Cavitary lung lesion in a patient with Sjögren's syndrome (original) (raw)
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BJSTR, 2021
Background/Objective: Patients with thrombocytopenia accompanied with positive Ro/SS-A and/or La/SS-B autoantibodies have possible diagnosis of Sjögren's syndrome (SS). Because of its low prevalence, large-sample controlled studies on thrombocytopenia in primary SS (pSS) are scarce. Thus, this study aimed to investigate the clinical and laboratory characteristics of pSS complicated with mild to severe thrombocytopenia and compared them with pSS patients without thrombocytopenia. Methods: This medical records review study analyzed demographic data, clinical manifestations, laboratory examinations, and other results of 88 patients diagnosed with pSS between March 2007 and March 2018 in the Department of Rheumatology of the First Affiliated Hospital of Soochow University. Platelet count of peripheral blood below 50×109/L (≤50×10 9 /L) was regarded as mild to severe thrombocytopenia. Results: Of the 88 pSS patients, 43 developed mild to severe thrombocytopenia (thrombocytopenia group) and 45 had no thrombocytopenia (control group). No significant difference was found in the levels of autoantibodies and inflammatory markers between the thrombocytopenia group and the control group. Dry mouth (P < .01) and dry eyes (P < .01) were not frequently observed in the thrombocytopenia group, but the level of complement C4 dropped significantly (P < .05). In contrast, the thrombocytopenia group more likely had leukopenia (P = .01) and interstitial lung disease (P < .01). Conclusion: In pSS patients with mild to severe thrombocytopenia, the incidence of xerostomia, xerophthalmia, and lung involvement reduced markedly. Knowledge on the features of pSS associated with thrombocytopenia will lead to early and better diagnosis and treatment.
A Survey of 121 patients with Idiopathic Thrombocytopenic Purpura
International Journal of …, 2005
Introduction: Idiopathic Thrombocytopenic Purpura is one of the most common causes of thrombocytopenia with varius clinical courses and different responses to its treatment modalities. Therefore, we decided to evaluate the clinical course of this disease in our patients. Methods: In this case series study, from March 1998 to March 2004, we evaluated 121 patients. As a first line of treatment, all of those patients received prednisolone (1mg/kg/day) for 6 weeks. Those who didn't respond or were refractory to prednisolone were candidates for splenectomy, and those who refused these modalities or didn't respond to them were treated with Azathioprine. Results: 99 patients (81.8%) were female and 22 (18.2%) were male, aged 11-73 years (mean; 28.6). Two patients had immune hemolytic anemia in addition to thrombocytopenia, 4 had systemic lupus erythematosus, and one was HIV positive. %44.6 of patients fully responded to prednisolone, 42 patients (%34.7) underwent splenectomy and %83.3 of them responsed to it.31 splenectomies were performed in the first three months after diagnosis and 27 (87.1%) of them showed complete response, but 8 (72.7%) of the patients splenectomized after 3 months, had complete response. 14 patients were treated with Azathoprine, 2 of which (14.3%) platelet count normalized. Conclusion: ITP is more common in females than males. Prednisolone is preferred as a non-invasive treatment with suitable response in comparison to other modalition responses to splenectomy, indicating that this modality of treatment is useful and effective, especially when performaed in the first 3 months of the diseases.
American Journal of Hematology, 2001
The acute platelet response to Intravenous Gammaglobulin (IVIG) has been reported to predict response to subsequent splenectomy of patients with ITP. The current study was undertaken to determine if the platelet response to IV anti-D (Winrho-SDF) predicts response to subsequent splenectomy. The 61 HIV-uninfected children and adults in this study had taken part in the pre-licensing studies of IV anti-D and were all those who not only had evaluable platelet responses to IV anti-D but also had undergone splenectomy and had information available describing its 1-year outcome. Results of treatment with IVIG were available in 38 of these 61 patients. Neither response to the initial infusion of IV anti-D, nor response to the initial or last IVIG, predicted the response in either children or adults to subsequent splenectomy. However, response to the last anti-D infusion in adults was strongly correlated (P = 0.003) to response to subsequent splenectomy as was hemolysis ≥2.0 gm/dl after IV anti-D (P = 0.03). There was no overall relationship between response to IV anti-D or IVIG, and response to subsequent splenectomy. However, a good platelet response in adults to the last IV anti-D and a hemoglobin decrease ≥2.0 gm/dl both appeared to predict response to subsequent splenectomy. Am. J. Hematol. 67:27-33, 2001.
The Eurasian Journal of Medicine, 2010
Objective: Immune thrombocytopenic purpura (ITP) is also known as idiopathic thrombocytopenic purpura. Increased platelet destruction and insuffi cient platelet production are both responsible for its etiopathogenesis. ITP can be diagnosed after excluding other possible causes of thrombocytopenia. Materials and Methods: One hundred forty-three cases of chronic ITP that were monitored in a hematology clinic were retrospectively evaluated. All cases received fi rst line treatment of 1 mg/kg/day prednisolone. Corticosteroid nonresponsive (CN) cases and corticosteroid-dependent (CD) cases underwent splenectomies. Results: The rate of CN/CD cases was found to be 53% (n=76). Sixtysix percent of these cases (n=50) underwent splenectomies. The ratio of non-responsive cases to relapse cases after splenectomy (SN/SR) was 30% (n=15). The total number of cases was 41, including those without splenectomy (n=26) and with SY/SR (n=15). Helicobacter pylori (Hp) eradication, immunosuppressive agents and danazol treatments were administered to patients (n=10, n=14 and n=4, respectively). Currently, 13 patients are being monitored without treatment. Fifteen patients who were non-responsive to Hp eradication treatment, immunosuppressive treatment or danazol treatment are still being monitored without any treatment. Conclusion: Optimal treatment is not available for splenectomy-resistant cases of ITP. The response rates for Hp eradication treatment, immunosuppressive treatments and anabolic agents are low. Therefore, larger studies with more patients are required using new agents, such as thrombopoietin (TPO) receptor agonists and anti-CD20 monoclonal antibodies.
Further variant of the thrombocytopathy with abnormalities in platelet release reaction
Thrombosis Research, 1974
Multiple platelet abnormalities were found in a patient with bleeding symptoms. The platelet content of ADP and PF4 was decreased and the uptake of 14C-serotonin was impaired. The content of acid phosphatase, beta-qlucuronidase and beta-N-acetylglucosaminidase was, however, normal, and these enzymes were normally released or made available by bovine fibrinogen or ADP. There was no adhesion of platelets to collagen, which also failed to induce reptilase clot retraction, platelet aggregation and release of any of the platelet constituents. The platelets therefore exhibited siqns of thrombopathy of a combined type with a decreased storage pool as well as a qualitative dysfunction with impaired reactivity to collagen.
Blood, 1985
Wiskott-Aldrich syndrome (WAS) thrombocytopenia is frequently improved by splenectomy, although the mechanism of the thrombocytopenia and its resolution are unknown. Previous studies in two patients have shown that mean platelet volume, which is characteristically reduced in WAS, increased along with platelet count postsplenectomy. Additional studies in a limited number of patients have also demonstrated that platelet-associated immunoglobulin G (PAIgG) is elevated presplenectomy, but to date no postsplenectomy data have been reported. The present study was performed to more fully evaluate the effect of splenectomy on platelet volume and PAIgG in WAS. Before splenectomy, mean platelet volume was reduced but platelet size was broadly distributed with substantial overlap of the normal range. PAIgG was significantly elevated in 13 of 14 presplenectomy WAS patients (means = 78.9 fg per platelet) and fell to normal levels postoperatively (means = 4.0 fg per platelet). Platelet count and ...