Managing uncontrolled postsplenectomy reactive thrombocytosis in idiopathic thrombocytopenic purpura: role of thrombocytapheresis.

Reactive thrombocytosis occurs in response to infection, trauma, or surgery. Splenectomy alone accounts for 19% of all possible causes of extreme thrombocytosis. We performed thrombocytapheresis in a young lady with chronic idiopathic thrombocytopenic purpura (ITP) who developed postsplenectomy reactive thrombocytosis. Her post splenectomy platelet count was 227 × 10(6)/ml which elevated to 1623 × 10(6)/ml on the 7th postoperative day. A single thrombocytapheresis procedure reduced her platelet to 403 × 10(6)/ml. She was discharged on the 10th postoperative day and then maintained a count of 204-238 × 10(6)/ml with aspirin. Thrombocytapheresis reduces the platelet count rapidly in thrombocytosis and prevents patients from having thrombotic events. However, such procedures should be performed very meticulously to ensure patient safety.

Bilateral nephrectomy for the treatment of refractory lupus nephritis with features overlapping with thrombotic microangiopathy resembling thrombotic thrombocytopenia purpura.

We report a patient with a diagnosis of systemic lupus erythematosus who concurrently developed a syndrome of thrombotic microangiopathy that resembled thrombotic thrombocytopenic purpura. The patient underwent plasma exchange and immunosuppressive therapy for months before clinical improvement was finally achieved through bilateral nephrectomy. Ultimately, our patient died of disseminated aspergillosis from prolonged immunosuppression. We believe that recognition of bilateral nephrectomy as a potential treatment earlier in her course would have spared her this unfortunate demise. We hope that this review of current literature will help the reader to consider bilateral nephrectomy in patients with refractory systemic lupus erythematosus with clinical overlap of thrombotic microangiopathy resembling thrombotic thrombocytopenic purpura.

Single-access transumbilical laparoscopic splenectomy using curved reusable instruments.

BACKGROUND: The authors report a single-access transumbilical laparoscopic splenectomy (SATLS) performed with curved reusable instruments. METHODS: A 23-year-old female consulted for steroid-resistant idiopathic thrombocytopenic purpura and clinical evidence of secondary Cushing syndrome. Preoperative workup showed a normal-sized spleen and thrombocytopenia. The umbilicus was incised and a purse-string suture was applied. A reusable 11-mm trocar was inserted for a 10-mm, 30° angled scope. Curved reusable instruments (Karl Storz-Endoskope, Tuttlingen, Germany) were advanced without trocars transumbilically. After opening the splenocolic and gastrosplenic ligaments, the main splenic artery and vein were dissected off at the level of the hilum, clipped with 5-mm clips introduced transumbilically, and sectioned. Posterior splenic attachments were freed at the hilum cranially and caudally. The spleen was finally retrieved transumbilically in a plastic bag. RESULTS: Addition of supplementary trocars or incisions was not necessary. Operative time was 180 min and final umbilical scar 16 mm. The patient was discharged on postoperative day 3, and after 6 months she was doing well. CONCLUSIONS: SATLS was feasible and safe to be performed using curved reusable instruments. The curves of the instruments permitted the surgeon to work in an ergonomic position, without the instruments clashing thanks to the obtained triangulation. Since only reusable instruments were used, the cost of SATLS remained similar to that of standard laparoscopy.

Recurrent hypersplenism caused by giant accessory spleen due to portal hypertension after splenectomia.

Splenectomy is one of the primary choices of treatment in immune thrombocytopenic purpura. However, the disease may relapse despite splenectomy. One of the leading causes of relapse is the presence of accessory spleen, which may become enlarged significantly with underlying pathologies such as presence of portal hypertension. The accessory spleen, which will inevitably enlarge in time, may grow significantly within a short period of time in the presence of portal hypertension and may thus be misdiagnosed as a tumoral mass. Presence of ectopic spleen should be borne in mind in patients diagnosed with immune thrombocytopenic purpura with relapsing hypersplenism following splenectomy. This article discusses a patient developing portal hypertension secondary to chronic liver disease and presenting with a significantly enlarged accessory spleen as well as hypersplenism findings.

Laparoscopic accessory splenectomy for recurrent idiopathic thrombocytopenic purpura.

BACKGROUND: Splenectomy has 50% to 70% long-term efficacy for immune thrombocytopenic purpura (ITP). In some patients, relapse is due to the presence of residual accessory splenic tissue. METHODS: A 44-year-old male had ITP since 1983 with splenectomy in 1985. He had a transient response, but then developed severe thrombocytopenia refractory to multiple modalities for 20 years. An accessory spleen was first visualized in 2000. RESULTS: A laparoscopic accessory splenectomy was performed without difficulty. The patient had an initial response with a significant increase in platelet count. Although over time the thrombocytopenia recurred, there has been a long-term benefit in that the patient is on much lower doses of prednisone to maintain an adequate platelet count. CONCLUSION: The finding of accessory splenic tissue after prior splenectomy may be an increasingly common problem in patients with recurrent ITP. Although reported response rates for resection of residual splenic tissue vary, the availability of a safe, less morbid, minimally invasive approach makes the decision to operate easier.

Preoperative embolization of the splenic artery in patients that underwent splenectomy for immune thrombocytopenic purpura.

UNLABELLED: Transfusion of platelets, red blood cells, or both is usually necessary immediately after splenic artery ligature in patients with immune thrombocytopenic purpura who undergo splenectomy. PURPOSE: To investigate whether preoperative embolization of the splenic artery reduced the need for transfusion of platelets, red blood cells, or both. METHODS: Twenty- seven consecutive patients that underwent splenectomy for purpura between October 1999 and March 2006 performed by the same surgical team were enrolled. The first 17 patients did not undergo embolization and were compared with the next 10 patients, who composed the embolization group. RESULTS: The platelet count in the embolization group rose from a mean 7000 u/microl before to 75000 u/microl after the procedure. There was no need for platelet or red blood cell transfusion in the embolization group; in the group without preoperative embolization, 11 patients (p=0.001) required platelet transfusion and 8 (p=0.01), red blood cell transfusion. CONCLUSION: Embolization of the splenic artery before splenectomy is a safe method to avoid blood transfusions in patients with ITP.

Predictive factors for successful laparoscopic splenectomy in immune thrombocytopenic purpura: study of clinical and laboratory data.

BACKGROUND: Laparoscopic splenectomy (LS) offers better short-term results than open surgery for the treatment of immune thrombocytopenic purpura (ITP), but long-term follow-up is required to ensure its efficacy. The remission rate after splenectomy ranges from 49 to 86% and the factors that predict a successful response to surgical management have not been clearly defined. The goal of this study was to determine the preoperative factors that predict a successful outcome following LS. METHODS: From February 1993 to December 2003, LS was consecutively performed in a series of 119 nonselected patients diagnosed with ITP (34 men and 85 women; mean age, 41 years), and clinical results were prospectively recorded. Postoperative follow-up was based on clinical records, follow-up data provided by the referring hematologist, and a phone interview with the patient and/or relative. Univariate and multivariate analyses were performed for clinical preoperative variables to identify predictive factors of success following LS. RESULTS: Over a mean period of 33 months, 103 patients (84%) were available for follow-up with a remission rate of 89% (92 patients, 77 with complete remission with platelet count > 150,000). Eleven patients did not respond to surgery (platelet count < 50,000). Mortality during follow-up was 2.5% (two cases not related to hematological pathology and one case without response to splenectomy). Preoperative clinical variables evaluated to identify predictive factors of response to surgery were sex, age, treatment (corticoids alone or associated with Ig or chemotherapy), other immune pathology, duration of disease, and preoperative platelet count. In a subgroup of 52 patients, we also evaluated the type of autoantibodies and corticoid doses required to maintain a platelet count > 50,000. Multivariate analysis showed that none of the variables evaluated could be considered as predictive factors of response to LS due to the high standard error. CONCLUSION: Long-term clinical results show that LS is a safe and effective therapy for ITP. However, a higher number of nonresponders is needed to determine which variables predict response to LS for ITP.

Results of laparoscopic splenectomy for immune thrombocytopenic purpura.

OBJECTIVE: To study the safety and efficacy of Laparoscopic Splenectomy (LS) for Immune Thrombocytopenic Purpura (ITP). MATERIAL AND METHOD: Twenty-five consecutive adult patients with chronic ITP who did not achieve sustained remission or refractory to medical treatment underwent elective LS between March 1995 and July 2005. The perioperative course was documented and the follow up data were recorded. RESULTS: All 25 patients underwent successful LS by a single surgeon. Twenty patients were available to analyze with a median follow-up time of 739 days (range, 18-3,555). The mean age was 29.8 years (range, 15-44) and 17 patients were female. The median preoperative platetlet count was 16,500/microL (range, 2,000-180,000). Accessory Spleens (AS) were removed in three patients (15%). A female patient died 24 days after LS from fungal brain abscesses. Fifteen patients (75%) had platelet count > 100,000/microL at initial response. Thirteen patients (65%) are in Complete Remission (CR) (platelet count > 100,000/microL). The probability of staying in CR after LS was 60.2% by Kaplan-Meier analysis. All failures occurred within 218 days of the operation. The mean age of the patients with CR was 27.2 years (range, 15-43) while the mean age of the nonCR was 34.7 years (range, 21-44). CONCLUSION: LS should be considered as a safe and effective therapy when elective splenectomy is indicated for chronic ITP patients.

Emergency splenectomy in adult idiopathic thrombocytopenic purpura. A report of seven cases.

Seven adult patients with idiopathic thrombocytopenic purpura underwent emergency splenectomy. Six were female and one was a male, aged 16 to 61 years. All of them had a life-threatening episode. Six patients had progressive intracranial bleeding and one had postsurgical intra-abdominal bleeding. All patients were saved by surgery, except one for whom operation was delayed. There was no postoperative bleeding or surgical complication. Immediate splenectomy should be the treatment of choice in any patient with idiopathic thrombocytopenic purpura complicated by life-threatening hemorrhage.

Multiple autoimmune manifestations in a splenectomized subject with HLA-B8.

Idiopathic thrombocytopenic purpura developed in a 31-year-old man, and he underwent a splenectomy because of his failure to respond to steroid therapy. Subsequently, during a six-year follow-up, chronic active hepatitis, Coombs' positive hemolytic anemia, and pulmonary interstitial fibrosis developed. Since such a clustering of autoimmune manifestations in a single subject is unusual, possible contributing factors were sought. We suggest that both the splenectomy and the histocompatibility antigen HLA-B8, which the patient was found to carry, may have contributed to this rare clinical syndrome.

Recurrent thrombocytopenia following idiopathic thrombocytopenic purpura. The importance of platelet-bound IgG in establishing cause.

A 20-year-old man experienced two separate thrombocytopenic illnesses. The first episode represented classic idiopathic thrombocytopenic purpura (ITP) and was associated with elevated platelet-bound IgG values. Adequate control of thrombocytopenia could not be obtained with prednisone therapy, and splenectomy produced a clinical remission. Four weeks after splenectomy, an acute febrile illness typical of cytomegalovirus (CMV) infection developed, and CMV grew grom a sample of the patient's blood. Thrombocytopenia recurred during the CMV infection but was not associated with elevated platelet-bound IgG levels. Since the second episode of thrombocytopenia was associated with normal amounts of platelet-bound IgG, it was not ascribed to relapse of the ITP, and the thrombocytopenia resolved rapidly, without specific therapy. There are various therapeutic implications of an accurate causative diagnosis of thrombocytopenia.

Laparoscopic splenectomy for hematologic disorders: experience with the first fifty patients.

BACKGROUND: Splenectomy is increasingly being performed by various minimal access surgical modalities for select hematologic disorders. METHODS: A retrospective analysis was performed on the first 50 patients on whom laparoscopic splenectomy (LS) was attempted. The data studied included indications for surgery, patient demographics, intraoperative parameters, and patient outcomes. A total laparoscopic approach (TLS) was employed in 38 patients and a hand-assisted technique (HALS) was used in 12 patients with massive splenomegaly. Eight patients had concomitant surgical procedures: 7 patients underwent laparoscopic cholecystectomy and 1 patient received a kidney transplant. The most common indications for LS were idiopathic thrombocytopenic purpura (ITP) (50%) and hereditary spherocytosis (24%). RESULTS: LS was successfully completed in 48 patients (96%). Thirty-four patients (68%) required perioperative blood or platelet transfusions. The mean spleen diameter was 17.1 cm (range, 11.2-28.4 cm) on imaging study and mean intact splenic weight was 1019 gm. The mean operative time was 188 minutes (range, 90-340 minutes) in the TLS group and 171 minutes (range, 120-240 minutes) in the HALS group. The mean intraoperative blood loss was 306 mL (range, 40-640 mL) in the TLS group and 163 mL (range, 100-300 mL) in the HALS group. The mean postoperative hospital stay was 3.2 days (range, 2-5 days). CONCLUSION: TLS is safe and feasible in patients with nonpalpable spleens. A concomitant laparoscopic procedure for treating coexisting abdominal pathology may be performed without additional morbidity. The HALS technique may be preferable in patients with splenomegaly (palpable spleens), as it appears to offer intraoperative advantages for retraction, dissection, hemostasis, and organ retrieval.

Laparoscopic splenectomy: a selected retrospective review.

Previous investigators have suggested that laparoscopic splenectomy should be the procedure of choice for the treatment of benign hematologic disorders unresponsive to medical therapy. To evaluate the safety and utility of laparoscopic splenectomy for a variety of splenic disorders, we reviewed our collective experience at 2 institutions. We studied our 8-year experience by retrospective chart review. Patient demographic data, splenic pathology, intraoperative events, concomitant procedures, and all adverse perioperative events were recorded. A total of 131 patients had laparoscopic splenectomy, and there were 8 conversions to open surgery. Pathology included 63 with idiopathic thrombocytopenic purpura (ITP), 23 malignancies, 12 thrombotic thrombocytopenic purpura (TTP), 10 autoimmune hemolytic anemia (AIHA), and 23 others. Accessory spleens were noted in 21 patients (16%). Concomitant surgical procedures included 12 hepatic biopsies, 4 distal pancreatectomies, 4 cholecystectomies, and 7 others. Mean operative time was 170 minutes. There were 16 major complications in 16 patients and 2 deaths. Median postoperative length of stay was 3 days. Conversions, due mostly to bleeding, are related to splenic pathology and medical comorbidity and are not temporally related to surgical experience (learning curve). The morbidity, mortality, and conversion rates were low. Laparoscopic splenectomy permits an appropriate abdominal exploration and is associated with a short hospital stay. It is the procedure of choice for most indications for splenectomy.

Splenectomy in idiopathic thrombocytopenic purpura: its correlation with the sequestration of autologous indium-111-labeled platelets.

We present a retrospective analysis of 111In-platelet sequestration studies in 111 patients with the clinical diagnosis of idiopathic thrombocytopenic purpura (ITP). Fifty-one of these patients underwent splenectomy, independent of the results of the 111In-platelet studies to determine if these isotopic results could accurately predict a beneficial response to splenectomy. Between January 1984 and June 1990, 111 patients who presented with ITP were subjected to a study of autologous 111In-labeled platelets through autotransfusion. The platelet sequestration site was splenic (81%), mixed (12%), or hepatic (7%). Fifty-one patients with persistent drug-resistant thrombocytopenia underwent splenectomy regardless of the isotopic results: 33 patients beyond 6 mo after diagnosis and 18 with high hemorrhagic risks before this delay. The follow-up median duration was 2.9 yr. Thirty-three of the 38 patients with splenic sequestration showed a normalized platelet count, as opposed to 2 of the 13 with mixed or hepatic sequestration (p < 0.001). In addition, platelet survival extended beyond 8 days in six patients, with no apparent sequestration site. The platelet isotopic study performed with this technique appears to be indicated in ITP: it guides clinicians in their decision to perform splenectomy and relates to a more central mechanism certain thrombocytopenias that are inappropriately categorized as ITP.

Acquired disorder of platelet function associated with autoantibodies against membrane glycoprotein IIb-IIIa complex--1. Glycoprotein analysis.

A patient with idiopathic thrombocytopenic purpura developed after splenectomy a thrombasthenia-like severe haemorrhagic diathesis characterized by a normal or subnormal platelet count, prolonged bleeding time, strongly reduced platelet adhesion to glass and defective platelet aggregation in response to ADP and collagen. In contrast to hereditary thrombasthenia membrane glycoproteins (GP) IIb and IIIa were normally present in the patient's platelets. Immunoelectrophoretic analysis revealed an abnormal behaviour of the patient's GP IIb-IIIa complex. Autoantibodies against GP IIb-IIIa were detected in Triton-extracted washed platelets. Incubation of normal platelets with plasma from the patient resulted in a similar immunoelectrophoretic abnormality of the GP IIb-IIIa complex indicating that bound autoantibodies (IgG) are responsible for the abnormal immunoelectrophoretic behaviour of the patient's GP IIb-IIIa complex. Platelet fibrinogen was severely reduced similar to classical thrombasthenia suggesting that the GP IIb-IIIa complex is involved in platelet fibrinogen storage.

Long-term results of laparoscopic splenectomy for immune thrombocytopenic purpura.

OBJECTIVE: To assess the results of laparoscopic splenectomy as a treatment for immune thrombocytopenic purpura (ITP). MATERIAL AND METHODS: We conducted a retrospective study of all patients who underwent laparoscopic splenectomy for ITP at our institution between August 1992 and May 1997. RESULTS: Of 27 patients who underwent attempted laparoscopic splenectomy for ITP at our institution during the study period, 26 had completion of the procedure without conversion to an open splenectomy. The median postoperative hospital stay was 1.5 days, and no postoperative deaths occurred. In one patient, pancreatitis developed postoperatively. In four patients, splenectomy failed--two initially and two subsequently--and reinstitution of medical therapy was necessary. The other patients have remained free of medication, and 19 patients have platelet counts greater than 100 x 10(9)/L. The 3-year actuarial success rate was 81.5%. Response to corticosteroid therapy preoperatively may be an indicator of success of splenectomy. CONCLUSION: Laparoscopic splenectomy is safe and allows prompt recovery. Long-term response rates are similar to those achieved with open splenectomy.

Thrombocytopenic purpura as the sole manifestation of a recurrence of Hodgkin's disease.

A man with apparently quiescent Hodgkin's disease presented with acute severe isolated thrombocytopenic purpura. Splenectomy revealed macroscopic involvement with Hodgkin's tissue and cured the thrombocytopenia.

Accessory splenectomy for idiopathic thrombocytopenic purpura.

Not all patients who undergo splenectomy for idiopathic thrombocytopenic purpura (ITP) respond to operation. This may be due to the presence of an accessory spleen. This is a report of three patients who underwent successful accessory splenectomy after relapse of their disease. Each patient initially experienced a complete response to splenectomy, but recurrence occurred months to years later. All of the patients had persistent Howell-Jolly bodies on peripheral blood smear, yet two of the patients had complete and sustained remissions of their disease after accessory splenectomy. Intraoperative localization of an accessory spleen may be exceedingly difficult in the patient who has undergone a previous operation. However, localization of accessory splenic tissue is greatly facilitated by use of a sterile isotopic detector probe intraoperatively after the injection of technetium-99m-labeled red blood cells. Given the morbidity and mortality rates of refractory ITP, patients who have a relapse after, or who fail to respond to, splenectomy should be evaluated for the presence of an accessory spleen, even if Howell-Jolly bodies are present on peripheral blood smear.

Splenectomy for HIV-related immune thrombocytopenia: comparison with results of splenectomy for non-HIV immune thrombocytopenic purpura.

OBJECTIVE: To determine the effectiveness and safety of splenectomy for patients with human immunodeficiency virus (HIV)-related immune thrombocytopenia, using the results of splenectomy for patients with non-HIV immune thrombocytopenic purpura as a control group for comparison. DESIGN: Retrospective study. SETTING: Tertiary care university hospital. PATIENTS: Fourteen patients who underwent splenectomy for symptomatic, medically refractory HIV-related immune thrombocytopenia at this hospital from 1988 to 1997. During the same period, 20 patients had splenectomy for treatment of non-HIV immune thrombocytopenic purpura. INTERVENTION: Splenectomy. MAIN OUTCOME MEASURES: Platelet response, need for postsplenectomy medical therapy, progression of HIV disease, and complications. RESULTS: All patients with HIV-related thrombocytopenia had a complete early platelet response to splenectomy, with an elevation of the platelet count to greater than 100X10(9)/L. After a median follow-up of 26.5 months, all but 1 patient had a sustained complete remission with no need for medical therapy for thrombocytopenia. Splenectomy was more effective in the HIV-related thrombocytopenia group than in the non-HIV immune thrombocytopenic purpura group, with significantly higher platelet counts at 1 week and 1 month after splenectomy in the HIV group (t test, P=.02 and P=.009, respectively). There were significantly fewer patients needing medical therapy for thrombocytopenia after splenectomy in the HIV group (chi2 test, P=.02). There were no remarkable short- or long-term complications in the patients with HIV infection, including no overwhelming postsplenectomy infections. Three patients have died, and 2 patients have developed AIDS since operation. CONCLUSIONS: Splenectomy is effective treatment for patients with symptomatic HIV-related thrombocytopenia that is resistant to medical therapy. The effectiveness of this treatment suggests that the predominant mechanism of thrombocytopenia in HIV-infected patients is increased destruction of platelets because of platelet-associated immunoproteins.

Combined coronary revascularization and splenectomy.

Idiopathic thrombocytopenic purpura is rarely associated with coronary artery disease. In this report, we describe the successful surgical management of a patient with idiopathic thrombocytopenic purpura and angina pectoris.