Occlusive Phlebitis Research Articles

Cocaine Abuse as an Immunological Trigger in a Case Diagnosed with Eales Disease

Background: Eales disease is a clinical syndrome affecting the mid-peripheral retina with an idiopathic occlusive vasculitis and possible subsequent retinal neovascularization. The disease can develop into visually threatening complications. Case Presentation: We report the case of a 40-year-old Caucasian male with a history of cocaine abuse who presented with blurred vision in the left eye (LE). Fundus examination showed vitreous hemorrhages, peripheral sheathing of venous blood vessels, areas of retinal neovascularization in the LE, and peripheral occlusive phlebitis in the right eye. The full serologic panel was negative except for the heterozygous mutation of factor V Leiden. Clinical and biochemical parameters suggested a diagnosis of Eales disease. Therapy with dexamethasone, 1 mg per kg per day, tapered down slowly over 4 months, and peripheral laser photocoagulation allowed a regression of clinical signs and symptoms. Conclusion: This case shows an uncommon presentation of Eales disease associated with cocaine abuse. Both cocaine abuse and a thrombophilic pattern, as cofactors, might have sensitized the retinal microcirculation on the pathogenetic route to this retinal pathology. Furthermore, in view of this hypothesis, a thorough ocular and general medical history investigating drug abuse and coagulation disorders is recommended for ophthalmologists in such cases.

Advances in IgG4-related sinonasal diseases

IgG4 related disease (IgG4-RD) is an independent clinical pathological entity which is different from common chronic inflammation and other autoimmune diseases in recent years. It often appears in the form of tumor like tissue-destructive lesions and may be accompanied by the increase of concentration of serum IgG4. Histopathology is characterized by a large number of lymphocytes and plasma cells infiltration, storiform fibrosis and occlusive phlebitis. The characteristics of IgG4-RD in nose and sinuses have not been widely investigated. The aim of the present study is to review the advances in IgG4 related sinonasal diseases from four aspects including pathogenesis, clinical features, treatment and future research directions.

Analysis of clinical diagnosis and treatment of IgG4 related pancreatitis and cholangitis

Objective To study the clinical features, experience of diagnosis and treatment in the IgG4 related pancreatitis and cholangitis. Methods Clinical date of sixteen cases of IgG4 related pancreatitis and cholangitis were analyzed as the observation group collecting from March 2006 to August 2016 of General Hospital of the Chinese People′s Liberation Army. The clinical symptoms, imaging features and pathological findings were studied. Ten cases of bile duct carcinoma or pancreatic cancer with normal IgG4 value and no related immunodeficiency disease were selected as control group during the same period. Serum IgG4 values of two groups were observed. The patients were followed up in outpatient department after their discharged. The serum bilirubin, liver enzymes, serum IgG4, CA19-9 and CEA antibodies were checked up every 6 months, including imaging studies. The longest follow-up period was 6 years. Measurement data with normal distribution was represented as (±s) and comparison between the two groups was analyzed using t test. The count data were described as n(%). Results In observation group, cases with jaundice accounted for 68.75% (11/16), abdominal pain and distension, loss of appetite and weight loss accounted for 43.75% (7/16), 62.50% (10/16) and 37.50% (6/16), respectively. Comparing different imaging methods, there were advantage in displaying the morphological signs of bile duct and pancreatic duct by MRCP. MRI was superior to the other methods in demonstrating pancreatic swelling and peripheral edema. Serum IgG4 value was an important basis for the diagnosis of the disease, the average value of serum IgG4 in the observation group was (105.7±21.6) g/L in 12 cases. The mean serum IgG4 level of 10 cases of control group (bile duct cancer and pancreatic cancer) was (9.1±0.05) g/L, There was significant difference between the two groups (P=0.004). The pathological features of IgG4 associated cholangitis were massive lymphocytes, plasma cell infiltration, bile duct fibrosis, occlusive phlebitis, and IgG4 positive plasma cell infiltration. The observation group was followed up for 16 cases, 12 cases survival, 4 cases lost, the longest follow-up period was 6 years. Except for 3 patients who suffered jaundice again during hormone therapy, all the clinical symptoms of the remaining patients disappeared, all the indexes were normal. Conclusions The diagnosis of IgG4 related pancreatitis and cholangitis should be carefully diagnosed, serum IgG4 examination is an important criterion. The image data are an important means to confirm the diagnosis. Surgical intervention should be performed in failure of the hormone treatment. Key words: Jaundice, obstructive; Cholangitis; Diagnosis; Treatment outcome

Immunoglobulin G4-related acquired hemophilia: A case report.

Acquired hemophilia A (AHA) is a relatively rare and life-threatening bleeding disorder whose pathogenesis is not completely understood. The present study reports a rare case of immunogubulin (IgG)4-related AHA with multisystemic involvement. A 55-year old male patient presented with symptoms of bronchial asthma and multiple subdermal hematomas. Chest computed tomography showed multiple diffuse nodular lesions with thickening of bronchovascular bundles, and scattered high-density spots in both lung lobes. Laboratory investigations showed increased activated partial prothrombin time (120.0 sec), a markedly decreased factor VIII (FVIII) activity (0.5%), a high-titer of FVIII inhibitor (27.2 Bethesda units/ml) and a marked increase in serum IgG4 (>4.03 g/l) level. Left inguinal lymph node biopsy revealed capsular thickening with marked lymphoplasmacytic infiltration, occlusive phlebitis and irregular fibrosis. Immunostaining revealed numerous IgG4-positive plasma cells (>100 cells/human plasma fibronectin) in the nodular lesions, with an IgG4/IgG ratio of >40%. The symptoms were markedly alleviated following corticosteroid therapy. The current study presents the first reported case of a rare IgG4-related AHA that presented with unusual clinical features and multisystemic involvement. The patient responded well to corticosteroid therapy. Documentation of such rare cases will help in characterizing the pathogenesis, and prompt recognition and timely treatment of this rare disorder.

Inflammatory pseudotumor of the liver in children: Report of cases and review of the literature

Two pediatric cases of inflammatory pseudotumor of liver were reported and compared with seven previously reported cases. Clinical presentation was variable but often consisted of fever and vague abdominal symptoms. These two cases were considered to be clinically malignant because angiographically one showed hypervascularity and the other showed the portal vein draining into the lesion to be occluded. There are four previous reports of similar cases complicated with occlusive phlebitis within and around the lesion. All but two of the reported cases underwent major hepatectomy or liver transplantation. However, because of the benign nature of the lesion, the treatment might be more conservative after adequate diagnostic procedures, including needle biopsy, are applied.

Inflammatory pseudotumor of the liver

The clinicopathologic features of a rare case of hepatic "inflammatory pseudotumor" are described and compared with those of three similar cases reported in the literature. In all four cases symptoms were systemic. The marked reduction in tumor size and the changing cellular composition, from a predominance of fibroblasts to a predominance of histiocytes, confirmed the reactive nature of the lesion in the present case. The etiology is obscure, and the significance of the associated occlusive phlebitis remains to be determined.