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Thrombocytopenia (defined as a platelet count <150×10(9)) is a well-known complication in patients with liver cirrhosis and has been observed in 76% to 85% of patients. Significant thrombocytopenia (platelet count <50×10(9) to 75×10(9)) occurs in approximately 13% of patients with cirrhosis. Thrombocytopenia can negatively impact the care of patients with severe liver disease by potentially interfering with diagnostic and therapeutic procedures. Multiple factors can contribute to the development of thrombocytopenia including splenic platelet sequestration, immunological processes, bone marrow suppression by chronic viral infection, and reduced levels or activity of the hematopoietic growth factor thrombopoietin. The present review focuses on the etiologies and management options for severe thrombocytopenia in the setting of advanced liver disease.

Objective: To develop effective and safe measures of postoperative multimodal analgesia (optimizing the use of narcotic analgesics) in surgical treatment of hematological patients with thrombocytopenia., Design: A pilot and prospective comparative controlled study., Methods: We studied 27 patients with acquired thrombocytopenia who underwent laparoscopic splenectomy. Three schemes of anesthesia were used: 1) Nefopam 20 mg/day + trimeperidin (n = 7); 2) Paracetamol 4 g/day + trimeperidin (n = 10); 3) paracetamol + trimeperidin + glucocorticosteroids (GC) (through treatment of the underlying disease) (n = 10). Analgesic properties and effect of each scheme were assessed according to a rating scale of pain NRS (10 points) and VAS (100 points) studied. Additionally we evaluated the effect of nefopam and paracetamol on the functional properties of platelets and hemostasis, platelet levels while monitoring and indicators of thromboelastogram (TEG)., Results: Application of paracetamol + trimeperidin accompanied with effectively reducing of postoperative pain (less than 5 points on the NRS). The level of postoperative pain was lower in patients who were treated with corticosteroids within the therapy of the underlying disease before surgery and who continued to receive it in postoperative period (2-3 points NRS). Nefopam use in the perioperative period is not only inferior to the analgesic effect of paracetamol, but also causes frequent side effects. Consumption of narcotic analgesic--trimeperidin when applying nefopam averaged 43 mg/day, the appointment of paracetamol--28 mg/day, using a combination of paracetamol + GC--20 mg/day. Thus, GC within the underlying disease treatment substantially reduces the need for opioid analgesics. A monitoring of the number of platelets and TEG did not shows negative effect of paracetamol and nefopam on platelet and plasma hemostasis., Conclusions: Nefopam and paracetamol may be used in patients with thrombocytopenia, as do not affect the hemostasis and platelet count. Application of postoperative analgesia scheme paracetamol + trimeperidin in patients receiving corticosteroids provides the maximum reduction of pain with the least consumption of narcotic analgesics.

Thrombocytopenia is a major risk factor for cirrhotic liver disease. Patients with thrombocytopenia may have esophageal or gastric varices secondary to portal hypertension, leading to variceal bleeding which exposes the liver to further damage. Here, we present a female pediatric patient with PSC and CD, whose progressive thrombocytopenia was successfully controlled by romiplostim, a TPO receptor agonist. The patient developed bloody diarrhea at four yr of age, and was subsequently diagnosed with PSC and CD when seven yr old. While CD was well-controlled by immunomodulators, the patient's thrombocytopenia gradually progressed resulting in petechiae (platelet count of 11 × 10(9) /L) when she was 10 yr and four months old. She responded poorly to immunoglobulin and corticosteroids. Weekly subcutaneous injection of romiplostim was therefore initiated, and platelet counts were maintained over at 50 × 10(9) /L. She was able to undergo successful LDLT without platelet transfusion seven months after the initiation of romiplostim. Romiplostim was not required after LDLT with improved platelet counts. This case report suggests that romiplostim may be effective in the treatment of thrombocytopenic children with liver cirrhosis and portal hypertension, and in eliminating the need for platelet transfusion during the peri-transplant period., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

We designed the GammaKey system for the acquisition, storage and analysis of images from semi-analogue gamma scintillation cameras (GSCs). The GammaKey system, operating on a standard PC, replicates the functionality of earlier dedicated computer systems, allows the exchange of data in the DICOM format and has an open architecture enabling the development of new diagnostic techniques. The main purpose of the GammaKey is to enable the continued use of old GSCs which have functional scintillation crystals, but also to permit data exchange with new digital GSCs. The GammaKey has been technically validated by standards established by the National Electrical Manufacturers Association. The GammaKey has been used for seven years in two leading centres for nuclear medicine in Serbia (the Clinical Center of Serbia, Belgrade, and the Clinical Center of Vojvodina, Novi Sad) in approximately 30,000 patients. Clinical application proves that the GammaKey is a robust and reliable system with high-quality image output. Data processing can be upgraded with non-standard features added on request as shown in two examples: (1) the testing of splenectomy efficacy in the case of thrombocytopenia with normal production; and (2) the detection and localisation of parathyroid adenomas., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Background: Laparoscopic splenectomy (LS) is considered the standard treatment for patients with refractory primary immune thrombocytopenia (ITP)., Patients and Methods: All patients with ITP who underwent LS during the last 17 years and who had a minimum follow-up of 1 year were included. Several perioperative variables such as age, platelet count, and duration of preoperative therapy were recorded and analyzed, looking for potential predictive variables of clinical response., Results: In total, 150 patients were included: 108 (72%) women and 42 (28%) men, with a mean age of 37.3±15.8 years. In the evaluation 1 year after surgery, 133 (88.7%) patients had achieved complete response, 4 (2.7%) had a response, and in 13 (8.6%) there was no response. None of the analyzed preoperative variables was identified as a predictive factor of response at 1 year. Immediate responders after surgery (≥150,000 platelets/mL during the first week) had a higher platelet count and rate of complete response at 1 year (94.2%)., Conclusions: LS has a high success rate in patients with refractory ITP. Potential predictive indicators of success remain to be determined.

A 6-year-old girl presented with presumed relapse of childhood immune thrombocytopenia. Investigations revealed deranged coagulation parameters, abnormal small bowel thickening, and splenomegaly. A clinically significant bleeding diathesis emerged which was refractory to most hemostatic interventions. Laparatomy revealed a composite diagnosis of splenic hemangiomatosis and small bowel lymphangiomatosis. Splenectomy resulted in complete resolution of the coagulopathy. The diagnosis and management of these conditions is inherently complex and without clear guidance. We discuss our perioperative management of the bleeding diathesis. There is a need for long-term follow-up of the underlying pathologies particularly as potentially useful therapeutic agents have emerged.

Background: MYH9-related disease (MYH9-RD) is a rare syndromic disorder deriving from mutations in MYH9, the gene for the heavy chain of non-muscle myosin IIA. Patients present with congenital thrombocytopenia and giant platelets and have a variable risk of developing sensorineural deafness, kidney damage, presenile cataract, and liver abnormalities. Almost all MYH9-RD patients develop the hearing defect, which, in many individuals, progresses to severe to profound deafness with high impact on quality of life. These patients are potential candidates for cochlear implantation (CI), however, no consistent data are available about the risk to benefit ratio of CI in MYH9-RD. The only reported patient who received CI experienced perisurgery complications that have been attributed to concurrent platelet defects and/or MYH9 protein dysfunction., Methods: By international co-operative study, we report the clinical outcome of 10 patients with MYH9-RD and severe to profound deafness who received a CI at 8 institutions., Results: Nine patients benefited from CI: in particular, eight of them obtained excellent performances with restoration of a practically normal hearing function and verbal communication abilities. One patient had a slightly worse performance that could be explained by the very long duration of severe deafness before CI. Finally, one patient did not significantly benefit from CI. No adverse events attributable to MYH9-RD syndrome were observed, in particular no perisurgery bleeding complications due to the platelet defects were seen. Patients' perioperative management is described and discussed., Conclusions: CI is safe and effective in most patients with MYH9-RD and severe to profound deafness and should be offered to these subjects, possibly as soon as they develop the criteria for candidacy.

Secondary immune thrombocytopenia is a rare paraneoplastic syndrome of lung cancer. We report a case of pulmonary pleomorphic carcinoma with newly diagnosed secondary immune thrombocytopenia. On referral, the patient's complete blood cell count was normal; however, it showed marked thrombocytopenia after 1 month. Blood biochemistry and bone marrow puncture showed normal findings. We speculated that he had immune thrombocytopenia associated with the lung cancer and planned lung resection. Sleeve middle and lower lobectomy was successfully performed with preoperative intravenous immunoglobulin and intraoperative platelet transfusion. His platelet count was restored and maintained a normal level at 8 months after the operation., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Interferon (IFN) therapy is a well-established antiviral treatment for hepatitis C virus (HCV) - infected patients. However, susceptibility to thrombocytopenia is a major obstacle in its initiation or continuation, particularly in patients with HCV who underwent liver transplantation (LT). We previously showed that the coexistence of splenomegaly and thrombocytopenia could result in persistent thrombocytopenia after LT. Here we retrospectively evaluated the validity of this criterion for simultaneous splenectomy in recipients with HCV., Patients and Methods: Subjects included 36 recipients with HCV who received LT between January 2006 and February 2012 at Hiroshima University. We analyzed the spleen volume, body surface area, platelet (PLT) count, and rate of completion or continuation with IFN therapy in these recipients., Result: Of these recipients, 30 did not require simultaneous splenectomy according to the criterion, and 24 actually did not receive simultaneous splenectomy. In this group, 21 (87.5%) started IFN therapy. Fifteen (71.4%) of these recipients completed or continued IFN therapy, whereas 13 (61.9%) achieved either a sustained virological response (SVR) or an end-of-treatment response. The PLT count increased to >100,000/mm(3) 1 month after LT in 16 (66.7%) recipients from this group., Conclusion: Our criterion detected the PLT count outcome after LT in recipients with HCV and achieved a better SVR result after IFN therapy., (Copyright © 2014 Elsevier Inc. All rights reserved.)

The precise mechanism of prolonged thrombocytopenia following living donor liver transplantation (LDLT) remains unclear. To determine risk factors associated with prolonged thrombocytopenia following LDLT, with a focus on the activity of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs member 13) and the influence of splenectomy. Adult LDLT patients were divided into two groups on the basis of platelet counts (100 × 10(3)/μL) on POD 14: high and low platelet (HP and LP) groups. Survival analysis was performed in the 100 patients, and ADAMTS13 activity and von Willebrand factor (VWF) levels in the plasma were measured in 65 adult recipients. The 6-month survival rate was significantly lower in the LP group (n = 36) than in the HP group (n = 62) (61.1 vs. 93.5 %). ADAMTS13 activity had been significantly lower in the LP group (n = 23) than in the HP group (n = 42). The VWF/ADAMTS13 ratio was significantly higher in the LP group than in the HP group. The independent risk factors for thrombocytopenia on POD14 were preoperative AT levels and ADAMTS13 activity on POD14. TPO levels on POD14 were significantly higher in the LP group than in the HP group, while those on POD28 in the LP group were significantly decreased, despite the low platelet levels. Irrespective of splenectomy, platelet counts and ADAMTS13 activity in the LP group remained low until POD28, while VWF/ADAMTS13 ratio significantly increased until POD28. These results suggest that prolonged thrombocytopenia after LDLT was associated with not only a decrease in ADAMTS13 due to sinusoidal endothelial cell injury, but also low TPO production due to hepatocyte dysfunction, irrespective of splenectomy [corrected].

MYH9-related disorders (MYH9 RD) are genetic disorders by the variation of MYH9 gene that encodes for the nonmuscle myosin heavy chain IIA. The clinical and laboratory findings of Fechtner syndrome, an MYH9 RD, are macrothrombocytopenia, basophilic cytoplasmic inclusion bodies in leukocytes, glomerulopathy, sensorineural deafness, and cataracts. Fechtner syndrome is a rare cause of chronic kidney disease. To our knowledge, this is first report of successful renal transplant in MYH9 RD in Korea. We report the two cases with a brief review of literatures since we experienced successful living donor kidney transplantation in Fechtner syndrome with end-stage renal disease, showing very serious thrombocytopenia due to MYH9 mutation., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Argatroban was used as the anticoagulant during cardiopulmonary bypass (CPB) in a patient with heparin-induced thrombocytopenia (HIT) type II undergoing mitral valve replacement. Dosage was reduced because of preoperative congestive liver disorder. Perioperative coagulability was poor, and, ultimately, failure of hemostasis led to a fatal outcome. Although argatroban use as an anticoagulant for HIT is reported, the optimal dose has not been established. During long-term CPB, increasing the total dosage may extend anticoagulant ability, leading to dose dependence. Because no antagonist for argatroban exists, failure of hemostasis might occur.

Untreated symptomatic high-grade aortic stenosis remains a lethal disease. Therefore, a comprehensive evaluation is necessary to obtain the best individual treatment for each patient. Recently, transcatheter aortic valve implantation (TAVI) was developed as an innovative therapy for high-risk and inoperable patients. Persistent thrombocytopenia is an established risk for conventional open heart surgery, but is not covered by traditional surgical risk scores. The aim of the study was the investigation of safety and feasibility of TAVI in patients with severe thrombocytopenia. Because of the complicated outcome of patients with persistent thrombocytopenia undergoing heart surgery, we considered all patients with high-grade aortic stenosis and a thrombocyte count of less than 100 per nl as surgical high-risk patients. Out of these high-risk surgical patients, six patients with symptomatic high-grade aortic stenosis and severe thrombocytopenia were deemed to be TAVI candidates and underwent TAVI procedures in 2010 and 2011 (transfemoral: n = 4; transapical: n = 2) at the University Hospital of Frankfurt. The outcome of these patients was analyzed prospectively in order to document safety and feasibility of TAVI in such patients. All TAVI procedures were performed successfully with excellent functional results. There was no occurrence of major or minor bleeding complications, acute renal failure or nosocomial infection. One patient died of an ischemic stroke 12 days after the procedure. The five remaining patients were alive at the 12-month follow-up without relevant cardiovascular events and excellent valve performance. TAVI is an effective and well tolerated method to treat patients with chronic persistent thrombocytopenia and symptomatic high-grade aortic stenosis, and therefore a reasonable alternative to conventional heart surgery in such patients. The indication for TAVI in patients with thrombocytopenia and symptomatic high-grade aortic stenosis might be generated independently from conventional scoring systems.

In this report, we describe the first successful case of microvascular free tissue transfer in a patient with Evans Syndrome (ES), a rare form of idiopathic thrombocytopenic purpura (ITP) and associated autoimmune hemolytic anemia (AIHA). Microvascular surgery in the setting of ES is likely to have higher complication rates because of the increased risk of postoperative bleeding and free flap thrombosis. The case presented here opens up to the feasibility of microvascular reconstruction of patients with coagulation disorders like ES. Every effort should be made to control for hemolytic, thrombocytopenic, and thrombophilic states associated with ES. In the absence of evidence-based treatment guidelines for ES, personalized treatment protocols with high-dose corticosteroids, immunoglobulin, and postoperative anticoagulation regimen are highly recommended., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Heparin-induced thrombocytopenia (HIT) is a rare complication of heparin treatment resulting in a severe acquired thrombophilic condition with an associated mortality of about 10 %. We report the first case of successful urgent liver transplantation (LT) in a patient with end-stage liver disease due to a Budd-Chiari syndrome, portal vein thrombosis and pulmonary embolism due to acquired thrombophilia associated to polycythemia vera carrying JAK2V617F gene mutation and HIT in the acute phase. Lepirudin was used to provide anticoagulation in the LT perioperative period that was performed without haemorrhagic and thrombotic complications despite the donor received heparin during liver explantation.

Background/aims: The management of hepatocellular carcinoma with hypersplenic thrombocytopenia remains controversial, because of the high surgical morbidity and mortality. Splenectomy has been reported to be useful for patients with hypersplenic thrombocytopenia, but the timing and route of splenectomy and hepatic resection remains unclear. This report evaluated the efficacy of laparoscopic surgery for simultaneous splenectomy and hepatic resection in the treatment of hepatocellular carcinoma with hypersplenic thrombocytopenia., Methodology: Among 65 patients with hypersplenic thrombocytopenia who underwent hepatic resection for hepatocellular carcinoma, 20 patients underwent simultaneous splenectomy and hepatic resection. Initially, ten patients underwent the procedure by conventional laparotomy (open group) and ten patients underwent by laparoscopy (laparoscopic group). The clinicopathological results from the two groups were compared retrospectively., Results: The blood loss in the laparoscopic group was significantly less and the postoperative morbidity rate in the laparoscopic group was lower than that in the open group. The duration of the postoperative hospital stay in the laparoscopic group was significantly shorter and rate of patients administered interferon in the laparoscopic group was significantly higher than that in open group., Conclusions: Simultaneous splenectomy and hepatic resection under laparoscopy is a safe and useful in the treatment of hepatocellular carcinoma with hypersplenic thrombocytopenia.

The pathogenesis of renal involvement in Wiskott-Aldrich syndrome (WAS) is unclear and renal outcome is generally poor in such situations. Here we present the case of an 8-year-old boy with WAS who developed hematuria, proteinuria, and declining renal function that did not improve with the combined use of immunosuppressive agents and angiotensin-converting-enzyme inhibitor. Renal pathology revealed IgA nephropathy (IgAN). The patient underwent splenectomy for refractory thrombocytopenia. The proteinuria remitted and renal function improved after splenectomy, long-term antibiotic prophylaxis, and tapering of immunosuppressive agents.

Background: Although laparoscopic splenectomy (Lap-Spleen) has become the standard surgical approach for normal-sized spleens, open splenectomy (Open-Spleen) is still recommended by many in the setting of splenomegaly. We set out to compare the impact of spleen size on Lap-Spleen and Open-Spleen outcomes using a national database., Study Design: We reviewed the American College of Surgeons' National Surgical Quality Improvement Program database to identify patients who had undergone non-emergency splenectomy during 2005-2010. To evaluate the impact of spleen size on outcomes, we considered patients with diagnoses of splenomegaly and hypersplenism as those having large spleens (Large-Sp group) and those with diagnoses of primary thrombocytopenia and immune thrombocytopenic purpura as having normal spleens (Normal-Sp group). Patients were also categorized based on surgical approach into Lap-Spleen and Open-Spleen groups., Results: We identified 639 patients in the Large-Sp group and 879 patients in the Normal-Sp group. During 2005-2010 laparoscopy was used in 84.2% of cases in the Normal-Sp group (annual range, 77.8%-90.8%). However, the rate of laparoscopy in the Large-Sp group remained consistently below 50% with an average of 41.8% (annual range, 20%-47%). In the Lap-Spleen group, those with Large-Sp had longer operative time and length of stay and higher blood transfusion and morbidity compared with the Normal-Sp group. However, when looking specifically at the Large-Sp group, patients with Open-Spleen had more transfusion requirements, longer length of stay, and higher morbidity, compared with those with Lap-Spleen., Conclusions: Lap-Spleen leads to significant improvement in outcomes. These advantages were believed to be limited to normal-sized spleens, but this study demonstrates that laparoscopy can still be advantageous in patients with splenomegaly. We hope such data encourages wider utilization of laparoscopy in the setting of splenomegaly, especially among surgeons who are experienced with the technique.

Antibodies directed against platelet-surface antigen cause immune thrombocytopenia. Transplantation from a donor with immune thrombocytopenia has rarely been reported in the literature and never with a platelet count of 1 × 10(9)/L. We report one liver transplant recipient and one kidney transplant recipient who received organs from a donor with immune thrombocytopenia dying from intracranial hemorrhage. The kidney recipient showed no evidence of thrombocytopenia after transplantation. However, in the liver recipient, the platelet count nadired at 4 × 10(9)/L and normalized within 3 months. Transplantation of a liver from a donor suffering from immune thrombocytopenia must be considered with great caution. Other organs are suitable for transplantation; however, recipients of these organs must be followed carefully for evidence of immune thrombocytopenia and treatment offered accordingly., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: Although splenectomy plays an important role in the management of patients with liver cirrhosis, the optimal technique, open surgery, total laparoscopic surgery or hand-assisted laparoscopic surgery (HALS), has not yet been defined. The present study evaluated the outcomes of HALS splenectomy for cirrhotic patients., Methods: A total of 28 consecutive patients with cirrhosis that underwent HALS splenectomy were enrolled into this study. The preoperative laboratory and morphometric data, intraoperative variables and postoperative outcomes were reviewed from the hospital charts., Results: The postoperative platelet count was remarkably elevated in all cases. A re-operation was required in 1 patient complicated with postoperative hemorrhage. Enhanced CT on POD 7 revealed a high incidence of portal or splenic vein thrombosis (PSVT; 22 patients, 78.6 %). PSVT was significantly associated with higher serum bilirubin, higher indocyanine green retention value at 15 min (ICG R-15), and larger splenic vein diameter., Conclusion: HALS splenectomy was a very feasible and appropriate procedure for cirrhotic patients with hypersplenism. PSVT was a frequent complication and large splenic vein diameter, high serum bilirubin, and high ICG R-15 were found to be significant risk factors for PSVT after HALS splenectomy in cirrhotic patients.

This study aims to investigate the efficacy and safety of laparoscopic splenectomy (LS) in the management of refractory thrombocytopenia associated with systemic lupus erythematosus (SLE). From January 2003 to February 2012, 20 patients underwent splenectomy for thrombocytopenia associated with SLE. Of these, 11 underwent open (SLE-OS group) and 9 underwent laparoscopic splenectomy (SLE-LS group). Another 15 patients with ITP underwent LS (ITP-LS group) were categorized as the control group. Surgical indications, perioperative details, and short- (90 days) and long- (median, 42 months) term hematological outcomes were assessed. Splenectomy was successful in all 20 SLE patients. The mean platelet count increased from 23 × 10(9)/L before splenectomy to 289.2 × 10(9)/L and 144.2 × 10(9)/L after 3 and 6 months, respectively, and was 115.5 × 10(9)/L at the last visit, with a 3-month complete response (CR) rate of 100 %. After a median follow-up of 42 months, 17 patients (85 %) had a CR or partial response (PR) to splenectomy plus medical therapy. SLEDAI score and dosage of steroids decreased significantly after splenectomy. None of these patients experienced any postoperative infection, bleeding, or thrombotic events. SLE-LS group had lower volumes of estimated blood loss and postoperative drainage and shorter postoperative hospital stay than SLE-OS group. There were no statistically significant differences between the SLE-LS and ITP-LS groups in operation time, estimated blood loss, and postoperative hospital stay. Splenectomy is effective and safe in the management of refractory thrombocytopenia secondary to SLE. LS may be safe and effective in thrombocytopenia associated with SLE.

Immune thrombocytopenia (ITP) is a rare disorder. Evidence-based guidelines provide important information for hematologists, as well as diagnostic and therapeutic recommendations to other physicians with limited expertise in the field. However, guidelines in pediatric and adult ITP do not answer some imperative questions: which patient is at risk of severe bleeding and requires pharmacologic treatment? Who will recover spontaneously? Is splenectomy still an appropriate second-line treatment for all chronic or persistent ITP patients? This review summarizes the current approach to these important issues, the patients' perspective, and how we can improve individual patient management., (Copyright © 2013 Elsevier Inc. All rights reserved.)

In the field of emerging innovative therapies, such as thrombopoietin mimetics, the question of who needs splenectomy remains highly relevant. Removal of the spleen is an accepted and potentially curative treatment of immune thrombocytopenia (ITP) after decades with a favorable economical-effect ratio but with relevant morbidity particularly in the young patients. ITP is rare and splenectomy is performed in a minority of children, which makes its research almost impossible, resulting in a poor standardization of the procedure. Hence, in children, recommendation and decision for splenectomy is individually based and rests on expert opinions. Furthermore, local practice and availability of health products affect the frequency of splenectomy. Current guidelines agree on one point: splenectomy should be postponed for at least 12 months after the initial diagnosis of ITP, due to the high probability of improvement or even spontaneous remission. However, evidence-based data are lacking and splenectomy remains controversial. This article reviews the current literature and delineates controversies and complexities of splenectomy in children with ITP. There is an urgent need for consensus of this procedure in pediatric patients., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Splenectomy may be a treatment option in hepatocellular carcinoma (HCC) and cirrhosis when there is no potential donor for liver transplantation. We retrospectively investigated the long-term outcome of splenectomy on survival in advanced cirrhotic patients with HCC and thrombocytopenia. Between 1999 and 2009, 46 cirrhotic patients with thrombocytopenia (Child-Pugh class B or C) who underwent splenectomy for the simultaneous or secondary treatment of HCC at our institute were evaluated. The 1-, 3-, and 5-year survival rates were 93.5, 76.0, and 37.9%, respectively. Splenectomy resulted in a significant reduction in mean portal venous pressure from 21.2 to 16.8 mmHg and improvements in liver function tests such as total bilirubin, prothrombin time, platelet count, Child-Pugh score for 3 years, and albumin for 2 years. The mean frequency of treatment for HCC recurrence after surgery was 3.0 times (range 1-11). Seven patients out of 16 scheduled for Interferon (IFN) therapy after surgery achieved a sustained virological response (SVR). Multivariate analysis identified SVR after IFN therapy as an independent significant prognostic factor (Hazard ratio 0.18, 95%CI 0.03-0.65, P=0.006). Postoperative complications including liver failure (n=1), portal thrombosis (n=7), ascites (n=5), and bacterial infections (n=4) were observed in 14 patients (30%). Splenectomy can be a feasible supportive therapy for the continuation of anticancer therapy and completion of IFN therapy based on improvements in liver function and thrombocytopenia with minimum complications in patients with HCC and advanced cirrhosis with no potential donor.

Thrombocytopenia can be a real challenge during cardiac surgery in children with cyanotic congenital heart disease. This report describes a 7-year-old girl with d-transposition of the great arteries, ventricular septal defect, pulmonary hypertension, chronic thrombocytopenia, polycythemia, and chronic renal impairment. The thrombocytopenia improved several days after splenectomy. The child then underwent a successful arterial switch operation with ventricular septal defect closure.

Background/aim: Hypersplenism due to splenic congestion is observed in portal hypertensive patients. This study was done to know the change in platelets count following early ligation of splenic artery during splenectomy in patients with thrombocytopenia due to portal hypertension with a hypothesis that splenic decongestion results in increased platelets count; thereby platelet transfusion can be avoided., Materials and Methods: Patients with platelets count <100,000 per mm(3) due to portal hypertension were involved and we followed a protocol of ligating splenic artery first, followed by 30 minutes waiting period for splenic decongestion. Blood sample was collected at 5 and 30 minutes for the estimation of platelets count., Results: Significant rise in platelets was observed after 5 and 30 minutes of early ligation of splenic artery with mean rise being 23735 ± 15417 and 35085 ± 20458 per mm(3), respectively. The rise in platelets at 30 minutes was significant when compared with 5 minutes rise with mean platelets count being 91661 and 103070 per mm(3) at 5 and 30 minutes, respectively. The platelets rise was equal to 4 and 6 units of platelets concentrates, respectively., Conclusion: Early ligation of splenic artery during splenectomy for portal hypertension results in significant rise in platelets after 5 and 30 minutes. This method conserves platelets and avoids platelets transfusion and its complications.

This multicenter, randomized trial assessed the efficacy and safety of a recombinant human thrombopoietin (rhTPO) in patients with persistent primary immune thrombocytopenia (ITP) who had failed glucocorticosteroid treatment. A total of 140 eligible patients were randomized to receive rhTPO + danazol (rhTPO group, 73 patients) or danazol (control group, 67 patients) alone. During the first phase, the increase in the mean maximal platelet counts (101.2 × 10(9)/L) and the area under curve (749.6) in the rhTPO group were significantly higher compared to control (33.3 × 10(9)/L and 316.2; P = 0.0060 and 0.0000, respectively). The major response rate (MRR) and total response rate (TRR) in the rhTPO group were 38.4 and 60.3 %, respectively, significantly higher than in control (MRR 7.9 %, P = 0.0003; TRR 36.5 %, P = 0.0104). In the control group, 45 patients with platelet counts <20 × 10(9)/L were given rhTPO during the second phase and achieved MRR 31.1 % and TRR 66.7 %. The mean platelet counts in the rhTPO group were still approximately 50 × 10(9)/L on day 28 of the study. The overall incidence of rhTPO-related adverse events was 13.6 %. All the adverse events were generally mild. This study demonstrated that rhTPO was well tolerated, and it markedly increased platelet counts in chronic ITP patients. Stimulation of platelet production by rhTPO may provide a new therapeutic option for patients with ITP.

Context: Hematological manifestations are less known in primary hyperparathyroidism (PHPT). Anemia has been recognized as a complication of PHPT in 5-30% of the individuals in various series., Objective: Our objective was to identify hematological improvement after the curative parathyroidectomy in a PHPT patient with anemia and thrombocytopenia., Patient and Methods: A 30-yr-old female presented with recurrent renal stone disease, anemia, and thrombocytopenia diagnosed as PHPT. Bone marrow examination revealed megakaryocytic thrombocytopenia. Two months after the curative parathyroidectomy, her symptoms and hematological parameters were improved., Conclusion: Our finding showed a causal association of both anemia and thrombocytopenia with marrow fibrosis in a symptomatic PHPT patient because both anemia and thrombocytopenia improved after the curative parathyroidectomy.

Background: Posttransplant cytopenias are a severe complication after allogeneic stem cell transplantation (allo-SCT) and their origin is often multifactorial or unknown. They are frequently refractory to standard therapy, which may include steroids and/or immunoglobulins. Mesenchymal stem cells (MSCs) are an attractive therapeutic tool in the allo-SCT setting for the ability to enhance engraftment as well as acting as immunosuppressants for graft-versus-host disease. There is no prior experience in the literature of the use of MSCs to treat cytopenias after allo-SCT., Case Reports: In this work we report for the first time four cases of refractory posttransplant cytopenias (three patients with thrombocytopenia and one with neutropenia) that were treated with MSCs from a third-party donor. MSCs were expanded from 100 mL of marrow obtained under standard good manufacturing practice conditions. Most patients received more than one cell dose, and median dose of MSCs administered was 1 × 10(6) /kg., Results: All patients recovered normal blood counts, with a mean follow-up of 12.5 months. There were no adverse events related to MSC administration., Conclusion: MSC therapy may contribute to the recovery of refractory posttransplant peripheral cytopenias in patients undergoing allo-SCT., (© 2011 American Association of Blood Banks.)

Blood-free pediatric surgery is increasingly used for surgical correction of simple cardiopathies. Herein, we describe a complex cardiopathy, arterial switch operation and ventricular septal defect, with pre-operative thrombocytopenia in a 4.5 Kg baby treated with warm surgery and intermittent warm blood microplegia without any blood product. Bypass time was 89 min and aortic cross-clamp time 61 min. The maximal length of warm ischemia (time between microplegia injections) was 42 minutes. The postoperative course was uneventful. The patient was weaned off the ventilator after 7 hours, was discharged from the ICU on day 2 and was discharged from the hospital on day 7. The two main factors involved in this result were high pre-operative hemoglobin level and bypass technique with small prime volume, microplegia and warm perfusion. However, the success of this challenging case is also the result of teamwork and of rigorous patient care.

Introduction: Laparoscopic splenectomy using pneumoperitoneum has been performed since 1992. The gasless abdominal wall-lifting method for laparoscopic splenectomy was introduced as an alternative. This retrospective study was undertaken to compare results using the two techniques., Methods: Between 1995 and 2010, 54 patients underwent laparoscopic splenectomy at a single institution; 30 underwent the procedure using the gasless technique and 24 using pneumoperitoneum. There were no significant differences between the two groups regarding age, sex or BMI, but more patients underwent concurrent operations in the pneumoperitoneum group. The abdominal wall-lift system with subcutaneous K-wires was used for the gasless method., Results: Intraoperative blood loss was similar in the two groups (193.0 ± 196.7 mL gasless, 217.3 ± 296.6 mL pneumoperitoneum; P > 0.05), but operative time (182.1 ± 92.1 min, 135.1 ± 46.1 min; P < 0.05), and resected spleen weight (306.1 ± 297.7 g, 138 ± 81.0 g; P < 0.05) were significantly different. In the gasless group, additional procedures included conversion (n = 1), mini-laparotomy (n = 2), and CO(2) insufflation (n = 2). Excluding the concurrent living-related kidney donor patients, hospital stay was similar (6.9 ± 2.5 days, 6.3 ± 2.0 days, P > 0.05)., Conclusion: Although gasless laparoscopic splenectomy is feasible, there are disadvantages, particularly the restricted operative working space in some patients. These results suggest that either technique may be used on an individual basis in patients undergoing laparoscopic splenectomy., (© 2011 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and Blackwell Publishing Asia Pty Ltd.)

Aim: The efficacy of argatroban as an anticoagulant in patients with heparin-induced thrombocytopenia type II (HIT) was shown in two large trials (ARG 911 and ARG 915) using a composite endpoint of death, amputation and new thrombosis, but no benefit was seen in amputation rate. Factors that influenced patients proceeding to amputation are unclear., Methods: We retrospectively undertook exploratory data analyses comparing patient- and treatment-related factors in patients with (N.=48) and without (N.=520) all-cause amputation., Results: Of the amputation cases, 46% had received warfarin, and 97.9% had severe ischemia/gangrene diagnosed pre-argatroban. Thromboembolic complications prior to argatroban were more common in amputation cases (91.7% vs. 71.9%, P=0.003), with a higher proportion of arterial than venous events (68.2% vs. 52.3%, P=0.031; mean 1.7/patient vs. 1.4/patient, P=0.031), largely occurring in the limbs (94.1%). More females than males suffered amputation (P=0.003), and cardiovascular risk was more frequent in amputation cases than non-amputation (hypertension, P=0.008; peripheral vascular disease (PVD), P<0.001; diabetes, P<0.001). There were no differences in baseline platelet count, platelet recovery (24 h post-argatroban), or weighted mean aPTT between groups; amputation was associated with longer treatment duration (5 vs. 7 days, P=0.001)., Conclusion: Almost all amputation cases had severe ischemia prior to argatroban; female gender and cardiovascular risk factors, in particular PVD with peripheral vascular surgery, appear to identify a subgroup of patients at high risk of amputation. The pharmacodynamics of argatroban appeared to be unchanged in the amputation cases suggesting that argatroban action was undiminished in the amputation cases despite the negative outcome of treatment.

Background: Thrombocytopenia is a serious side effect following high-dose chemotherapy or whole-body irradiation. For many patients, a hematopoietic stem cell (HSC) transplant is required as part of the treatment or to restore the integrity of the hematopoietic system. In this article, we first review the origin of thrombocytopenia in the context of umbilical cord blood (UCB) transplantation and current cellular therapies developed to overcome this condition. Results obtained in recent clinical trials and in the laboratory using a mouse-based xenograft model were also discussed. Second, we investigate the kinetic of human platelet production in two immunodeficient mouse strains transplanted with UCB cells to determine which of the two is better suited to measure the thrombopoietic potential of human hematopoietic cells., Study Design and Methods: NOD/SCID/IL2Rγ(null) or NOD.CB17-Prkdcscid/NcrCrl (NOD/SCID) mice were transplanted with ex vivo expanded UCB cells. Human platelet levels and marrow engraftments were measured by cytometry analyses., Results: Human platelets appeared earlier and at greater levels in the NOD/SCID/IL2Rγ(null) mouse strain. Consistent with these results and previous reports, human marrow engraftment was also greater in the IL2Rγ(null) -based NOD/SCID mice., Conclusion: The NOD/SCID/IL2Rγ(null) mouse strain is an ideal choice for preclinical studies aimed at measuring the in vivo thrombopoietic potential of human HPCs. Exploitation of such a model should facilitate the development of new cellular therapies aimed at improving hematological recoveries following HSC transplantation., (© 2011 American Association of Blood Banks.)

Unlabelled: Splenectomy is considered the second-line of treatment in patients with chronic primary immune thrombocytopenia (ITP) in whom glucocorticoids have failed. Some patients do not respond to splenectomy or they have postoperative complications. Based on our previous experience using kinetic and scintigraphic parameters, we did a retrospective study with the aim of comparing all these parameters as a means of predicting the success of splenectomy in persistent and chronic primary ITP. Forty-one consecutive patients with chronic primary ITP refractory to prednisone, who had been splenectomized, were included in the study. The response to splenectomy was assessed by evaluating bleeding and platelet counts before and at different times after surgery. A complete platelet kinetic study was performed before the splenectomy using autologous (111) In-labeled platelets. The scintigraphic parameters measured included different indices between spleen/heart, liver/hearth, and spleen/liver. Thirty-six patients gave a complete response after splenectomy and five patients did not respond. A statistically significant difference between both groups was found with initial platelet recovery and with some scintigraphic indices which also showed a variable prediction value for the success of splenectomy. Among these indices, the spleen/liver at 30 minutes demonstrated a predictive value with a 100% of sensitivity and a 100% of specificity., Conclusion: some platelet kinetic parameters and scintigraphic indices, in particular the spleen/liver at 30 minutes, were useful to predict the outcome of splenectomy in persistent and chronic primary ITP and, therefore, they should be taken into account when deciding whether or not to perform a splenectomy., (Copyright © 2011 Wiley-Liss, Inc.)

We present a case of a man diagnosed with systemic lupus erythematosus, associated antiphospholipid syndrome and Evans syndrome, who developed a severe thrombocytopenia refractory to treatment with first-line drugs, cyclophosphamide and rituximab, and who responded to romiplostim with a normalization of the platelet recount, which later enabled a therapeutic splenectomy to be performed.

Congenital amegakaryocytic thrombocytopenia (CAMT, MIM #604498) is a rare inherited bone marrow failure syndrome presenting as isolated hypomegakaryocytic thrombocytopenia at birth without other characteristic physical anomalies. Most of the patients develop a severe aplastic anemia and trilineage cytopenia during the first years of life and hematopoietic stem cell transplantation is the only curative treatment. In most of the cases the disease is caused by homozygous or compound heterozygous mutations in the gene MPL encoding the receptor for the hematopoietic growth factor thrombopoietin. The present review summarizes clinical and laboratory data for 96 patients with CAMT, reported since 1990., (Thieme Medical Publishers.)

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that usually occurs in infants. It is commonly associated with Kasabach-Merritt syndrome and lymphangiomatosis. It generally originates on the skin, later affecting deeper tissue by infiltrative growth. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. However, to our knowledge, a KHE in breast tissue has never previously been reported. Furthermore, the development of this type of tumor in adolescents and adults is very rare. We treated a 32-year-old woman with a right breast mass that was diagnosed based on pathologic examination as a KHE. We performed operations several times because of recurrence of KHE and finally performed mastectomy despite the patient's young age., (Copyright © 2011 Elsevier Inc. All rights reserved.)

We conducted a nationwide cohort study of adult Danish patients with primary chronic immune thrombocytopenia (cITP) to examine selected patient and clinical characteristics as predictors for splenectomy. We analyzed data from the Danish National Patient Registry and patient medical records from 1996 to 2007. Using Cox regression analyses, we calculated incidence rate ratios (IRRs) and associated 95% confidence intervals (CI) for splenectomy. We included 371 adult cITP patients. Of these, 87 patients (23%) underwent a splenectomy during a median of 3.6 years of follow-up. The majority (84%) of cITP patients who underwent splenectomy had splenectomy within the first year after cITP diagnosis. Predictors for splenectomy included age ≤ 75 years (adjusted 1-year IRR = 6.79 (95% CI, 2.10-21.90)) at least one platelet count ≤ 30 × 10(9)/L (i.e., high disease activity; adjusted 1-year IRR = 2.67 (95% CI, 1.37-5.22)) during follow-up and year of cITP diagnosis in early period (1996-2001; adjusted 1 year IRR = 2.37 (95% CI, 1.46-3.85)). Presence of chronic comorbidity was associated with lower rates of splenectomy (adjusted 1 year IRR = 0.58 (95% CI, 0.33-1.05)). Our findings suggest that high disease activity and absence of chronic comorbidity may be associated with higher rates of splenectomy, and that contraindications for splenectomy (i.e., patients' perceived frailty) cause the physicians to use the procedure cautiously.