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PubMed2002

Clinical and scientific advances in the Philadelphia-chromosome negative chronic myeloproliferative disorders.

The chronic myeloproliferative disorders are clonal hematopoietic stem cell disorders and include chronic myeloid leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and agnogenic myeloid metaplasia (AMM). These diseases are characterized by clonal expansion of the myeloid compartment, increased marrow angiogenesis, and varying risks for blastic transformation. A clear molecular abnormality exists (t(9;22) leading to the fusion of BCR-Abl) only for CML, which led to effective targeted therapy (STI-571). Since no similar pathogenetic mechanism has been discovered for the t(9;22) negative chronic myeloproliferative disorders, their respective diagnosis is currently based on a variety of rather cumbersome diagnostic criteria. Polycythemia vera is distinguished from reactive erythrocytosis through erythropoietin independent growth of erythroid progenitors in vitro, suppressed levels of endogenous erythropoietin, possible overexpression of PRV-1 (polycythemia rubra vera-1), decreased c-Mpl expression on megakaryocytes, as well as overexpression of bcl-xL, and potentially aberrant activity of the Jak-Stat pathway. ET is defined by thrombocytosis and is distinguished from reactive states by decreased megakaryocyte c-Mpl expression, and a propensity for thrombosis. AMM has been associated with a variety of observations including increased concentrations of pro-fibrotic cytokines, increased angiogenesis, and myeloid expansion. AMM is often indistinguishable clinically and prognostically from the advanced phases of other CMPD (specifically post-polycythemic and post-thrombocythemia myeloid metaplasia), all of which are subentities of a diagnosis of myelofibrosis with myeloid metaplasia (MMM). The management of CMPD patients is quite varied given the broad range of disease severity and survival observed. The role of stem cell transplantation is limited by the age and comorbidities encountered in CMPD patients. Since no broadly applicable therapy effects the mortality of the CMPD, management currently focuses on the prevention/palliation of disease morbidity (i.e. vascular complications, pruritus, organomegaly, constitutional symptoms). Palliative strategies which currently focus on non-specific myelosuppresion, will hopefully be soon replaced by targeted therapies as insight into pathogenetic mechanisms of these diseases evolves.

Chronic Disease↗
PubMed2002

Basic sciences of the myeloproliferative diseases: pathogenic mechanisms of ET and PV.

The molecular pathogenesis of ET and PV is unknown, although the relatively indolent clinical course observed in most patients suggests that the defect may be subtle and difficult to establish. Clonality analysis using X-chromosome inactivation patterns in females on purified CD34+ cells have confirmed that a defect is present in the hematopoietic stem cell. However, at least in ET, a significant proportion of patients have polyclonal hemopoiesis, and this presumably reflects the heterogeneous nature of the disorder(s). Attention has focussed on the potential disruption of the physiological regulators EPO and TPO and their respective receptors. In familial disorders, pathological mutations have been identified in some, but by no means all, cases: EPO receptor mutations in PFCP, TPO mutations in FT and, conversely, TPO receptor (c-mpl) mutations in CAMT. Equivalent ligand or receptor mutations have not been detected in ET or PV patients. However, there is evidence to suggest that c-mpl expression may be dysregulated, with low or absent c-mpl mRNA or protein reported in ET and/or PV patients. At present it is not clear whether this is the cause or consequence of the paradoxically normal/increased TPO levels found with both primary and secondary thrombocytosis. In vitro culture analysis has demonstrated both cytokine independence and hyper-sensitivity as a generalised feature of progenitor cells from many patients, but differences exist depending on the assays used and there is little understanding of the mechanism(s) underlying these responses. Two genes have recently been identified with increased mRNA expression in PV granulocytes: PRV-1, a novel cell surface receptor closely related to the uPAR/Ly6/CD59/snake toxin family of proteins, and NFI-B, a member of the nuclear factor I family which may be associated with TGF-beta resistance. Investigation of their regulation and biological effects may assist in determining the pathobiology of these elusive disorders.

Cytokines↗
PubMed1984

Auranofin, gold sodium thiomalate, and placebo in the treatment of rheumatoid arthritis. Cooperative systematic studies of rheumatic diseases.

Two hundred eight patients were studied in a prospective, controlled, double-blind multicenter trial comparing auranofin (AUR), gold sodium thiomalate (GST), and placebo. One hundred sixty-one patients completed at least 20 weeks of therapy. Response to a variety of measures of efficacy was generally modest for both gold treatment groups although improvement was continuing in both groups at the end of the study. There was statistically significant improvement with both gold preparations compared to placebo for the number of tender joints, the joint tenderness score, and physician assessment of disease severity. GST was also significantly better than placebo for the joint swelling score. GST demonstrated more improvement in patients with anemia and thrombocytosis compared to the other treatment groups and both gold preparations were superior to placebo in improvement of an elevated erythrocyte sedimentation rate. Twenty-seven percent of patients on GST were withdrawn from the study for adverse drug reaction with rash and stomatitis being the predominant cause. Only 6% of patients on AUR were withdrawn for untoward drug effect. The time of onset of the adverse reactions is discussed. The two gold preparations were similar in efficacy although AUR was better tolerated.

Anti-Inflammatory Agents↗
PubMed2003

Interleukin-6 producing pheochromocytoma presenting with acute inflammatory syndrome.

Pheochromocytomas are tumors able to produce catecholamines and a variety of biologically active neuropeptides. We report the case of a 36-yr-old female patient with pheochromocytoma exhibiting headache, intermittent fever, thrombocytosis, and marked inflammatory signs. Nonsteroidal anti-inflammatory drugs were ineffective in lowering the body temperature, while a corticosteroid agent obtained excellent results. IL-6 was found elevated (20 pg/ml); it fell to 4.5 pg/ml 3 weeks after the adrenalectomy, in parallel to normalization of other laboratory data. The interleukin-6 (IL-6) over-production can either be ascribed directly to the tumor (as confirmed by immunohistochemistry) or indirectly accounted for by tumoral production, as a consequence of the high levels of circulating norepinephrine. To our knowledge, our paper represents the 6th case report of IL-6 secreting pheochromocytoma associated with clinical markers of inflammatory response.

Adrenal Cortex Hormones↗
PubMed1998

[Treatment of advanced Budd-Chiari syndrome by liver transplantation].

Budd-Chiari syndrome (BCS) with hepatic vein occlusion is a rare disorder that can effectively be treated in advanced stages with orthotopic liver transplantation. We report on 16 patients who received 18 liver grafts and were followed up for at least 2 years. In 7 patients a hematological disorder was confirmed by bone marrow biopsy. One patient died after 4 months due to cytomegalovirus pneumonia; another patient died after 2 years due to progressive liver failure after portal vein thrombosis. The actuarial 5-year survival rate is 87.5% compared to 85.3% in all other 710 orthotopic liver transplantations performed from September 1988 to December 1995 at our institution. Anticoagulation consisted of intravenous heparin and overlapping continuation with dicoumarin. Three patients received hydroxyurea for thrombocytosis, one patient for 1 week only early after the transplantation. Two postoperative abdominal hemorrhages required laparotomy. Two patients had to be retransplanted, one for thrombosis of the hepatic artery and portal vein after discontinuation of dicoumarin due to GI bleeding and one for hepatic vein thrombosis after insufficient dicoumarin intake. Terminal BCS represents a good indication for orthotopic liver transplantation; however, life-long, closely monitored anticoagulation is essential.

Adolescent↗
PubMed1999

[Experience with the therapy of acute finger ischemia by high-dose intra-arterial infusion of PGE1 through a surgically introduced catheter].

We present five patients with serious ischemia of the fingers caused by intra-arterial injection injury, trauma, rheumatoid arthritis, chronic myeloid leukemia with thrombocytosis and thoracic-outlet syndrome. The treatment consisted of thrombectomy and intra-arterial lysis if possible and intra-arterial infusion of 80 microg PGE1/24 h in combination with 25,000 U of heparin/24 h for 6-10 days. PGE1 and heparin were administered through a 3-F catheter introduced surgically into a forearm artery. In three cases a complete remission was possible, in the other two cases the therapy led to significant improvement. No side effects or complications were noticed.

Acute Disease↗
PubMed2004

Exercise-induced platelet and leucocyte activation is not enhanced in well-controlled Type 1 diabetes, despite increased activity at rest.

AIMS/HYPOTHESIS: Stress can evoke a prothrombotic state with activated platelets and leucocytes, and increased platelet activation at rest has been reported for diabetic subjects. Thus, prothrombotic responses to stress may be enhanced in diabetes mellitus. We therefore evaluated platelet and leucocyte responses to exercise in Type 1 diabetic patients. METHODS: Type 1 diabetes mellitus patients with good metabolic control and healthy subjects matched for age and body mass index ( n=15 for both) performed a maximal exercise test. Platelet and leucocyte activation and their heterotypic aggregation were monitored by whole blood flow cytometry. RESULTS: Diabetic platelets did not show higher basal levels of P-selectin expression, but were more reactive to ADP and thrombin stimulation. Diabetic patients had increased lymphocyte and monocyte CD11b expression, and increased circulating platelet-monocyte aggregates. Exercise evoked thrombocytosis, increased circulating platelet P-selectin expression, enhanced platelet sensitivity to ADP and thrombin, and elevated plasma levels of soluble P-selectin to a similar degree in diabetic patients and healthy subjects. Exercise induced marked leucocytosis and elevated plasma elastase, but only slightly increased leucocyte CD11b expression and leucocyte reactivity to stimulation by N-formyl-methionyl-leucyl-phenylalanine. In all of these there was no difference between diabetic patients and healthy subjects. The numbers, but not percentages of circulating platelet-leucocyte aggregates were markedly increased by exercise, but the increase was less prominent among diabetic patients. CONCLUSIONS/INTERPRETATION: Strenuous exercise evokes platelet and leucocyte activation in Type 1 diabetic patients and healthy subjects. Platelet and monocyte hyperactivity were found at rest, but responses to stress were not augmented in metabolically well-controlled Type 1 diabetes mellitus patients.

Adenosine Diphosphate↗
PubMed2001

Efficacy and tolerability of piperacillin/tazobactam versus ceftazidime in association with amikacin for treating nosocomial pneumonia in intensive care patients: a prospective randomized multicenter trial.

OBJECTIVE: To compare clinical and bacteriological efficacy as well as tolerability of two regimens of broad-spectrum antibiotics (ceftazidime versus piperacillin/tazobactam) combined with amikacin in the treatment of nosocomial pneumonia in intensive care patients. DESIGN: Open label, prospective, multicenter, and randomized phase III clinical trial. SETTING: Medical or surgical intensive care units (ICUs) of nine acute-care teaching hospitals in Spain. PATIENTS AND PARTICIPANTS: One hundred and twenty-four ICU patients with nosocomial pneumonia and requiring mechanical ventilation were included. They were randomized to receive amikacin (15 mg/day divided into two doses) combined with either piperacillin (4 g every 6 h) and tazobactam (0.5 g every 6 h) (n = 88) or ceftazidime (2 g every 8 h) (n = 36). MEASUREMENTS AND RESULTS: The causative pathogen was determined in 60.2% of patients in the group of amikacin plus piperacillin/tazobactam and in 76.9% in the group of amikacin plus ceftazidime. A total of 94 bacterial organisms were isolated among which gram-negative bacilli predominated, Pseudomonas aeruginosa being the most frequent. Clinical response at the end of antibiotic therapy was considered satisfactory (cure and/or improvement) in 63.9% of patients in the amikacin plus piperacillin/tazobactam group and in 61.5% in the amikacin plus ceftazidime (odds ratio 1.1; 95% confidence interval 0.44-2.75). Eradication or presumptive eradication rates for each pathogen and for either gram-negative or gram-positive bacteria were similar in both antibiotic combinations (odds ratio 1.2; 95% confidence interval 0.39-3.66). A total of 21 adverse effects (23.9%) occurred in the amikacin plus piperacillin and tazobactam group and six (16.7%) in the amikacin plus ceftazidime group, thrombocytosis, renal dysfunction, and hepatic cytolysis being the most common. The efficacy and tolerability of the two therapeutic regimens were similar not only in the whole study population, but also in the subset of P. aeruginosa-related pneumonia (odds ratio 1; 95% confidence interval 0.08-13.37). CONCLUSIONS: Amikacin associated with either ceftazidime or piperacillin and tazobactam has shown comparable efficacy and tolerability in the treatment of ICU patients with nosocomial pneumonia.

Aged↗
PubMed2004

Transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of venous symptomatic chronic portal thrombosis in non-cirrhotic patients.

PURPOSE: To present a series of cases of non-cirrhotic patients with symptomatic massive portal thrombosis treated by percutaneous techniques. All patients underwent a TIPS procedure in order to maintain the patency of the portal vein by facilitating the outflow. METHODS: A total of six patients were treated for thrombosis of the main portal vein (6/6); the main right and left branches (3/6) and the splenic vein (5/6) and superior mesenteric vein (6/6). Two patients had a pancreatic malignancy; one patient with an orthotopic liver transplant had been surgically treated for a pancreatic carcinoma. Two patients had idiopathic thrombocytosis, and in the remaining patient no cause for the portal thrombosis was identified. During the initial procedure in each patient one or more approaches were tried: transhepatic (5/6), transileocolic (1/6), trans-splenic (1/6) or transjugular (1/6). In all cases the procedure was completed with a TIPS with either ultrasound guidance (3/6), "gun-shot" technique (2/6) or fluoroscopic guidance (1/6). RESULTS: No complications were observed during the procedures. One patient had a repeat episode of variceal bleeding at 30 months, one patient remained asymptomatic and was lost to follow-up at 24 months, two patients were successfully treated surgically (cephalic duodenopancreatectomy) and are alive at 4 and 36 months. One patient remains asymptomatic (without new episodes of abdominal pain) at 16 months of follow-up. One patient died because of tumor progression at 10 months. CONCLUSION: Percutaneous techniques for portal recanalization are an interesting alternative even in non-acute thrombosis. Once flow has been restored in the portal vein TIPS may be necessary to obtain an adequate outflow, hence facilitating and maintaining the portal flow.

Aged↗
PubMed2002

Splenectomy in patients with mixed myelodysplastic/myeloproliferative disease.

According to the classification of the World Health Organization, the designation myelodysplastic/myeloproliferative disorder, unclassifiable may be applied to cases that have clinical, laboratory, and morphologic features that support a diagnosis of a myelodysplastic syndrome (MDS) as well as a myeloproliferative disorder (MPD), but that do not meet the criteria for any of the other entities included in the MDS/MPD category [3]. In this paper we report on two Caucasian patients with unclassifiable myelodysplastic syndromes with proliferative characteristics. Both patients were suffering from thrombocytopenia and splenomegaly and underwent splenectomy. The weight of the spleen specimens was more than 2000 g. Histopathology findings revealed a marked infiltration of the spleen with extramedullary hematopoiesis. After surgery, one patient showed a rapid increase of platelets in peripheral blood and developed severe thrombocytosis. In the other case, the patient was suffering from a decrease of platelets and died in hypovolemic shock caused by gastrointestinal bleeding. In summary, these two cases demonstrate the difficulties of prognosis and treatment in patients with mixed myelodysplastic/myeloproliferative disorders. Additionally, we indicate the potential positive outcome of splenectomy as ultima ratio in patients with these hematological features and severe thrombopenia.

Blood Cell Count↗
PubMed2005

Conventional cytogenetics of myeloproliferative diseases other than CML contribute valid information.

In chronic myeloproliferative disorders other than CML (CMPD) recurrent cytogenetic abnormalities occur, but specific patterns of chromosomal aberrations in the specific entities have so far not been detected. Thus, the value of conventional cytogenetics in the routine diagnostic setting of CMPD remains to be clarified. We performed a cytogenetic study on 409 patients with different CMPD [polycythemia vera, essential thrombocytosis (ET), idiopathic osteomyelofibrosis, chronic myelomonocytic leukemia (proliferative subtype), idiopathic hypereosinophilic syndrome (HES), myeloproliferative syndrome (unclassifiable)] and on 102 patients with suspected CMPD. Cytogenetic abnormalities occurred in different frequencies ranging from 3 to 40% depending on the subtype, and showed some specific differences with respect to their type. The highest frequency and the most complex pattern of clonal aberrations were observed in idiopathic osteomyelofibrosis. However, clonal aberrations were also found in 10% of patients with suspected CMPD establishing the diagnosis of a malignant disease. In conclusion, cytogenetics are essential in the routine diagnostic setting of CMPD or cases suspicious for CMPD. In ET and in HES the aberration rate was only 3 and 7%, respectively. Thus, cytogenetics can be omitted. However, in some of these cases molecular procedures should be integrated into the routine diagnostic process.

Adult↗
PubMed2006

Grade of bone marrow fibrosis is associated with relevant hematological findings-a clinicopathological study on 865 patients with chronic idiopathic myelofibrosis.

Controversy continues to exist regarding not only the exact definition and grading of myelofibrosis (MF), but also whether, and to what extent, this feature may be correlated with clinical findings. A retrospective study was performed involving 865 bone marrow (BM) biopsies together with the clinical records from patients with chronic idiopathic myelofibrosis (CIMF). Diagnosis was established according to the World Health Organization criteria, and assessment of MF followed a consensus scoring system that included four grades (MF-0 to MF-3). Histopathological and clinical evaluations were carried out in an independent fashion. Prefibrotic and early CIMF (MF-0/-1) were presented by 565 patients showing borderline to mild anemia and no or slight splenomegaly, but frequently, thrombocytosis exceeding 500x10(9)/l was shown. In 300 patients, manifest reticulin and collagen fibrosis (MF-2/-3) were characterized by marked anemia, gross splenomegaly, peripheral blasts, and normal to decreased platelet and leukocyte counts. The latter cohort was consistent with findings generally in keeping with MF with myeloid metaplasia. Regarding the stepwise evolution of disease, sequential BM examinations showed that in 103 patients, prefibrotic and early CIMF transformed into advanced stages accompanied by correspondingly developing clinical and histomorphological features. Survival analysis (univariate calculation) revealed a significantly more favorable prognosis in prefibrotic vs advanced stages of CIMF. On the other hand, higher classes of MF also exerted a higher clinical risk profile (Lille score). In conclusion, the dynamics of the disease process in CIMF are characterized by evolving MF in the BM and closely associated changes of relevant hematological findings.

Biopsy↗
PubMed1996

Cloning of thrombopoietin and its therapeutic potential.

Recently, four groups reported the cloning of thrombopoietin (TPO), also called c-Mpl ligand, from various species. In this study, we examined the in vitro and in vivo biological activity of TPO and its therapeutic efficacy in experimental animal models. Recombinant human TPO (rhTPO) supported the formation of only megakaryocyte (MK) colonies from rat marrow MK progenitor cells [colony-forming units-megakaryocyte (CFU-MK)] and predominantly acted on GpIIb/IIIa+ CFU-MK at the late stage of differentiation. MKs generated from rat GpIIb/IIIa+ CFU-MK after 3 days of liquid culture in the presence of rhTPO had mature characteristics. rhTPO stimulated an increase in the size of TPO-induced cultured rat MKs and in the number of elongated cytoplasmic processes, also called proplatelets, from these MKs in a dose-dependent manner. Administration of rhTPO to normal BALB/c mice daily for 5 days caused dose-dependent thrombocytosis. Treatment with rhTPO induced an increase in the size and number of marrow MKs and an expansion of the marrow CFU-MK pool. We further examined the effects of rhTPO on chemotherapy-induced thrombocytopenia in animal models. Following treatment with mitomycin C, mice received daily injections of various doses of rhTPO. Administration of rhTPO reduced the severity of thrombocytopenia and accelerated the recovery of platelets in a dose-dependent fashion: there was a significant reduction in the decrease in numbers of marrow MKs and CFU-MK with rhTPO treatment. Treatment with rhTPO also significantly improved neutropenia in mitomycin C-treated mice. Similar therapeutic efficacy was observed in cynomolgus monkeys with thrombocytopenia induced by nimustine. In addition, there was no significant change in several serum-chemistry parameters, in C-reactive protein, an acute phase protein, or in some variables involved in the blood-coagulation system. Furthermore, platelets from mice made thrombocytotic by repeated administration of rhTPO showed normal aggregation function. These results strongly suggest the clinical usefulness of rhTPO for the treatment of thrombocytopenia.

Animals↗
PubMed1999

Erucylphosphocholine: pharmacokinetics, biodistribution and CNS-accumulation in the rat after intravenous administration.

The clinical use of alkylphosphocholines (APC) in cancer patients is restricted because of the high gastrointestinal toxicity and the need for oral administration. Therefore we evaluated the clinical pharmacology of erucylphosphocholine (ErPC), the first derivative of the APC family suitable for intravenous administration with strong antineoplastic activity, in vitro and in vivo in rats. The pharmacokinetic parameters after a single intravenous dose of 40 mg/kg were calculated using a two-compartment model: C(max) = 1.6 +/- 0.3 micromol/ml, T(1/2alpha) = 0.18 +/- 0.09 h, T(1/2beta) = 3.3 +/- 0.88 h, clearance = 9.7 +/- 1.2 ml/h, AUC = 2.5 +/- 0.3 micromol/ml per h and Vss = 40.4 +/- 7.9 ml. Biodistribution studies were performed after repeated ErPC administration at different doses. Intravenous injections of 20 mg/kg given at intervals of 48 h for up to 4 weeks were well tolerated. Neither clinical evaluation nor laboratory parameters (haematology and clinical chemistry) revealed toxic side effects. In contrast, higher doses of ErPC (40 mg/kg per 48 h) led to weight loss. After 2 and 4 weeks of therapy with 20 mg/kg per 48 h a high ErPC accumulation was found in the adrenal glands, small intestine and brain. The brain to serum concentration ratios averaged 2.1 after 2 weeks and 4.5 after 4 weeks. Significant leucocytosis and thrombocytosis were observed after 4 weeks of ErPC treatment. The findings suggest that ErPC is a suitable candidate for clinical trials. In particular, owing to the high accumulation in brain tissue, ErPC is a potential agent for chemotherapy against malignant brain tumours.

Animals↗
PubMed2004

A case of familial Mediterranean fever and polyarteritis nodosa complicated by spontaneous perirenal and subcapsular hepatic hemorrhage requiring multiple arterial embolizations.

The association of familial Mediterranean fever (FMF) and polyarteritis nodosa (PAN) has been well established. These patients have been reported to have an overall better prognosis than other PAN patients. Herein we report a patient with FMF and PAN who died of sepsis following a severe course of recurrent bleeding episodes which required multiple embolization attempts. The 39-year-old Turkish male presented with abdominal pain of 1-month duration. He had been diagnosed with FMF at the age of 24. On admission, he had pallor with general ill appearance. Rebound tenderness was obtained in the right upper abdominal quadrant. He had mild anemia, leukocytosis, thrombocytosis, and hypoalbuminemia. On the 2nd day of his admission, he developed hypotension with a rapid decline in hemoglobin level. Abdominal angiography showed multiple aneurysms in the branches of renal arteries, superior mesenteric artery, and hepatic arterial system including left renal infarct, suggesting PAN. He was put on high-dose steroids and oral cyclophosphamide. Despite medical treatment, he developed intense abdominal pain, hypotension, tachycardia, and a rapid fall in hemoglobin on four occasions. Active bleeding sites were embolized in two different angiography sessions. Although the patient experienced no more recurrent bleeding, he died of multiorgan dysfunction syndrome resulting from sepsis 6 weeks after admission. Polyarteritis nodosa associated with FMF may follow a grave course despite immunosuppressive therapy. Arterial embolization should be considered in the presence of bleeding aneurysms in addition to immunosuppressive therapy.

Adult↗
PubMed2004

The effect of infliximab on extraintestinal manifestations of Crohn's disease.

OBJECTIVE: The purpose of this open pilot study was to assess prospectively the effect of infliximab on extraintestinal manifestations in patients with active Crohn's disease refractory to conventional treatment. PATIENTS AND METHODS: Twenty-two consecutive patients with Crohn's disease and one with ulcerative colitis presenting at least one of the known extraintestinal manifestations of Crohn's disease participated in the study. All the patients had Crohn's disease activity index (CAI) scores above 2. Each patient received an intravenous infusion of infliximab at a dosage of 5 mg/kg. A thorough questionnaire was used reviewing the extraintestinal manifestations of Crohn's disease such as erythema nodosum, pyoderma gangrenosum, eye lesions, arthritis or arthralgia, sacroiliitis or inflammatory back pain, hepatic disease, hematologic manifestations (megaloblastic, iron deficiency or hemolytic anemia, thrombocytosis), thrombosis, and nephrolithiasis. Musculoskeletal complaints were evaluated using the parameters intensity of pain, duration of morning stiffness (in minutes), presence of inflammatory back pain, Schober's test of the lumbar region, chest expansion, and distance from occiput to wall. The clinical assessment was performed on the day of the infusion and 2 weeks later. RESULTS: Eleven out of 23 patients had arthralgia of inflammatory nature, three others had evidence of active synovitis on physical examination, and 11 reported inflammatory back pain. Four patients suffered from protracted pyoderma gangrenosum; three had resistant aphthous stomatitis. Eleven patients had more than one extraintestinal manifestation. All four with pyoderma gangrenosum demonstrated significant improvement of their ulcers after one course of infliximab, with complete resolution of the skin lesions in three of them after repeated infusions of infliximab. Aphthous stomatitis completely responded in all patients after a single infusion. Seven out of 11 patients with arthralgia and seven out of 11 with inflammatory back pain/sacroiliitis experienced benefit after treatment with infliximab and reported at least partial clinical improvement in duration of morning stiffness, tender joint count, and visual analogue scale for pain. Only one of three patients with frank arthritis demonstrated clear improvement, and two others failed to respond to infliximab treatment. CONCLUSION: These preliminary results are encouraging and suggest a promising role of infliximab in the treatment of extraintestinal symptoms of Crohn's disease.

Adult↗
PubMed2003

A trend of improved survival of childhood hepatoblastoma treated with cisplatin and doxorubicin in Taiwanese children.

Hepatoblastoma is the second most common childhood malignant hepatic tumor in Taiwan. Its prognosis used to be poor. We reviewed our cases in this decade to see if there has been any improvement of survival in our patients with hepatoblastoma. From 1988 to 2000, 19 patients with hepatoblastoma in this institution were included in the study. These patients' clinical manifestations, laboratory and image studies, histological findings, treatment modalities and prognostic significance were analyzed. The mean age at diagnosis was 13.5 months, ranging from 0 to 4 years old (male:female =11:8). Abdominal distension was the most common symptom, and hepatomegaly was the most common physical finding. Laboratory abnormalities included elevated alpha-fetoprotein, thrombocytosis and abnormal liver function profiles. Treatment modalities included primary surgery with postoperative chemotherapy in three, chemotherapy only in four and preoperative chemotherapy plus surgery with or without postoperative chemotherapy in nine patients. The overall 2-year survival rate is 38.6%. The significant prognostic factors include patients' compliance, resectability and chemotherapy protocol. The introduction of a new chemotherapy protocol designed by the International Society of Pediatric Oncology Study (SIOPEL) in 1994 improved the 2-year survival rate from 12.5 to 58.4% ( P=0.01). In conclusion, the improved chemotherapy protocol enhances the survival rate of hepatoblastoma in Taiwanese children.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Splenic rupture complicating periinterventional glycoprotein IIb/IIIa antagonist therapy for myocardial infarction in polycythemia vera.

Polycythemia vera is a myeloproliferative disorder predisposing to thromboembolic and bleeding complications. We report the case of a patient with polyglobuly, leukocytosis, and thrombocytosis, who suffered from acute ST-segment elevation myocardial infarction due to thrombotic high-grade pre-stent stenosis two months after percutaneous coronary intervention for complex coronary one vessel disease. Following re-PTCA and stent implantation in conjunction with periinterventional GP IIb/IIIa antagonist treatment, the patient was initially symptom free for about two hours before rapidly developing signs of a hemorrhagic shock. An abdominal CT scan showed splenic rupture with massive intraabdominal hemorrhage as a consequence of secondary bleeding into multiple pre-existing splenic infarctions. The patient's condition stabilized after emergency splenectomy. Subsequent bone marrow biopsy revealed the presence of polycythemia vera. Post-operatively, the patient was treated with the anti-platelet agents aspirin and clopidogrel to prevent subacute stent thrombosis. Additionally, cyto-reductive therapy with hydroxyurea was initiated because of a further increase in the platelet count. In patients with polycythemia vera, the indication for treatment with GP IIb/IIIa antagonists should be carefully weighed against the potentially serious bleeding complications. Should treatment be established, a risk stratification using abdominal sonography and bleeding time testing is recommended, while during treatment red blood count, platelet count, coagulation tests, and hemodynamic parameters should be closely monitored.

Abciximab↗