBJECTIVE: To observe the change in expression of Toll-like receptor 4 (TLR4) of splenic macrophage in patients with hypersplenism due to portal hypertension (PH), and investigate the role of TLR4 of splenic macrophage in hypersplenism. METHODS: Splenectomy was performed on 20 patients with hypersplenism due to PH (hypersplenism group) and 6 patients with rupture of spleen by trauma (control group) and the specimens of spleen were collected. The splenic macrophages were isolated by adhibit wall method. The expression of TLR4 of splenic macrophage was detected by immunohistochemical method (SABC), and the result was analyzed by the image analysis system. The phagocytosis of splenic macrophage was measured by chicken red blood cell (CRBC) phagocytosis assay. The level of serum endotoxin was detected before the operation by limulus assay. The results of these 2 groups were compared, and correlation analysis was made among different results of the hypersplenism group. RESULTS: The expression of TLR4 of splenic macrophage was 109 +/- 32 in the hypersplenism group, significantly higher than that of the control group (62 +/- 5, P < 0.01). The rate of phagocytosis and index of phagocytosis of splenic macrophage in the hypersplenism group were 12.6% +/- 3.0% and 0.146 +/- 0.035 respectively, both significantly higher than those of the control group (6.9% +/- 0.5% and 0.076 +/- 0.008 respectively, both P < 0.01) The level of endotoxin of the hypersplenism group was 0.28 EU/ml +/- 0.21 EU/ml, significantly higher than that of the control group (0.054 EU/ml +/- 0.014 EU/ml, P < 0.05). The rate of phagocytosis and the index of phagocytosis of splenic macrophage were notably positively correlated with the expression of TLR4 (r = 0.601, P < 0.01 and r = 0.553, P < 0.05), and the level of endotoxin (r = 0.724 P < 0.01 and r = 0.506, P < 0.05). The expression of TLR4 of splenic macrophage was positively correlated with the level of endotoxin (r = 0.525, P < 0.05). CONCLUSION: The expression of TLR4 of splenic macrophage in patients with hypersplenism due to PH was increased significantly. It may be one of the important factors of hypersplenism due to PH that "endotoxemia-->increase of expression of TLR4 of splenic macrophage (activation of TLR of splenic macrophage)-->increased destruction of red blood cells by macrophage".
BACKGROUND: Portal hypertension is frequently associated with secondary hypersplenism, two common clinical manifestations of which are leukopenia and thrombocytopenia. Surgical portosystemic shunts alleviate portal hypertension but their effect on hypersplenism remains unpredictable. Transjugular intrahepatic portosystemic shunt (TIPS) is a minimally invasive procedure for portal decompression. From current reports it is not clear if TIPS improves hypersplenism in patients with portal hypertension. We present a retrospective review of our experience with TIPS to determine the effect on hypersplenism. PATIENTS AND METHODS: Sixty-five patients who had a TIPS procedure between December 1991 and June 1994 were evaluated retrospectively. The records were specifically reviewed for platelet and white blood cell counts performed before the procedure, within a week after the procedure, and then again within the subsequent 3 weeks. Hypersplenism was defined as thrombocytopenia (platelet count of <100,000/mm3), leukopenia (white blood cell count of <5,000/mm3), or both. RESULTS: Thrombocytopenia alone was present in 33 patients and leukopenia alone in 4 patients before TIPS was performed. Both leukopenia and thrombocytopenia were present in 12 individuals. At least one of these indices of hypersplenism was present in 49 patients. Leukocyte count improved in 11 of 16 patients (69%) whereas platelet count improved in 34 of 45 patients (75%) within a week of the procedure. In the subsequent 3 weeks, leukopenia was relieved in 5 of 10 patients (50%) and thrombocytopenia in 21 of 28 patients (75%), respectively. Of the 12 patients who had both leukopenia and thrombocytopenia before TIPS, the indices improved in 4 patients (33%) within a week of the procedure. Thrombocytopenia was more consistently corrected as opposed to leukopenia, albeit in the short term. CONCLUSION: The TIPS procedure is a promising, minimally invasive method of portal decompression that is effective in the treatment of complications of portal hypertension including secondary hypersplenism.
BACKGROUND: Splenomegaly and hypersplenism occur in patients with chronic liver disease and liver transplant recipients. The traditional treatment for hypersplenism is surgical removal. Percutaneous interventional methods, such as partial splenic embolization, are alternatives to surgery for hypersplenism. This article gives preliminary findings for a new percutaneous technique in which a narrowed stent is placed in the splenic artery. METHODS: The study focused on 10 patients (eight males and two females) who were treated for hypersplenism. Partial splenic embolization was performed in six patients (age range, 1-43 years) who were waiting for liver transplantation, and narrowed stents were placed in four patients (age range, 12-47 years) who had undergone either orthotopic two patients) or heterotopic two patients) liver transplantation. For embolization, the splenic artery was catheterized and polyvinyl alcohol particles were infused to the distal branches, reducing blood flow in the spleen by 40% to 50%. In the other cases, a narrowed stent was deployed to the middle portion of the splenic artery. RESULTS: Hypersplenism was successfully treated in all 10 cases. Compared with partial splenic embolization, placement of narrowed stents was associated with lower frequencies of postintervention fever and pain, shorter hospital stay, and decreased need for antibiotics. In addition to treating hypersplenism, narrowed-stent placement also completely resolved splenic artery steal syndrome in the two patients (orthotopic liver transplant recipients) with this condition. CONCLUSION: Percutaneous placement of a narrowed stent in the splenic artery is a promising new technique for treating hypersplenism and splenic arterial steal syndrome.
Leukopenia, thrombocytopenia, and hemolytic anemia occur commonly in advanced cirrhosis. Some investigators have reported that portacaval anastomosis (PCA) abolished hypersplenism while others have not found PCA to be uniformly beneficial. We compared the frequency of hypersplenism before and after admission to a controlled investigation of the effects of PCA in 52 unoperated control subjects and 38 patients with patent PCA. The two groups were followed for an average period of 5 1/2 years. On admission to the study leukopenia was present in about 2% of patients, thrombocytopenia in 6%, and hemolytic anemia in 4%. Splenomegaly was present in 48% and hypersplenism in 11%. After randomization splenomegaly disappeared more frequently in the shunted group. In addition, fewer patients with PCA developed splenomegaly for the first time after inclusion into the study than did unoperated control subjects. Leukopenia, thrombocytopenia, and hemolytic anemia, when present at inclusion into the study, disappeared with equal frequency in the shunted and unshunted patients, and appeared with equal frequency in both groups after randomization in previously unaffected patients. In no instance was hypersplenism clinically significant nor was splenectomy considered or carried out in any of these 90 patients. In additional uncontrolled studies we observed that therapeutic PCA did not affect hypersplenism differently from prophylactic PCA. We conclude that PCA has neither clinically nor statistically significant effects on hypersplenism.
Partial splenic embolization (PSE) has been demonstrated to be an effective alternative to splenectomy for patients with hypersplenism. Splenectomy in these patients can be associated with an increased risk of perioperative complications, overwhelming post-splenectomy sepsis (OPSS) and mortality. Partial splenic embolization has the advantages of non- operative intervention and resolution of the complications of hypersplenism. We report the use of this technique in patients with portal hypertension and hypersplenism awaiting liver transplant and patients that have undergone othotopic liver transplantation (OLTx) with persistent hypersplenism post-transplant. Six patients--three awaiting liver transplantation and three patients with persistent hypersplenism status post-OLTx--were treated during the period of 1993-99 at the LSUHSC/Willis Knighton Regional Transplant Center in Shreveport, Louisiana. Three patients were male and three female. All six patients had concomitant thrombocytopenia and neutropenia with platelet counts below 50,000. Patients underwent selective arterial catheterization and embolization via a percutaneous approach with Cook microcoils or PVA particles. The lower pole of the spleen was selectively embolized in all patients to achieve a 30-50% reduction in flow as determined by angiography. Patients were followed with routine computed tomography (CT) scans, platelet and WBC counts for a mean of 26 months in the pre-transplant and 37 months in the post-transplant group. In both groups, all patients had persistent resolution of thrombocytopenia and neutropenia after embolization. In the post-transplant group, one patient had persistent splenomegaly and required splenectomy for pain control. No procedure-related complications occurred in any patient. In this limited review, PSE appears to be a safe and effective treatment of persistent hypersplenism in patients with portal hypertension and those who have undergone OLTx.
Blood volume in "hypersplenic" and normal rats was assessed by a simultaneous measurement of erythrocyte and plasma volumes by means of 59Fe-labelled erythrocytes and 131I-labelled human serum albumin, respectively. The "hypersplenic" condition was induced by prolonged intraperitoneal application of methylcellulose. Mean blood volume in normal rats was 6.3 ml/100 g body weight, the venous haematocrit being 48%. Mean blood volume in "hypersplenic" rats was 7.5 ml/100 g body weight, and the venous haematocrit lower by 22% than in normal animals. Compared with normal animals, the erythrocyte volume in "hypersplenic" rats was lower by 15% only. Plasma volume in "hypersplenic" rats exceeded the compensation in response to the reduction in erythrocyte mass. In addition to haemolysis, haemodilution due to plasma expansion seemed to be responsible for the anaemia in "hypersplenic" rats.
This is a retrospective study of 14 patients with lymphosarcoma with hypersplenism. Eight patients received radiation to the spleen followed by splenectomy for recurrence of hypersplenism. Six patients underwent primary splenectomy for hypersplenism. Radiation decreases the size of the spleen but recurrence of hypersplenism is seen in an average of 8 mo. Splenectomy in these cases improves the hematological picture. Average postsplenectomy survival is 27.6 mo. Splenectomy alone in hypersplenism also improves the hematological picture. Postsplenectomy survival in this group is 13 mo. One case of primary lymphosarcoma of the spleen is reported.
Hypersplenism frequently accompanies cirrhosis with portal hypertension. In this series of 76 patients, 36 percent had thrombocytopenia, 41 percent had leukopenia, and 25 percent had both thrombocytopenia and leukopenia. However, hypersplenism was severe enough to necessitate splenectomy in only two patients (3 percent). Nonalcoholic cirrhotic patients exhibit hypersplenism more frequently and to a greater magnitude than do alcoholic cirrhotic patients. Fourteen and 44 percent of alcoholic and nonalcoholic cirrhotics, respectively, had both thrombocytopenia and leukopenia. Distal splenorenal shunts and nonselective shunts are equally effective in relieving preoperative hypersplenism. Approximately two thirds of the patients were relieved of thrombocytopenia or leukopenia after either of these procedures. Splenectomy invariably corrects hypersplenism associated with cirrhosis and should be included as part of the operative procedure in patients requiring surgery for control of variceal hemorrhage.
The register of Latvian children with portal hypertension (PH) includes 34 patients from 2 to 16 years old. In 26 children the PH is caused by a prehepatic obstruction. The most widespread symptoms of PH are variceal bleeding and hypersplenism. The traditional management of hypersplenism has been splenectomy. We have used partial splenic embolization (PSE) as an alternative to splenectomy. From 1998 to 2000 we carried out PSE in 8 patients with PH. A percutaneous femoral artery approach to the splenic artery was used to deliver microspheres with a diameter of 0.3 mm into the spleen, minimizing blood circulation to the organ by 60 - 70 %. In 7 patients symptoms of hypersplenism disappeared and blood tests returned to normal status. In one patient we carried out PSE twice. In one case PSE was not successful and the patient underwent partial resection. Abdominal pain and subfebrility were observed on average 2 weeks after PSE. In 7 patients the 4 - 20 months follow-up period demonstrated the efficiency of the non-operative hypersplenism management method. We conclude that PSE is the method of choice to treat hypersplenism.
BACKGROUND: Hepatocellular carcinoma (HCC) is a common disease with high mortality and serious effect on the life quality of patients. Operation is still the most effective treatment. Currently, in China, patients with HCC are often complicated by hepatitis B related liver cirrhosis and secondary hypersplenism. This study was undertaken to evaluate the effect and indications of synchronous hepatectomy and splenectomy for HCC patients with hypersplenism. METHODS: The clinical records and treating processes of 24 patients with HCC and hypersplenism during the period of January 1991 to July 2004 were analyzed retrospectively. RESULTS: Sixteen patients underwent hepatectomy and splenectomy, including extensive devascularizasion around the cardia (9 patients). Seven patients were treated with microwave ablation and splenectomy plus extensive esophagogastric devascularization. One patient underwent hepatectomy combined with microwave ablation and splenectomy plus extensive esophagogastric devascularization. There were no deaths during the operation. During the first week after operation, the symptoms of hypersplenism disappeared and the platelet (Plt) and white blood cell (WBC) counts were significantly elevated (Plt: 247 X 10(9)/L vs. 45.9 X 10(9)/L, WBC: 13.0 X 10(9)/L vs. 3.3 X 10(9)/L, P<0.01). CONCLUSIONS: Synchronous splenectomy can increase the safety of hepatectomy in selected patients with HCC and secondary hypersplenism by reducing bleeding complications. Splenectomy enhances patients' immunity against tumor in a long period as well.
The effect of the distal spleno-renal shunt on hypersplenism has been investigated on 19 out of 20 patients undergoing this procedure at the 2nd Department of Surgery Pizzamiglio, Niguarda Hospital of Milan. 6 patients had an hypersplenism of high degree (platelet count less than 50;000/mm3), 11 of moderate degree (platelet count greater than or equal to 50,000 and less than 100,000/mm3) and 2 of low degree (platelet count greater than 100,000 and less than 100,000/mm3). Platelet count significantly improved after the operation (p less than 0.01) and hypersplenism resulted cured or ameliorated in 17 out of 19 patients under study. After briefly discussing the pathogenesis of hypersplenism in patients bearing portal hypertension, the A. conclude that hypersplenism also severe is not "per se" a contraindication to Warren shunt.
PURPOSE: To study the value of partial splenic embolization (PSE) for the treatment of hypersplenism in patients undergoing embolization of hepatocellular carcinoma (HCC). METHODS: Transcatheter hepatic arterial embolization (THAE) combined with PSE was performed in 30 patients with HCC complicating liver cirrhosis, portal hypertension, and hypersplenism. Gelfoam sponge was used as the embolic material for PSE and limited to 100-150 pieces. RESULTS: More than 50% of splenic parenchyma was infarcted in 27 patients. Leukopenia and thrombocytopenia were corrected by PSE in 25 of 27 patients with hypersplenism. In 26 patients with esophageal varices, including 5 patients with bleeding, no rebleeding occurred during a 6-17 month follow-up. Hypersplenism was not corrected in 2 of 3 patients whose infarcted splenic parenchyma was less than 50%. No splenic abscesses or other severe complications were observed. Of the 30 patients treated, 19 are still alive after 1 year. CONCLUSIONS: THAE combined with PSE is a safe and effective measure for patients with HCC.
PURPOSE: Massive splenomegaly with severe hypersplenism can occur as a late complication of portal hypertension (PH) caused by extrahepatic portal venous obstruction (EHPVO) in children. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy (EST) and shunt surgery. We report our experience of managing this disorder surgically. METHODS: We performed splenectomy and esophagogastric devascularization via laparotomy in 14 children with an average age of 9.7 years. Upper gastrointestinal endoscopy had shown esophageal varices of varying grade, and EST had been done for patients with a history of bleeding. The indications for surgery were pain and discomfort caused by a large spleen greater than 15 cm below the costal margin, and intractable symptomatic hypersplenism with a total leukocyte count <2500/mm3 and a platelet count <50,000/mm3, or both. RESULTS: Postoperative recovery was uneventful and the leukocyte and platelet counts reverted to normal. After follow-up for 1-5 years, all 14 children were asymptomatic, with improved growth and nutrition and no reported episodes of gastrointestinal bleeding, sepsis, or encephalopathy. CONCLUSION: Splenectomy with devascularization is effective for children with massive splenomegaly and severe hypersplenism secondary to EHPVO.
BACKGROUND: For many years, the hematologic changes occurring in hepatosplenic Manson's schistosomiasis have been defined as hypersplenism. Initially, the belief was that removal of the spleen would normalize the hematologic values. However, hematimetric normalization was observed in surgeries for the treatment of portal hypertension in which the spleen was preserved. In view of these findings, it is necessary to verify the clinical and laboratory profile of these patients in order to define the real presence of hypersplenism. METHODS: This study was conducted on 51 patients with schistosomatic portal hypertension divided into five groups: group 1, non-operated patients under clinical control; group 2, patients submitted to distal splenorenal shunt; group 3, patients with subtotal splenectomy and central splenorenal shunt; group 4, patients with total splenectomy and central splenorenal shunt; and group 5, patients with total splenectomy and portal-variceal disconnection. Clinical signs of hypersplenism were assessed in all patients. Hematologic values of the pre- and postoperative period were compared. Immunoglobulin counts of the patients were verified in the late postoperative period. RESULTS: In all operated groups, hematimetric values, leukocyte counts, platelet number and prothrombin time were higher during the postoperative period. With respect to the immunoglobulins, the postoperative values of IgM, IgG and IgA were normal. CONCLUSIONS: Patients with schistosomatic portal hypertension and peripheral cytopenia cannot be classified as having hypersplenism but rather as having pancytopenia due to splenomegaly caused by difficulty on portal blood drainage.