- Successful implementation of an automated electronic support system for patient safety monitoring: The alemtuzumab in multiple sclerosis safety systems (AMS3) study. [Journal Article]
- MSMult Scler 2018 Jun 01; :1352458518783673
- CONCLUSIONS: We successfully developed automated pathology monitoring with a CDSS, demonstrating real-world benefits of high compliance and timely alerting of important results.
- Onset of type 1 diabetes mellitus and heparin-induced thrombocytopenia in a patient with Basedow's disease and idiopathic thrombocytopenic purpura: Novel combination as autoimmune polyglandular syndrome. [Letter]
- JDJ Diabetes Investig 2018 Jun 15
- Pediatric thrombotic thrombocytopenic purpura. [Review]
- EJEur J Haematol 2018 Jun 11
- Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (...
Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)-cleavage protease. This deficiency may be either acquired (associated anti-ADAMTS13 autoantibodies) or congenital (resulting from biallelic mutations of ADAMTS13 gene). ADAMTS13 deficiency is responsible for the accumulation of high molecular weight multimers of VWF and the formation of platelet thrombi in the microcirculation. Consequently, microangiopathic hemolytic anemia and consumption thrombocytopenia are associated with organ ischemia. The differential diagnosis with other TMAs, autoimmune cytopenias or hematological malignancies may be challenging. The exploration of ADAMTS13 (activity, antibodies, antigen, ADAMTS13 gene) supports the diagnosis of TTP. The first-line treatment of the acute phase of TTP is based on plasmatherapy. In congenital TTP, patients with a chronic disease benefit from a prophylactic plasmatherapy. In autoimmune TTP, steroids and B-cells depleting therapies increasingly are used together with plasma exchange. Long-term follow-up including the monitoring of ADAMTS13 activity is mandatory. A severe decrease in ADAMTS13 activity (< 10%) may predict relapses and preemptive B-cell depletion with rituximab can be used to prevent relapses. This article is protected by copyright. All rights reserved.
- Idiopathic thrombocytopenic purpura in a patient with IgG4-related disease. [Letter]
- CEClin Exp Dermatol 2018 Jun 11
- [New aspects of immunotherapy in multiple sclerosis]. [Review]
- NNervenarzt 2018 Jun 07
- The spectrum of therapeutic options for immunotherapy of multiple sclerosis is continuously broadening. After the approval of cladribine and ocrelizumab in Europe, two new drugs are now available wit...
The spectrum of therapeutic options for immunotherapy of multiple sclerosis is continuously broadening. After the approval of cladribine and ocrelizumab in Europe, two new drugs are now available with ocrelizumab being the first approved option for treatment of primary progressive multiple sclerosis; however, the increased use of highly effective therapies is accompanied by a rise in severe side effects. During recent months, special attention was paid to the new progressive multifocal leukoencephalopathy (PML) risk assessment in natalizumab-treated patients, cardiac side effects of fingolimod, cases of idiopathic thrombocytopenic purpura and listeria meningitis associated with alemtuzumab and cases of daclizumab-treated patients with liver failure or encephalitis. These case reports highlight the importance of careful monitoring of all patients treated with immunomodulatory therapies.
- The diagnostic value of splenectomy in patients without a definitive preoperative diagnosis. [Journal Article]
- AJAm J Surg 2018 May 29
- CONCLUSIONS: The diagnostic ability of splenectomy was 46% when the diagnosis was unknown preoperatively. Additionally, a majority of patients experienced relief of symptoms postoperatively. Splenectomy may be a useful diagnostic and therapeutic tool in select UD and HM patients.
- Association between Helicobacter pylori infection and platelet count in mice. [Journal Article]
- EAExp Anim 2018 Jun 07
- Strong evidence for an association between idiopathic thrombocytopenic purpura (ITP) and Helicobacter pylori (HP) infection has been reported in humans. Chronic ITP is known to be improved by the era...
Strong evidence for an association between idiopathic thrombocytopenic purpura (ITP) and Helicobacter pylori (HP) infection has been reported in humans. Chronic ITP is known to be improved by the eradication of HP. The purpose of this study was to reproduce these events by the experimental infection of several strains of mice with HP. BALB/c, C57BL/6, and DBA/2 mice were untreated or orally inoculated with HP. Two months later, platelet counts were compared in samples from HP-infected and noninfected mice. Platelet counts (mean ± SD, × 104)/μl) in blood samples from HP-infected BALB/c, C57BL/6, and DBA/2 mice were 102.28 ± 14.71, 99.65 ± 17.00, and 111.57 ± 16.20, respectively; the respective counts from noninfected mice were 121.80 ± 13.30, 104.35 ± 18.20, and 107.84 ± 14.33. A significant difference in platelet counts between HP- infected and noninfected mice was observed in BALB/c mice (P≤0.01) but was not observed in DBA/2 mice, even though the histocompatibility (H)-2 type of the DBA/2 was the same as that of BALB/c mice. According to ELISA results, the optical density value for the anti-HP antibody in HP-infected BALB/c mice was not correlated with the number of platelets (P>0.50). These results suggest that the decrease in platelet count caused by HP infection is not related to antibody titer and histocompatibility-2 type. Experimental infection of BALB/c mice with HP can reproduce the relationship between HP and ITP and serves as a good model to investigate the mechanistic basis for the effectiveness of HP eradication therapy for ITP treatment.
- Two potentially lethal conditions of probable immune origin occurring in a pregnant woman: a case report. [Journal Article]
- JMJ Med Case Rep 2018 Jun 06; 12(1):158
- CONCLUSIONS: A possible autoimmune association might have caused both conditions in our patient. This case report serves as a warning message that pregnant women with one possible condition with autoimmune association could go on to develop other similar conditions.
- Is Helicobacter pylori infection the potential link between interleukin-17 and idiopathic thrombocytopenic purpura? [Journal Article]
- MMMinerva Med 2018 Jun 01
New Search Next
- Post-Partum Thrombotic Thrombocytopenic Purpura (TTP) in a Patient with known Idiopathic (Immune) Thrombocytopenic Purpura: a case report and review of the literature. [Journal Article]
- JMJ Med Case Rep 2018 Jun 01; 12(1):147
- CONCLUSIONS: Rituximab in conjunction with mycophenolate mofetil following plasma exchange therapy was effective in controlling the low platelet count in our patient. Only two doses of rituximab were sufficient to normalize our patient. We present here a case of safe and effective use of rituximab in pregnancy-induced thrombotic thrombocytopenia.