Aortic valve replacement and splenectomy in a patient with chronic idiopathic thrombocytopenic purpura—preoperative management with high-dose γ-globulin

Summary We report the management of a patient with chronic idiopathic thrombocytopenic purpura and severe aortic valvular disease. Preoperative intravenous high-dose -globulin administration was employed, and aortic valve replacement combined with splenectomy were performed during the same operation. The platelet count at admission was 34,000/mm³ and increased to 146,000/mm³ after the -globulin therapy. Platelet transfusion at the end of the cardiopulmonary bypass was considered no longer necessary in the postoperative period, because the platelet count increased quickly after the procedure. The post-operative course was uneventful. We believe that open heart surgery and splenectomy can successfully be performed simultaneously in a patient with chronic idiopathic thrombocytopenic purpura treated with high-dose -globulin therapy.     

Immune thrombocytopenic purpura associated with rheumatoid arthritis: case report

A 54-year-old Japanese woman was diagnosed with rheumatoid arthritis (RA) in 1995 on the basis of symmetric effusive polyarthritis, morning stiffness, and strongly positive rheumatoid factor. She had received low-dose prednisolone, indomethacin, methotrexate (MTX), and cyclophosphamide (CPA), at least, over 4 years before the current admission and showed partial improvement of polyarthralgia. In November 2002, she suddenly developed thrombocytopenia (platelet count was 0.3×10⁴ mm^–3) with purpura and was diagnosed with immune thrombocytopenic purpura (ITP). As she had refractory ITP, the administration of pulsed high-dose dexamethasone (DEX) therapy was started, resulting in the complete remission of ITP. The present paper reports that pulsed high-dose DEX therapy was useful for the treatment of refractory ITP associated with RA.     

A case of pulmonary tuberculosis accompanied with immune thrombocytopenic purpura

A 30-year-old Japanese woman with chest pain and nodules in the left upper lung field was diagnosed as having pulmonary tuberculosis by sputum examination. Purpura on her legs had lasted for 3 months and her platelet count was 1.9 × 10⁴/mm³ on admission. She was also diagnosed as having immune thrombocytopenic purpura because of elevation of serum PA-IgG and proliferation of megakaryocytes in the bone marrow. Anti-tubercular therapy and steroid therapy were concurrently performed, resulted in recovery of the platelet count. Steroid therapy was gradually tapered off and then withdrawn, thereafter anti-tubercular therapy was finished. She has been relapse-free.Cases of pulmonary tuberculosis accompanied with immune thrombocytopenic purpura are rare. The pathogenesis in the present case was suggested to have occurred through an immunological mechanism.     

Successful treatment of idiopathic thrombocytopenic purpura by Chinese herbal medicine EK-49 and ascorbic acid in an elderly patient developing chronic subdural hematoma

Treatment of idiopathic thrombocytopenic purpura can present difficult therapeutic choices. An 88-year-old woman was admitted for treatment of a right subdural hematoma. She had a history of chronic thrombocytopenia, with follow-up evaluations by a local physician. Platelet count on admission was 5 × 10³/µL, with a high serum concentration of platelet-associated immunoglobulin G. Bone marrow examination showed a marked increase in megakaryocytes. The patient was diagnosed with idiopathic thrombocytopenic purpura accompanied by chronic subdural hematoma. An increase in platelet count and gradual resolution of the subdural hematoma were obtained with prednisolone and azathioprine administration, but platelet count decreased when steroid tapering was attempted. Ultimately we administered the Chinese herbal medicine EK-49 and ascorbic acid, with a gradual increase in platelet count and no adverse effects. Some elderly patients with chronic subdural hematoma can be treated non-invasively. Further, a combination of EK-49 and ascorbic acid may be an effective treatment for refractory idiopathic thrombocytopenic purpura.     

Appendiceal tumour--retrospective clinicopathological analysis.

Appendiceal tumours are rare and often discovered unexpectedly in an acute situation in which decision-making is difficult. We report the spectrum of appendiuar tumours seen in our institution over a period of more than 10 years, and discuss the clinicopathological behaviour, investigations, surgical procedures and outcomes in these patients. We have also reviewed the literature with regard to appendiceal tumours. Appendicular tumours were identified from the database of 1646 appendictomies (18% in children) performed in single centre and case notes were reviewed. Clinical presentation, investigations, histopathology, surgical procedures and outcome were analysed. Twelve patients with appendiceal tumours were identified (0.72%): 8 carcinoid, 2 mucinous (mucocele) and 2 adenocarcinoma. All the patients with a carcinoid tumour presented with features suggestive of acute appendicitis and were diagnosed postoperatively following appendicectomy and formal histology. No further surgical intervention was required as these lesions were less than 1cm away from the base of the appendix. One of the patient with mucinous cystadenoma presented acutely and underwent an appendicectomy; in the other patient with chronic pain, apreoperative MRI suggested the diagnosis leading to a planned hemicolectomy as the lesion was close to the base of the appendix. While one of the patient with an adenocarcinoma localized to the appendix did well following a right hemicolectomy, the other patient with disseminated disease succumbed within a year. Carcinoid tumours are the commonest appendiceal tumours, which present often as acute appendicitis. While appendicectomy would be adequate in most of these patients, in patients with a cystadenoma close to the base of the appendix or in case of a carcinoma, a right hemicolectomy is the appropriate option. While the prognosis is good in patients with carcinoid tumour and cystadenoma, it remains dismal in patients with disseminated malignant disease.     

Appendiceal mucocele with concomitant colonic cancer

PURPOSE: Mucocele of the appendix is an uncommon disorder, usually found incidentally during ultrasonography or radiographic studies. We report two cases of combined appendiceal mucocele and colonic cancer. METHODS: The two cases were analyzed for the clinicopathologic characteristics such as history, presentation, laboratory data, radiologic and endoscopic studies, pathology, and p53 immunoreactivity. RESULTS: Two patients were diagnosed with an appendiceal mucocele by ultrasound of the abdomen, together with computed tomography. Colonoscopic examination subsequently revealed synchronous colonic adenocarcinoma in both patients. Ileocecal resection following endoscopic polypectomy and a right hemicolectomy was performed for each patient. An appendiceal mucocele was histologically diagnosed as a mucinous cystadenoma. Immunohistochemical detection of abnormally high level of p53 protein was observed in colonic adenocarcinomas of both patients, whereas both appendiceal cystadenomas were negative for p53. CONCLUSIONS: To be remembered is the high frequency of concomitant gastrointestinal tumors in patients with appendiceal mucocele, especially caused by mucinous neoplasms. A total colonoscopic surveillance will afford earlier diagnosis of synchronous colonic cancers in these patients.     

Helicobacter pylori-associated immune thrombocytopenia

Idiopatic thrombocytopenic purpura (ITP), a disorder characterized by autoantibody-mediated platelet destruction, may be primary or secondary to various illnesses including lymphoproliferative, autoimmune, or infectious diseases. There are increasing data on the association between Helicobacter pylori infection and idiopathic thrombocytopenic purpura and the significant increase in platelet count after bacterial eradication. The aim of this review is to consider the studies so far published on Helicobacter pylori infection and idiopathic thrombocytopenic purpura in order to evaluate a possible pathogenic correlation between these two conditions. A review of the literature data show that Helicobacter pylori eradication in patients with idiopathic thrombocytopenic purpura is effective in increasing platelet count in approximately half of the cases. However, since the studies so far published are few, sometimes controversial and involve small series of patients, further controlled studies on larger numbers of patients with longer follow-up are needed to confirm these preliminary findings.     

Thrombotic thrombocytopenic purpura in a previously splenectomized patient

Idiopathic thrombocytopenic purpura was diagnosed in a 26 year old man who had rectal bleeding and marked thrombocytopenia (10,000 platelets/mm³). Complete recovery followed treatment with steroids and splenectomy. There was no clinical, laboratory or histopathologic evidence of thrombotic thrombocytopenic purpura. Several months later typical thrombotic thrombocytopenic purpura developed; recovery followed treatment with steroids, aspirin and dipyridamole. The presence of Howell-Jolly bodies and a negative scan indicated that an accessory spleen was not present. Since thrombotic thrombocytopenic purpura developed in the absence of a spleen in this case, it may be that in some, if not all, instances of thrombotic thrombocytopenic purpura the spleen is not importantly related to the pathogenesis of thrombotic thrombocytopenic purpura; this is in contrast to the situation in idiopathic thrombocytopenic purpura. Benefits attributed to splenectomy in thrombotic thrombocytopenic purpura, therefore, may in fact be due to the platelet inhibitory properties of common anesthetic agents, or to some other factor in the surgical procedure rather than to removal of the spleen per se.     

Intravenous immunoglobulin (i.v. IgG) for previously treated acute or for chronic idiopathic thrombocytopenic purpura (ITP) in childhood: a prospective multicenter study

Summary In a prospective multicenter study 42 thrombocytopenic (<30×10⁹ platelets/l) children with chronic idiopathic thrombocytopenic purpura (ITP) or with acute ITP, dependent on or refractory to corticosteroids, were given 0.4 g i.v. IgG/kg body weight/day on 5 consecutive days and thereafter once a week if the platelet count fell to <20×10⁹/l or if the patient bled. After the initial 5 days of i.v. IgG the platelets rose within a mean of 7–8 days to >30×10⁹/l in all and to >150×10⁹/l in 33 of 42 patients (79%). After a mean observation time of 26.6 months 26 of 42 patients (62%) showed a satisfactory long-term effect, i.e. no need for treatment for at least 6 months without bleeding and with no platelet counts below 20×10⁹/l. No difference in response rate was found between children with chronic and those with previously treated acute ITP. These results indicate that i.v. IgG could be used to control emergency situations, e.g. to stop bleeding or to prepare a patient for surgery. I.v. IgG also represents a good alternative to treatment modalities, such as splenectomy and/or the administration of cytostatic immunosuppressants with potentially serious side effects. In addition to the expected transient rise in serum IgG levels, i.v. IgG induced a more prolonged elevation of serum IgM. Platelet associated IgG, elevated before therapy, was correlated with the clinical long-term outcome.This paper is part of the doctoral thesis of B. I. Responsible physicians of hospitals: see Appendix     

Cyclosporin therapy for idiopathic thrombocytopenic purpura

Nine adult patients with chronic idiopathic thrombocytopenic purpura (ITP) were treated with cyclosporin. Their platelet counts were all below 5 x 10(4)/microliters. It was administered orally at 5 mg/kg/day for 8 weeks. In one patient, the platelet count increased over 10 x 10(4)/microliters in 4 patients it did over 5 x 10(4)/microliters. Gingival hyperplasia was observed in one patient. Renal dysfunction was not observed in any patients. The elevation of PAIgG declined during the period of treatment. These results suggest that this therapy may be useful in refractory idiopathic thrombocytopenic purpura.     

Mucinous cystadenoma of appendix concomitant with perityphlic granuloma

We describe a case of mucinous cystadenoma of the appendix with perityphlic granuloma. The patient developed a hard palpable mass in the right lower quadrant and barium enema inducted irregular bowel wall at the cecum. Based on a preoperative diagnosis of carcinoma of the appendix, we performed a laparotomy. On operation, we found a tumor mass in the region of the appendix; the mass adhered strongly to the retroperitoneum and cecum. Right hemicolectomy was carried out on a diagnosis of carcinoma of the appendix. Microscopic examination revealed mucinous cystadenoma of the appendix with perityphlic granuloma. Mucinous cystadenoma in the appendix is a rare tumor. In this patient, the tumor was accompanied by granuloma formation in adjacent tissues because of mucin expelled from the appendix. This case emphasizes that granuloma formation can make of difficult to differentiate mucinous cystadenoma in the appendix from cancer.     

Giant appendiceal mucocele: Report of a case and brief review

Mucocele of the appendix is a rare lesion, characterized by distension of the lumen due to accumulation of mucoid substance. This disease is often asymptomatic and pre-operative diagnosis is rare. If untreated, one type of mucocele may rupture producing a potentially fatal entity known as pseudomyxoma peritonei. The type of surgical treatment is related to the dimensions and to histology of the mucocele. Appendectomy is used for simple mucocele or for cystadenoma. Right hemi-colectomy is recommended for cystadenocarcinoma. In this paper, we report a case of a 51-year-old woman with a mobile, painless mass in the right lower quadrant of abdomen caused by a giant appendiceal mucocele. Imaging showed a large, tubular,cystic structure extending below from the inferior wall of the cecum. Surgery revealed a giant retro-cecal appendix measuring 17 cm in length and 4 cm in diameter. The final pathologic diagnosis was mucocele caused by mucinous cystadenoma.     

Giant appendiceal mucocele： Report of a case and brief review

Mucocele of the appendix is a rare lesion, characterized by distension of the lumen due to accumulation of mucoid substance. This disease is often asymptomatic and pre-operative diagnosis is rare. If untreated, one type of mucocele may rupture producing a potentially fatal entity known as pseudomyxoma peritonei. The type of surgical treatment is related to the dimensions and to histology of the mucocele. Appendectomy is used for simple mucocele or for cystadenoma. Right hemi-colectomy is recommended for cystadenocarcinoma. In this paper, we report a case of a 51-year-old woman with a mobile, painless mass in the right lower quadrant of abdomen caused by a giant appendiceal mucocele. Imaging showed a large, tubular, cystic structure extending below from the inferior wall of the cecum. Surgery revealed a giant retro-cecal appendix measuring 17 cm in length and 4 cm in diameter. The final pathologic diagnosis was mucocele caused by mucinous cystadenoma.     

Study of platelet-associated immunoglobulins of IgG,IgM, IgA,and IgE classes and platelet kinetics in 33 patients with idiopathic thrombocytopenic purpura

Summary The role of platelet-associated immunoglobulins (PAIg) of four different immunoglobulin classes -IgM, IgG, IgA, and IgE- and their relation to platelet count and platelet kinetics was studied in 33 patients with idiopathic thrombocytopenic purpura (ITP). During the course of 1 year, repeated determinations of PAIg were made. The results indicate that PAIgG, PAIgM, and PAIgA are present in all ITP patients, and that autoantibodies of all three Ig classes show highly significant correlations to the platelet counts (p< 0.0001). Double logarithmic negative correlations have been found between PAIgG and platelet count (r=–0.71), PAIgM and platelet count (r=–0.84), and PAIgA and platelet count (r=–0.79). Statistical analyses using partial correlation and multiple regression methods showed that PAIgM is predominantly related to the platelet count, whereas PAIgG and PAIgA are only of secondary importance. Accordingly, a relation of PAIgM (and PAIgA) to increased liver destruction of platelets was found in kinetic studies using¹¹¹indium-labeled platelets. Taken together, these results suggest a predominant role of PAIgM in the pathogenesis of ITP.     

特发性血小板减少性紫癜合并抗磷脂抗体综合征患者ACS后成功PCI术1例

1 病例资料 患者,女,71岁,因"胸闷1d,突发晕厥1次"入院.患者本次入院为1d前(上午10时许)活动时出现压迫性胸骨中段胸闷,伴左侧手臂痛,出冷汗,伴头晕恶心,家属急送至我院急诊(约14:00时),患者于急诊门口突发晕厥,心电监护提示心室颤动,立即予心肺复苏、电除颤,10余分钟后好转,急查肌钙蛋白7.52 ng/...     

Mucinous cystadenoma of the appendix associated with adenocarcinoma of the sigmoid colon and hepatocellular carcinoma of the liver： Report of a case

Mucinous cystadenoma of the appendix is a rarecondition and represents one of the three entities withthe common name mucocele of the appendix.It ischaracterized by a cystic dilatation of the lumen withstasis of mucus inside it.Histopathologically mucoceleis divided into three groups:focal or diffuse mucosalhyperplasia,mucinous cystadenoma and mucinouscystadenocarcinoma.This condition is often associatedwith other neoplasia,especially adenocarcinoma ofthe colon and ovaries.We here describe a 57 year oldmale patient who presented with abdominal discomfort,constipation,fresh blood in stool and frequent urination.He had a big cystadenoma of the appendix associatedwith adenocarcinoma of the colon and hepatocellularcarcinoma of the liver.The patient underwent righthaemicolectomy,sigmoid colon resection and segmentalresection of the liver.Now 3 years later he has noevidence of disease relapse.According to this,westress the need of accurate preoperative diagnosis andintraoperative exploration of the whole abdomen in thesepatients.     

Immune Thrombocytopenic Purpura ITP

Immune thrombocytopenic purpura ITP is characterized by early platelet destruction due to an unbalanced immune response. In acute ITP, a transient increase of HLA-DR molecules has been detected while in individuals with chronic ITP, in addition, increased serum concentrations of IL-2 and other cytokines reflecting in vivo T-cell activation have been observed. Clinically, the hemorrhagic manifestation of ITP rather than the platelet count should define the indication for active intervention. In a staging system a patient with stage III has bleeding signs and platelet counts below 10 or 20 times 10⁹/L and needs treatment, a patient with stage II should be treated on an individual level (prevention of bleeding) and a patient with stage I (no bleeding, platelet count above 50 times 10⁹/L) should be observed only.     

Treatment of autoimmune thrombocytopenic purpura with rhesus antibodies (anti-Rh0(D))

Summary There is evidence that blockade of the reticuloendothelial system (RES) by sequestration of autologous red blood cells (RBC) leads to an elevation of platelet counts in immune thrombocytopenia. To substantiate this hypothesis, 10 Rh₀(D)-positive adult patients (9 female, 1 male) with chronic autoimmune thrombocytopenic purpura (ITP) (1 to 21 years duration) were treated with low doses of intravenous IgG-anti-Rh₀(D) (200 to 1,000 g per dose; 300 to 3,600 g per course; administration within 1 to 5 days). All patients improved clinically as indicated by cessation of bleeding. In eight out of ten patients there was a rise in platelet count. Platelet increments were excellent (>100×10⁹/l) in one, good (50–100×10⁹/l) in three, fair (20–50×10⁹/l) in two and low (10–20×10⁹/l) in two patients. Splenectomized patients (N=4) had a poorer response than non-splenectomized patients (N=6) with mean increments of 16×10⁹/l (range 5–43×10⁹/l) versus 60×10⁹/l (range 10–110×10⁹/l). The increase in platelet counts persisted for seven to over 150 days. Transient and slight signs of haemolysis developed in seven out of ten patients (haemoglobin remained stable; increase of lactate dehydrogenase (>250 IU/l) in four, decrease of haptoglobin (<60 mg/dl) in five patients). The direct antiglobulin test became positive in all cases due to IgG1 without complement fixation. We conclude that the interaction of antibody-coated RBC with macrophages (and, probably, other means of RBC alteration) is a feasible therapeutic approach in selected cases of ITP and related conditions.     

Lower dose rituximab is active in adults patients with idiopathic thrombocytopenic purpura

Rituximab 375 mg/m(2) weekly for four weeks has significant activity in patients with immune thrombocytopenia. We evaluated the activity of lower dose rituximab (100 mg iv weekly for 4 weeks) in 28 adults with idiopathic thrombocytopenic purpura. Overall (platelet count > 50 x 10(9)/L) and complete responses (platelet count > 100 x 10(9)/L) were achieved in 21/28 (75%) and 12/28 (43%) patients respectively. The median time to response and time to complete response were 31 and 44 days respectively. After a median follow-up of 11 months (range 3-18), 7/21 (33%) patients relapsed and 3 needed further treatments. In patients with idiopathic thrombocytopenic purpura, lower dose rituximab seems to show similar activity to standard dose.     

Successful treatment of subdural hemorrhage and retinal hemorrhage in childhood-onset systemic lupus erythematosus associated with thrombocytopenia: Case report

Introduction:Thrombocytopenia (TP) is a common complication of childhood-onset systemic lupus erythematosus (SLE), and can range from mild to life-threatening. However, severe TP with multiple hemorrhagic complications is very rare and often predicts a poor prognosis. We describe a 12-year-old Chinese girl who had a history of idiopathic thrombocytopenic purpura who developed SLE that presented as subdural hemorrhage and retinal hemorrhage because of severe TP.Patient concerns:A 12-year-old girl was admitted into our hospital because of fever, purpura, and gum bleeding lasting for 12 days. She had a history of idiopathic thrombocytopenic purpura 2 years ago previously.Diagnosis:SLE was diagnosed according to American College of Rheumatology classification criteria. Subdural hemorrhage and retinal hemorrhage were diagnosed based on brain MRI and funduscopy. Severe TP was defined as platelet count <20 × 10⁹/L.Interventions:She was treated first with intravenous immunoglobulin, but it was not efficacious. High-dose methylprednisolone showed short-term efficacy. Then, she was given a glucocorticoid and cyclosporine A plus mycophenolate mofetil.Outcomes:Fever, purpura, and gum bleeding were resolved before hospital discharge. Subdural hemorrhage and left hemorrhagic retinopathy were improved remarkably. She had a durable response to refractory TP with no adverse effects during >1-year follow-up.Conclusion:Isolated TP may be an early symptom of childhood-onset SLE . A child with severe TP is prone to develop life-threatening hemorrhagic complications. Glucocorticoids and combined immunosuppressive drugs had a durable response to refractory TP in this patient with no adverse effects.