- Strangles, convalescent Streptococcus equi subspecies equi M antibody titers, and presence of complications. [Journal Article]
- JVJ Vet Intern Med 2018 Dec 06
- CONCLUSIONS: This outbreak demonstrates that SeM antibody titers can be increased after infection (≥1:12 800) in the absence of complications of strangles.
- Henoch-schonlein Purpura Nephritis with Renal Interstitial Lesions. [Journal Article]
- OMOpen Med (Wars) 2018; 13:597-604
- CONCLUSIONS: Clinically, Type II is the most common cause; pathologically, Type IIIa is more common. The severity of renal tubulo-interstitial lesions is positively correlated with a decline in renal function and GFR. There is a correlation between the severity of renal tubulo-interstitial lesions and the severity of hematuria. Most patients with HSPN have a good prognosis.
- TTP: long-term outcomes following recovery. [Review]
- HAHematology Am Soc Hematol Educ Program 2018 Nov 30; 2018(1):548-552
- Although risk for relapse may be the greatest concern following recovery from acquired, autoimmune thrombotic thrombocytopenic purpura (TTP), there are multiple other major health issues that must be...
Although risk for relapse may be the greatest concern following recovery from acquired, autoimmune thrombotic thrombocytopenic purpura (TTP), there are multiple other major health issues that must be recognized and appropriately addressed. Depression may be the most common disorder following recovery from TTP and may be the most important issue for the patient's quality of life. Severe or moderate depression has occurred in 44% of Oklahoma Registry patients. Recognition of depression by routine screening evaluations is essential; treatment of depression is effective. Minor cognitive impairment is also common. The recognition that cognitive impairment is related to the preceding TTP can provide substantial emotional support for both the patient and her family. Because TTP commonly occurs in young black women, the frequency of systemic lupus erythematosus, as well as other autoimmune disorders, is increased. Because there is a recognized association of TTP with pregnancy, there is always concern for subsequent pregnancies. In the Oklahoma Registry experience, relapse has occurred in only 2 of 22 pregnancies (2 of 13 women). The frequency of new-onset hypertension is increased. The most striking evidence for the impact of morbidities following recovery from TTP is decreased survival. Among the 77 patients who survived their initial episode of TTP (1995-2017), 16 (21%) have subsequently died, all before their expected age of death (median difference, 22 years; range 4-55 years). The conclusion from these observations is clear. Following recovery from TTP, multiple health problems occur and survival is shortened. Therefore, careful continuing follow-up is essential.
- Beyond plasma exchange: novel therapies for thrombotic thrombocytopenic purpura. [Review]
- HAHematology Am Soc Hematol Educ Program 2018 Nov 30; 2018(1):539-547
- The advent of plasma exchange has dramatically changed the prognosis of acute thrombotic thrombocytopenic purpura (TTP). Recent insights into TTP pathogenesis have led to the development of novel the...
The advent of plasma exchange has dramatically changed the prognosis of acute thrombotic thrombocytopenic purpura (TTP). Recent insights into TTP pathogenesis have led to the development of novel therapies targeting pathogenic anti-ADAMTS13 antibody production, von Willebrand factor (VWF)-platelet interactions, and ADAMTS13 replacement. Retrospective and prospective studies have established the efficacy of rituximab as an adjunct to plasma exchange for patients with acute TTP, either upfront or for refractory disease. Relapse prevention is a major concern for survivors of acute TTP, and emerging data support the prophylactic use of rituximab in patients with persistent or recurrent ADAMTS13 deficiency in clinical remission. Capalcizumab, a nanobody directed against domain A1 of VWF that prevents the formation of VWF-platelet aggregates, recently completed phase 2 (TITAN) and 3 (HERCULES) trials with encouraging results. Compared with placebo, caplacizumab shortened the time to platelet recovery and may protect against microthrombotic tissue injury in the acute phase of TTP, though it does not modify the underlying immune response. Other promising therapies including plasma cell inhibitors (bortezomib), recombinant ADAMTS13, N-acetyl cysteine, and inhibitors of the VWF-glycoprotein Ib/IX interaction (anfibatide) are in development, and several of these agents are in prospective clinical studies to evaluate their efficacy and role in TTP. In the coming years, we are optimistic that novel therapies and international collaborative efforts will usher in even more effective, evidence-based approaches to address refractory acute TTP and relapse prevention.
- Clinical and laboratory diagnosis of TTP: an integrated approach. [Review]
- HAHematology Am Soc Hematol Educ Program 2018 Nov 30; 2018(1):530-538
- Thrombotic thrombocytopenia purpura (TTP) is a rare, life-threatening disease with an incidence of approximately 2 persons per million per year. It is characterized by severe deficiency of the von Wi...
Thrombotic thrombocytopenia purpura (TTP) is a rare, life-threatening disease with an incidence of approximately 2 persons per million per year. It is characterized by severe deficiency of the von Willebrand cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), leading to formation of platelet-rich thrombi in the microvasculature. Prompt initiation of appropriate therapy, particularly plasma exchange, may be life-saving. Diagnosis of TTP is challenging because of its diverse clinical manifestations, overlap in clinical presentation with other thrombotic microangiopathies, and limited availability of ADAMTS13 testing. Clinical prediction scores have been developed to estimate the pretest probability of severe ADAMTS13 deficiency and may be used as an adjunct to clinical judgment to guide initial management decisions. An ADAMTS13 activity level of less than 10% supports the diagnosis of TTP in appropriate clinical contexts, but many centers do not offer testing in-house and must send out the test to a reference laboratory with a turnaround time of several days. In such instances, initial management decisions must be made without the benefit of laboratory testing. In patients with TTP, inhibitor tests may be useful for distinguishing immune-mediated from congenital TTP. In this article, we review the epidemiology, natural history, and clinical presentation of TTP and laboratory assays for TTP including ADAMTS13 activity and inhibitor assays. We also describe an evidence-based approach to the evaluation of a patient with suspected TTP that integrates clinical and laboratory assessment.
- Causal relationship between immunological responses and adverse reactions following vaccination. [Review]
- VVaccine 2018 Nov 29
- Vaccine adverse events and controversial safety issues have occurred in recent decades in Japan: aseptic meningitis following the measles-mumps-rubella combined vaccine (MMR), anaphylaxis after immun...
Vaccine adverse events and controversial safety issues have occurred in recent decades in Japan: aseptic meningitis following the measles-mumps-rubella combined vaccine (MMR), anaphylaxis after immunization with live virus vaccines and inactivated split influenza vaccine, an increased incidence of febrile illness following the simultaneous administration of inactivated vaccines, and chronic pain with neurological illness after immunization with the human papilloma virus vaccine (HPV). Vaccine adverse events are a matter of concern for the public as well as general practitioners; some are within the range of assumptions that adverse reactions after live attenuated vaccines are related to the nature of their parental wild-type viruses. Vaccines stimulate the innate immunity of host immunological defense mechanisms and induce the development of specific acquired immunity. Some adverse events related to autoimmune responses have been reported, such as idiopathic thrombocytopenic purpura and acute disseminated encephalomyelitis (ADEM). Although a plausible relationship was not demonstrated, the possibility of an association cannot be denied. The pathogenicity of adverse reactions was investigated for anaphylactic reactions, systemic and local reactions following vaccinations. Initial innate immune responses are essential for the development of acquired immunity and are related to adverse events from different viewpoints.
- [Expression and Clinical Significance of Peripheral Blood T cell JAK2/STAT3 mRNA in Chronic Idiopathic Thrombocytopenic Purpura]. [Journal Article]
- ZSZhongguo Shi Yan Xue Ye Xue Za Zhi 2018; 26(6):1746-1751
- CONCLUSIONS: The expression level of JAK2/STAT3 mRNA increases signficanlty in chronic ITP patients, which involves in pathogenesis of CITP.
- Vasculitis-What Do We Have to Know? A Review of Literature. [Journal Article]
- IJInt J Low Extrem Wounds 2018 Dec 03; :1534734618804982
- Cutaneous and other vasculitides are specific inflammations of the blood vessel wall that can take place in any organ system of the body including the skin. Vasculitis has been traditionally divided ...
Cutaneous and other vasculitides are specific inflammations of the blood vessel wall that can take place in any organ system of the body including the skin. Vasculitis has been traditionally divided according to the size of the vessel involved (small, medium, and large). Vasculitis is more of a reaction pattern rather than a specific disease entity. Therefore, the clinical presentation of vasculitis (most commonly palpable purpura on the lower extremities) dictates a thorough history, review of systems, and a meticulous physical examination. The diagnosis of vasculitis relies also on the histopathological and immunofluorescence studies. Wound care specialist may face with vasculitis-associated ulcers along with a spectrum of other cutaneous presentations associated with vasculitis. The focus of this article is to update the types, etiology, pathogenesis, and management options for cutaneous vasculitis.
- [Effect of miR-21 on the expression of interleukin-10 in B cell of patients with Henoch-Schonlein purpura]. [Journal Article]
- ZEZhonghua Er Ke Za Zhi 2018 Dec 02; 56(12):939-944
- Objective: To investigate the effect of microRNAs (miR)-21 on the expression of interleukin (IL)-10 in B cell of patients with Henoch-Schonlein purpura (HSP). Methods: From March 2016 to January 20...
Objective: To investigate the effect of microRNAs (miR)-21 on the expression of interleukin (IL)-10 in B cell of patients with Henoch-Schonlein purpura (HSP). Methods: From March 2016 to January 2017, twenty-four children with HSP hospitalized in rheumatology and immunology department of Shenzhen Children's Hospital were enrolled into the study, including 12 males and 12 females. Patients were divided into purpura nephritis group (HSPN, 14 cases) and non-nephritis group (NHSPN, 10 cases). The age-matched 34 healthy children were included as the control group for prospective cohort study. The expression levels of IL-10 in peripheral blood B cells (CD19(+)), transitional B cells (CD19(+) CD24(hi)CD38(hi)) and naïve B cells (CD19(+)CD24(int)CD38(int)) from patients with HSP and healthy children were detected by flow cytometry (FCM). Expression of microRNAs related to IL-10 in B cells were quantitated by real-time PCR, including miR-21-5p, miR-106a-5p, miR-98-3p, miR-142-3p, miR-142-5p, miR-98-5p, miR-155-5p and miR-let7b-5p. Agomir negative control-FAM and agomir-21-5p-FAM were transfected into B cells from patients with HSP. The uptake of miRNA by B cells was observed by laser scanning confocal microscope and FCM, and the expression of IL-10 was detected by FCM after transfection. For quantitative data of normal distribution, t test was used for two samples comparison and multiple comparisons among three groups were conducted by ANOVA. Spearman test was used for correlation analysis. Results: (1) The CD19(+) B cells and its two populations at different differentiation stages all could express IL-10. The expression levels of IL-10 in three B cell populations in patients were significantly lower than those in healthy controls (1.4±0.2 vs. 2.4±0.3, t=3.501, P<0.01; 1.2±0.2 vs. 2.2±0.3, t=2.688, P<0.05; 1.6±0.3 vs. 2.7±0.4, t=2.498, P<0.05). Compared with healthy control and NHSPN groups, the expression of IL-10 in CD19(+) B cells from patients within HSPN group was the lowest, and the difference was statistically significant (1.1±0.2 vs. 2.4±0.3, 1.8±0.3, t=4.006, 2.362, P<0.001, P<0.05). (2) The expression of miR-21-5p in B cell in patients with HSPN was lower than that in healthy control group (1.2±0.9 vs. 3.5±2.8, t=2.962, P<0.01). There was no significant change in the other microRNAs. (3) The expression of IL-10 was positively correlated with the expression of miR-21-5p in the B cells of patients with HSP (r=0.778, P<0.001). (4) The expression of IL-10 in B cells of miR-21-5p group was significantly higher than that in negative control group (2.7±0.2 vs. 1.6±0.3, t=3.091, P<0.05). Conclusion: The insufficient expression of miR-21-5p in peripheral blood B cells of patients with HSP is one of the reasons for the reduction of IL-10 expression in B cells.
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- Combination peptide immunotherapy suppresses antibody and helper T cell responses to the major human platelet autoantigen GPIIb/IIIa in HLA-transgenic mice. [Journal Article]
- HHaematologica 2018 Dec 04
- Platelet destruction in immune thrombocytopenia is caused by autoreactive antibody and T cell responses, most commonly directed against platelet glycoprotein IIb/IIIa. Loss of self-tolerance in the d...
Platelet destruction in immune thrombocytopenia is caused by autoreactive antibody and T cell responses, most commonly directed against platelet glycoprotein IIb/IIIa. Loss of self-tolerance in the disease is also associated with deficient activity of regulatory T cells. Having previously mapped seven major epitopes on platelet glycoprotein IIIa that are recognised by helper T cells from patients with immune thrombocytopenia, the aim was to test whether peptide therapy with any of these sequences, alone or in combination, could inhibit responses to the antigen in humanized mice expressing HLA-DR15. None of the individual peptides, delivered by a putative tolerogenic regimen, consistently suppressed the antibody response to subsequent immunization with human platelet glycoprotein IIb/IIIa. However, the combination of GPIIIa peptides aa6-20 and aa711-725, which contain the predominant helper epitopes in patients and elicited the strongest trends to suppress when used individually, did abrogate this response. The peptide combination also blunted, but did not reverse, the ongoing antibody response when given after immunization. Suppression of antibody was associated with reduced splenocyte T cell responsiveness to the antigen, and with the induction of a regulatory T cell population that is more responsive to the peptides than to purified platelet glycoprotein IIb/IIIa. Overall, these data demonstrate that combinations of peptides containing helper epitopes, such as platelet glycoprotein IIIa aa6-20 and aa711-725, can promote in vivo suppression of responses to the major antigen implicated in immune thrombocytopenia. The approach offers a promising therapeutic option to boost T cell regulation, which should be taken forward to clinical trials.