Surgical Splenectomy Research Articles

Role of partial splenic arterial embolization for hypersplenism in patients with liver cirrhosis and thrombocytopenia

Hypersplenism is traditionally treated by surgical splenectomy. Transcatheter ablation of splenic parenchyma is an alternative treatment modality. We evaluated the efficacy and safety of partial splenic arterial embolization in 10 patients with chronic liver disease and hypersplenism with thrombocytopenia (platelet count <80,000/microL). At six months follow up, median (range) platelet counts (134.5 [71.5-164] x 10(3)/microL) were significantly higher than those before treatment (33.5 [23-39] x 10(3)/microL; p<0.05]). All patients developed post-embolization syndrome. Left-sided pleural effusion and increase in amount or new development of ascites occurred in six and five patients, respectively. Our data suggest that partial splenic arterial embolization leads to an increase in platelet count in patients with thrombocytopenia due to chronic liver disease and hypersplenism. However, it is often associated with complications.

Oral Candida flora in a group of Jordanian patients with β-thalassemia major

Thalassemic patients present with multiple immune abnormalities that may predispose them to oral Candida, however this has not been investigated. The aim of this study was to assess oral candidal colonization in a group of patients with beta-thalassemia major both qualitatively and quantitatively. The oral mycologic flora of 50 beta-thalassemia major patients and 50 age- and sex-matched control subjects was assessed using the concentrated oral rinse technique. Candida species were identified using the germ tube test and the Vitek yeast identification system. Oral Candida was isolated from 37 patients (74%) and 28 healthy subjects (56%; P = .04). The mean candidal count was significantly higher in thalassemic patients compared with the healthy group (P < .05) and in patients who had surgical splenectomy compared with nonsplenectomized patients (P = .04). Oral Candida colonization and candidal counts are significantly higher in beta-thalassemia major patients than in healthy subjects. Surgical splenectomy may increase the quantity of colonizing oral candidal organisms in thalassemic patients.

Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS).

ALPS is a disorder of apoptosis resulting in accumulation of autoreactive lymphocytes, leading to marked lymphadenopathy, hepatosplenomegaly, and multilineage cytopenias due to splenic sequestration and/or autoimmune destruction often presenting in childhood. We summarize our experience of rituximab use during the last 8 years in 12 patients, 9 children, and 3 adults, out of 259 individuals with ALPS, belonging to 166 families currently enrolled in studies at the National Institutes of Health. Refractory immune thrombocytopenia (platelet count <20,000) in nine patients and autoimmune hemolytic anemia (AIHA) in three patients led to treatment with rituximab. Among them, seven patients had undergone prior surgical splenectomy; three had significant splenomegaly; and two had no palpable spleen. In seven out of nine patients with ALPS and thrombocytopenia, rituximab therapy led to median response duration of 21 months (range 14-36 months). In contrast, none of the three children treated with rituximab for AIHA responded. Noted toxicities included profound and prolonged hypogammaglobulinemia in three patients requiring replacement IVIG, total absence of antibody response to polysaccharide vaccines lasting up to 4 years after rituximab infusions in one patient and prolonged neutropenia in one patient. Toxicities including hypogammaglobulinemia and neutropenia constitute an additional infection risk burden, especially in asplenic individuals, and may warrant avoidance of rituximab until other immunosuppressive medication options are exhausted. Long-term follow-up of ALPS patients with cytopenias after any treatment is necessary to determine relative risks and benefits.

What is a reliable CT scan for diagnosing splenosis under emergency conditions?

Splenosis is a condition in which splenic tissue is present in a non-anatomical position. Implants of splenic tissue can mimic neoplasms and only specific examinations can confirm the correct diagnosis. Here we report a case of a 23-year-old male patient with a history of surgical splenectomy during childhood after trauma. He was admitted to the emergency department with acute bowel obstruction. An abdominal-pelvic computed tomography (CT) scan revealed small bowel obstruction and the presence of two rounded, solid masses located in the rectal-vescical pouch. Quantitative analyses of the different density values in the arterial phase and early portal venous phase demonstrated that these lesions were highly vascularised (92 and 97 Hounsfield Units, respectively). The hypothesis of an ectopic splenic mass was made after evaluation of the CT images and clinical history. The acute bowel obstruction caused by adhesive intestinal syndrome was resolved by surgical adhesiolysis. The smallest mass adherent to the rectum was removed. Histopathologic examination confirmed the benign nature of the lesion, which consisted of splenic tissue.

When a car accident can change the life: splenic lymphoma and not post-traumatic hematoma

In the last years, technological advance of high-resolution radiological imaging has contributed to the development of the situation during which clinically inapparent masses are discovered during studies performed for other reasons. Such findings, usually defined as incidentalomas, are relatively frequent for endocrine, but hormonally inactive, masses. However, the term incidentaloma, originally limited to endocrine masses, has been further more widely used for any casual mass, discovered by procedures performed for other reasons. A 71-year-old woman was admitted to the hospital complaining of persisting pain to the left knee and right hand. Two weeks before, she had a car accident with mild but multiple contusions. In her medical history, only an asymptomatic colelithiasis. On physical examination, axillary temperature was 36.5 C (95.9 F), arterial blood pressure 140/80 mmHg, regular heart beat 80 beats/min, with no heart murmur. There were no pulmonary noises. Examination of the abdomen revealed only a palpable, but painless, splenomegaly. Swelling and pain were limited to the left knee and right hand. Laboratory data showed: white blood count 5,300/mm (63% neutrophils); hemoglobin 12.9 g/dL; platelets 188,000/mm3; creatinine 0.8 mg/dL; glucose 129 mg/dL; fibrinogen 210 mg/dL; alanine-aminotransferase 25 U/L, alkaline phosphatase 294 U/L, lactate dehydrogenase 451 U/L, total bilirubin 0.54 mg/dL, c-glutamyltranspeptidase 42 U/L; prothrombin time international normalized ratio 1.05, potassium 4.3 mEq/L, sodium 146 mEq/L, chloride 108 mEq/L, D-dimer 1,571 ng/mL; erythrocyte sedimentation rate 21 mm; C-reactive protein 0.1 mg/dL. Urinalysis was normal. Ultrasonography of the knee showed a mild periarticular fluid flap. Chest radiography was negative and venous echocolor-doppler of the lower limbs excluded deep vein thrombosis. Abdominal ultrasonography revealed a gross splenic mass (13 9 9 cm), with the aspects of organized hematoma. Computerized tomography confirmed the presence of a solid ovalar mass, but with not univocal interpretation. Contrast-enhanced ultrasonography suggested the possibility of a neoplastic mass, deserving surgical approach. Thus, the patient underwent surgical splenectomy with interaortico-caval lymphadenectomy. The spleen showed increased dimensions (15 9 12 9 10 cm; weight 900 g), and the parenchyma was quite fully occupied by lardaceous and necrotic tissue. Histopathology of the spleen, the lymphnodes, and the adipose tissue, was positive for malignant lymphomonocitic B-cell lymphoma, with a monotypic IgG component, monoclonal for K light chains at the in situ hybridization, marked histiocitary granulomatous hepiteliod and gigantocellular reaction, and amyloid deposit in the spleen (polymorphic immunocytoma). The patient was then addressed to the Hematology Department. Primary diseases of the spleen are relatively unfrequent. The spleen, in fact, is more often a secondary site involved in the course of traumatic, infectious, immunological, vascular, and onco-hematologic diseases [1, 2]. As for lymphomas, splenic involvement is usually common, even if accurate imagine techniques are needed [3]. Oncohematologic diseases of the spleen may present with a R. Salmi P. Gaudenzi F. Di Todaro P. Morandi I. Nielsen Medicina ad Alta Rotazione, Azienda Ospedaliera-Universitaria S.Anna, Ferrara, Italy

Radiation therapy for palliation of Eisenmenger’s syndrome-associated painful splenomegaly

Painful splenomegaly has a clinical presentation that is often associated with myeloproliferative disorders, such as acute or chronic lymphoblastic or myelogenous leukemia. In these situations low-dose radiotherapy is effective in reducing the splenomegaly and relieving pain. The potential benefit of radiotherapy for cardiogenic splenomegaly is less well established. The present report discusses a case in which radiotherapy was employed to benefit a patient with Eisenmenger's-associated painful splenomegaly. Because of the patient's high anesthesia risk, palliative surgical splenectomy was not feasible. The patient underwent three-dimensional conformal treatment planning, and a total of 42.5 Gy at 2.5 Gy per fraction was prescribed to the spleen. At 4 months following radiotherapy completion, the patient reported durable pain relief and no untoward small bowel effects; moreover, there was a 43% reduction in splenic volume on follow-up CT. Although there have been previous reports of hematological and myeloproliferative-associated splenomegaly that have been treated with a lower dose per fraction and lower total dose radiotherapy, we advocate the use of 2.0-2.5 Gy per fraction to a total dose approaching 40 Gy for adequate duration of response when treating cardiogenic-associated painful splenomegaly in patients for whom surgical splenectomy cannot be performed.

Prediction of Response to Splenectomy in Patients with Idiopathic Thrombocytopenic Purpura

Chronic immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by destruction of opsonized platelets in the reticuloendothelial system, particularly the spleen, and by resulting low platelet counts. Splenectomy is the standard second-line treatment for patients with ITP who do not respond to corticosteroids. In the present study we determined predictive factors for the response of patients with ITP to splenectomy. For thirty-one patients with ITP referred from the Hematology Department of Shariati Hospital, open surgical splenectomy was performed between February 2002 and December 2004. All potentially important variables of response were collected and analyzed to determine predictive parameters. Older patients (>52) responded less positively than younger patients (<52) to splenectomy (p<0.01). Likewise, patients with a sustained remission after splenectomy had a significantly shorter interval of diagnosis of ITP to splenectomy (p<0.05) and shorter duration of corticosteroid therapy before splenectomy (p<0.05). This study confirms that splenectomy is an effective treatment for patients with ITP. Our findings highlight age as the major predictive factor of long-term response to splenectomy in patients with ITP.

Splenic Embolization in Liver Transplant Recipients: Early Outcomes

Clinical improvement has been reported following splenic embolization for a wide variety of indications. Improvement following splenic embolization has been described in cirrhotic patients awaiting hepatic transplantation who are not candidates for surgical splenectomy. Occasionally, patients who have undergone hepatic transplantation have conditions that may also benefit from nonsurgical intervention with splenic embolization. Indications include persistent hypersplenism and pancytopenia precluding optimal treatment with antiviral therapy or chemotherapy, risk for persistent gastroesophageal variceal hemorrhage, and splenic artery steal syndrome attenuating hepatic arterial perfusion. Limited data is available on the outcome of splenic embolization in liver transplant recipients. We present the early outcomes of liver transplant recipients who were treated with splenic embolization. A retrospective chart review of all liver transplant recipients who underwent splenic embolization between 1997 and 2006 was performed, under minimal-risk study approval by the institutional review board. Five liver transplant recipients received splenic embolization: 3 for persistent hypersplenism, 1 for increased risk of gastroesophageal variceal hemorrhage, and 1 for splenic artery steal syndrome. The patients with hypersplenism demonstrated hematologic improvement, the patient with gastroesophageal varices did not experience any hemorrhage on follow-up, and the patient with splenic artery steal experienced resolution of the steal phenomenon. Postembolization syndrome was observed but no splenic abscess or death occurred. Mean follow-up was 20.2 months. In conclusion, splenic embolization is a safe and effective nonsurgical alternative for a variety of indications in liver transplant recipients.

Use of Rituximab for Refractory Immune Cytopenias Associated with Autoimmune Lymphoproliferative Syndrome (ALPS).

ALPS is a heritable disorder of apoptosis resulting in accumulation of autoreactive lymphocytes, leading to childhood onset of marked lymphadenopathy, hepatosplenomegaly with multilineage cytopenias due to splenic sequestration and/or autoimmunity. Affected individuals have characteristic increases (≥1%) in circulating (TCRαβ+ CD4-CD8-) double negative T lymphocytes. ALPS Type Ia, Ib, II and IV are associated with genetic mutations in Fas, FasL, Caspases and NRAS respectively, while patients without identified mutations are classified as Type III or ALPS phenotype. Of the estimated 350 ALPS patients reported worldwide, 241 individuals with ALPS, belonging to 166 families are enrolled in studies at the NIH Clinical Center. Nearly 50% of them with refractory cytopenias associated with hypersplenism have undergone splenectomy leading to associated long term risk of pneumococcal sepsis. Currently published reports include occasional cases of rituximab used for refractory ITP and AIHA with variable response in patients classified as ALPS. We summarize experience of rituximab use during the last 7yrs in 11 ALPS patients (8 males and 3 females), including 2 adults aged 45, 46yrs and 9 children with a median age of 11yrs (range 1–14yrs) at commencement of this therapy for refractory grade 4 immune thrombocytopenia (platelet count <20,000) in 8 and AIHA in 3 patients. Eight of them are ALPS Type Ia and 3 are ALPS phenotype. The 46yr old adult male patient had hepatitis C and ALPS Type Ia comorbidity. Among them, 7 patients were asplenic (prior surgical splenectomy); 3 had significant splenomegaly; and 1 had no palpable spleen. Interventions prior to rituximab therapy included: red cell transfusions, IVIG, WinRho, corticosteroids and mycophenolate mofetil (MMF). Seven patients (including 2 adults) with thrombocytopenia responded with durable normalization of platelet counts over a > 12 months follow up; 2 of them (aged 14 and 15yrs) relapsed at +18 and +22 months after completion of therapy: only one of them responded to a second course of rituximab. Thrombocytopenia in the second patient is currently under control with MMF. One out of the 4 non-responders underwent splenectomy, while the other 3 patients, including the 2 asplenics, required prednisone pulses (5–30 mg/kg) followed by MMF and weekly vincristine for 6 wks (in 1 patient) as a steroid sparing measure. Noted toxicities include prolonged neutropenia in 1 patient, profound and prolonged hypogammaglobulinemia in 4 patients requiring replacement IVIG, and total absence of antibody response to polysaccharide vaccines lasting up to 4years after rituximab infusions in 1 patient. Rituximab therapy relieved thrombocytopenia for 12–18 months in 7 out of 11 ALPS patients. However associated toxicities, including quantitative and qualitative IgG deficiencies that are additional infection risk burden, especially in asplenic individuals, warrant further investigation and long term follow up of ALPS patients with cytopenias.

Splenic Pregnancy

Splenic pregnancy is extremely rare, with only a few previously well-documented cases reported. It usually presents with upper left abdominal pain and carries a high risk of potentially uncontrollable, life-threatening intraperitoneal bleeding. In the past, preoperative confirmation of this entity was very difficult and often required emergency surgical intervention and splenectomy. However, thanks to modern diagnostic technologies such as sonography and computed tomography (CT), it is now possible to make a diagnosis at an early stage without surgery. This report describes a case of an ectopic splenic pregnancy with an unusual clinical presentation. It highlights the potential benefits and importance of selecting appropriate imaging modalities to identify the implantation site of an ectopic gestational sac.

Taking NOTES: translumenal flexible endoscopy and endoscopic surgery

To review the current state of natural orifice surgery and examine the concerns, challenges, and opportunities presented by translumenal research. Translumenal endoscopic procedures have been the focus of extensive research. Researchers have reported natural orifice translumenal endoscopic surgery in a swine model in several areas involving the abdominal cavity. Diagnostic procedures have included endoscopic peritoneoscopy, liver biopsy, lymphadenectomy, and abdominal exploration. Several gynecologic procedures including tubal ligation, oophorectomy, and partial hysterectomy have been demonstrated using current commercial endoscopes. Gastrointestinal surgical procedures, including gastrojejunostomy, cholecystectomy, splenectomy, and distal pancreatectomy have been performed successfully via transgastric and/or transcolonic approaches. There have been no studies of natural orifice translumenal endoscopic surgery procedures published in humans. While fundamental questions about the emerging technology have not been scrutinized, limitations of the large animal model will pose a challenge to the development of large randomized trials. While natural orifice translumenal endoscopic surgery may represent a paradigm shift and may offer significant benefits to patients, rigorous testing of the techniques is lacking and current data have been drawn from case series.

Role of Somatic Fas Mutations in the Pathogenesis of Autoimmune Lymphoproliferative Syndrome (ALPS).

ALPS is a disorder of lymphocyte apoptosis associated with expansion of double negative T cells (DNT; TCRα/β+CD3+CD4−CD8−), an in vitro apoptosis defect and affected individuals typically present in early childhood with nonmalignant lymphadenopathy, splenomegaly, and multilineage cytopenias due to splenic sequestration and/or peripheral autoimmune destruction. ALPS classifications include Type Ia, Ib, and II associated with mutations in Fas, FasL, and Caspases, respectively, and Type III when the apoptosis pathway mutation is unidentified. Among our cohort of more than 200 patients, ALPS Type Ia with germline Fas mutations constitute the most common subtype (over 70%). These patients show an in vitro Fas mediated lymphocyte apoptosis defect and have a known increased risk of lymphoma. However, there are a number of patients that have an “ALPS-phenotype” manifesting typical historical and clinical features of ALPS and present with elevated circulating DNT cells, but have no demonstrable in vitro apoptosis defect. Based on the work of Holzelova et. al (NEJM , 2004, 351:1409) in which six such patients were reported with somatic Fas mutations in their DNT cell subsets, we sought to characterize our patient set without defined mutations. DNT cells from 20 patients, including 8 with ALPS-phenotype and 12 with ALPS Type III, were isolated using magnetic bead separation of frozen PBMCs. The sensitivity for PCR followed by sequencing required >20% abnormal cells using a mixture of normal and ALPS Type Ia PBMCs. However, the sensitivity proved to be lower (40%) using purified DNT cells from patients with potential somatic Fas mutations. Therefore, we chose an ungated DNT purity of 50% as a minimum requirement. Ungated DNT purities for the 20 evaluated patients ranged from 51% to 92%, with a mean of 66%. All nine exons of the Fas gene were PCR amplified prior to sequencing.We found that 3/8 (37.5%) patients with ALPS-phenotype had somatic mutations in the Fas gene: 825G−>T, 846(−9)del11, and 846(−1)G−>T, with the first two mutations being new. Only 1/12 (8.3%) ALPS Type III patients had a somatic Fas muation: 851delAG, which also represents a new mutation. These four somatic mutations were considered significant as two were in a splice site and two were in coding regions. The ALPS Type III patient is atypical given a late age of presentation at age 17 and had milder cytopenias. The three ALPS-phenotype patients with somatic Fas mutations presented similar to cases of “classic” ALPS, with an early age of presentation (median, 1 yr; range, 4 months to 4 years), cytopenias and features of autoimmunity; two require chronic immunosuppression with Cellcept (mycophenolate mofitil) for chronic persistent cytopenias, one having previously undergone surgical splenectomy. From these data we conclude that patients with ALPS-phenotype or atypical ALPS Type III should be screened for somatic mutations in their peripheral blood DNT cell subset using ungated DNT purities of >50%, with priority given to evaluation by sequencing of exons 7–9 of the Fas gene. It is not yet evident if there is an increased risk of lymphoma in patients with somatic Fas mutations as is the case with intracellular germline Fas mutations. Somatic Fas mutations explain the molecular basis of a small subset of patients and are more readily observed in ALPS-phenotype patients.

Splenic Artery Embolization for Non-Bleeding Gastric Varices and Hypersplenism Due to Isolated Splenic Vein Thrombosis

Splenic artery embolization has been used with success for treatment of bleeding gastric varices secondary to splenic vein thrombosis. We present a case of isolated splenic vein thrombosis causing non-bleeding gastric varices and hypersplenism which was successfully treated with splenic artery embolization. A 56 year old man with hemachromatosis previously treated with phlebotomy presented with intermittent epigastric abdominal pain and thrombocytopenia. The subsequent workup included an evaluation for cirrhosis and portal hypertension including a liver biopsy and hepatic wedge pressure measurements which were normal. Further evaluation included a normal bone marrow biopsy. A CT scan and duplex ultrasound revealed an enlarged spleen of 15 cm as well as a suggestion of gastric varices and nonvisualization of the splenic vein. Splenic vein thrombosis was confirmed by MR venogram. Hematological evaluation revealed no hypercoagulable state and there was no history of pancreatitis or other explanation for the isolated finding of splenic vein thrombosis. Subsequently, an EGD revealed significant gastric varices. It was decided that a surgical splenectomy would be high risk due to multiple abdominal venous collaterals and that a medical splenectomy with splenic artery embolization is indicated to decompress venous collaterals prior to a planned surgical splenectomy. After the embolization, follow-up endoscopies have shown improvement in gastric varices and repeat laboratory evaluation has shown normalization of platelet counts even after ten months of follow-up. The decision was made not to proceed with surgical splenectomy at this time. There are only a few reported cases of isolated splenic vein thrombosis without a clear etiology. Splenic vein thrombosis is well known to cause hypersplenism and gastric varices. Splenic artery embolization has been described for 25 years and is mainly utilized in patients with bleeding gastric varices and hypersplenism, usually prior to splenectomy. This procedure is traditionally reserved for high risk surgical patients as it has been reported to have complications such as hematoma or splenic abscess. We suggest this procedure as an effective treatment modality while monitoring clinically for complications or evidence of splenic regeneration at which time surgical therapy could be considered.

Opportunity for better protection of children with sickle hemoglobinopathy

Adamkiewicz et al provide data from 8 regional pediatric sickle cell programs on invasive episodes of Staphylococcus pneumoniae for the 3 years leading up to universal use of the 7-serotype pneumococcal conjugate vaccine (PCV7). They highlight opportunities for combined use of PCV7 with booster dose(s) of the 23-serotype pneumococcal polysaccharide vaccine (PPV23) to especially increase protection against penicillin nonsusceptible strains, serotypes not included in PCV7, and risks occurring across all age groups. The authors concur with recommending bodies that suggest that until we know more, penicillin prophylaxis should be used in all children with sickle hemoglobinopathy until age 5 years. They also suggest that continued prophylaxis beyond 5 years should be given to certain children with increased risk, eg, those with previous invasive infection and those who have undergone surgical splenectomy. Page 438

Invasive pneumococcal infections in children with asplenia.

Asplenia is associated with an increased risk of infections caused by Streptococcus pneumoniae. Overwhelming infection can be fulminate and lead to a fatal outcome. To review the epidemiology and clinical course of invasive S. pneumoniae infections in children with asplenia before the release of the conjugate pneumococcal vaccine. Children with S. pneumoniae infections from eight children's hospitals in the US were identified prospectively from September, 1993, to August, 1999. Further demographic, medical and microbiologic information was gathered retrospectively from the charts of patients with asplenia. Twenty-two asplenic patients with 26 episodes of invasive S. pneumoniae were identified. This represents 1% of the 2,581 episodes of invasive S. pneumoniae infections identified in our study. Twelve had congenital asplenia (CA), and 10 had undergone surgical splenectomy. Nine of the patients with CA had associated complex congenital heart disease. The median age at first infection was 12.5 months for CA patients as compared with 69 months in children with surgical splenectomy (P < 0.001). Seventy-five percent of those eligible had received the polysaccharide pneumococcal vaccine. The most common serotypes isolated were 6B (8), 23F (7), 18C (2) and 19A (2). Antimicrobial prophylaxis had been prescribed for 82% of the study cohort. Clinical presentations of the 26 episodes included fever (22), shock (7), petechiae or purpura (7), disseminated intravascular coagulation (5) and respiratory distress (5). Clinical illness included bacteremia alone (12), meningitis alone (8), bacteremia with otitis media-sinusitis (3), bacteremia with pneumonia (2) and meningitis with osteomyelitis (1). Five of the 6 patients who died had meningitis. Three of the survivors (19%) had significant morbidity, and all of them had meningitis. Two patients had 2 episodes each, and 1 patient had 3 episodes. All but 1 of the multiple episodes was with a different serotype. Forty-six percent of isolates were nonsusceptible to penicillin, and 19% were nonsusceptible to ceftriaxone. There was no association between antimicrobial resistance and mortality. Invasive pneumococcal disease in patients with asplenia has a high mortality, especially in those with meningitis. Even though the new conjugate vaccine might increase protection, 19% of patients had disease caused by serotypes not included in the current heptavalent vaccine. Clinicians should continue to be aggressive in evaluating asplenic patients with unexplained fevers.

An unusual cause of massive upper GI bleeding: dissecting hematoma of the gastric wall occurring three months after surgical splenectomy

An unusual cause of massive upper GI bleeding: dissecting hematoma of the gastric wall occurring three months after surgical splenectomy

Overwhelming infection in asplenic patients: current best practice preventive measures are not being followed

Aims—Patients without spleens are at increased risk of overwhelming infection. Recently, greater efforts, including the publication of national guidelines, have been made to improve the management of asplenic individuals. In...

The response to splenectomy in pediatric patients with idiopathic thrombocytopenic purpura who fail high-dose intravenous immune globulin

Background/Purpose: A recent article by Law et al concluded that patients with idiopathic thrombocytopenic purpura (ITP) who have a poor response to intravenous immune globulin (IgG) are unlikely to have a good or excellent response to surgical splenectomy. Methods: The authors studied retrospectively 23 pediatric patients age 11.7 ± 1.0 years with ITP who had been treated with IgG before undergoing splenectomy. As in the aforementioned article, the responses to the 2 treatments were classified on the basis of the platelet count as poor (<50,000/mm3), good (50,000 to 150,000/mm3), or excellent (>150,000/mm3). For patients who received multiple IgG treatments, both initial and final treatment responses were analyzed. Results: Sixteen patients had an excellent or good initial response to IgG. Of these 16 patients, 14 had an excellent or good response to splenectomy. Among the 7 patients who had a poor response to IgG there were 3 who had an excellent or good response to splenectomy (43%), and 4 patients who had a poor response to splenectomy. A good or excellent response to initial treatment with IgG was associated with a significant probability of a good or excellent response to splenectomy (P =.045). Conclusions: A good or excellent response to IgG may be predictive of a favorable response to splenectomy. However, a poor response to IgG does not preclude a satisfactory response to splenectomy in pediatric patients with ITP. J Pediatr Surg 35:967-972. Copyright © 2000 by W.B. Saunders Company.

The spleen and splenectomy in immune (idiopathic) thrombocytopenic purpura

The benefits of surgical splenectomy in patients with immune (idiopathic) thrombocytopenia purpura (ITP) probably reflect the combined effects of eliminating a source of antiplatelet antibody synthesis as well as the primary site of platelet destruction. The recent availability of intravenous Rho(D) immune globulin (WinRho SDF ™; Nabi, Boca Raton, FL) presents an opportunity to extend the duration of nonsurgical (spleen-sparing) management of chronic ITP by inducing reversible Fc blockade. While new methods for laparoscopic splenectomy may offer improved surgical outcomes and reduced costs for ITP patients in the near-term, the long-term consequences of splenectomy remain to be determined. Partial splenectomy has been shown to be effective in the management of anemia in hereditary spherocytosis and elliptocytosis, while preserving vital splenic phagocytic and immune functions. The concept that cell destruction occurs in reticuloendothelial cells has been updated with recognition that the mononuclear phagocyte is neither a reticular nor an endothelial cell. Immune phagocytosis is now understood to be mediated by macrophage IgG Fc and complement receptors. A key factor for devising a strategy for selecting medical or surgical splenectomy, or postponing splenectomy, is an assessment of the relative importance of splenic immune versus phagocytic function in the pathogenesis of ITP.

The effect of dosage, gestational age and splenectomy on anti-IgM interception of prenatal B-cell development in sheep.

The administration of a single bolus of anti-IgM antibody to foetal lambs early in pregnancy produces prolonged B-cell depletion. The present study investigated this depletion by examining the effect, on B-cell development in the ileal Peyer's patches, of varying the timing and dosage of antibody administration and by supplementing anti-IgM with surgical splenectomy. The capacity of a 1 mg bolus of anti-IgM to deplete Peyer's patches of B cells was lost if its administration was deferred until two thirds of the way through pregnancy, but persisted beyond this time if weight-adjusted doses were used. Splenectomy of the foetus performed at an earlier age failed to extend the age at which a 1 mg dose of antibody remained effective. As the concentration of murine immunoglobulin in foetal serum was greatly reduced after 21 days, it is inferred that ongoing suppression of B-cell development is not dependent on the continued presence of murine immunoglobulin. The enduring nature of suppression could be attributable to a limited period during which differentiation of B cells from stem cells normally occurs, although further studies will be needed to investigate this and other possible explanations for the effect of anti-IgM treatment on prenatal B-cell development in sheep.