Cardiac tuberculoma presenting as thrombotic thrombocytopenic purpura-hemolytic uremic syndrome

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a unique multisystem syndrome. It can present with either chronic or subacute infections. Tuberculosis (TB) is a chronic infection that has been reported to present with TTP-HUS as tuberculous endocarditis in the presence of immunodeficiency and implanted medical devices in regions where TB is endemic. Tuberculomas are space occupying lesions most commonly found in the brain in immunocompromised individuals. Herein, we present a rare association of tuberculosis with endocarditis manifesting as a tuberculoma and presenting as TTP-HUS in an immunocompetent patient and resident of the United States.     

Comparison of low fixed dose versus standard-dose rituximab to treat thrombotic thrombocytopenic purpura in the acute phase and preemptively during remission

The standard dose of rituximab used in B-cell hematological malignancies, 375 mg/m² weekly, may be excessive for autoimmune conditions. Successful use of a low, fixed dose of 100−200 mg of rituximab, weekly for 4 weeks, has been reported in the literature in the treatment of autoimmune thrombotic thrombocytopenic purpura (aTTP). We retrospectively analyzed our rituximab data in aTTP over a 13-year-period for 39 patients, with the aim of comparing response and outcomes with a standard lymphoma-dose course versus a low fixed 100 mg-dose course. Compared to the standard dose (17 patients, 17 courses of 4 infusions), our patients who received a low dose (8 patients, 9 courses of 4 infusions) had a possibly lower baseline risk but did achieve a similar time to remission and number of plasma exchange procedures to remission. Preemptive low-dose courses for ADAMTS13 activity <50 % during remission (6 patients, 10 courses of 4 infusions) achieved a median peak ADAMTS13 activity of 99 %, in a median of 1 month, with no clinical relapses. Our results provide additional evidence for the efficacy of low-dose rituximab, with the benefit of much lower cost, less infusion time, and theoretically lower risk of toxicity.     

Postoperative thrombotic thrombocytopenic purpura following cardiovascular surgeries

Among 47 patients with thrombotic thrombocytopenic purpura (TTP), 8 patients were diagnosed to have postoperative-TTP. Two patients underwent vascular surgery, 5 patients coronary artery bypass grafts, and 1 patient resection of myocardial sarcoma. Prior to surgery, all patients except one had normal hemograms and platelet counts, and blood smears showed no schistocytes. Five to 19 days after surgery, all 8 patients developed postoperative TTP, which clinical feature was characterized by unexplained progressive encephalopathy, thrombocytopenia, and microangiopathic hemolytic anemia. In addition, in 3 patients, progressive gangrene of the toes also developed. Four patients achieved complete remission following exchange plasmapheresis and 1 patient spontaneous remission. Due to complicated surgical settings after surgery, recognition of TTP was often delayed and it contributed to death in 3 patients despite treatment with exchange plasmapheresis. In view of occurrence of postoperative TTP following cardiac and vascular surgeries, pathogenic mechanism for postoperative TTP may be explained on the basis of injury of diseased endothelial surface and release of a humoral factor(s) that results in platelet aggregation in the capillaries and arterioles. Our experience with these cases indicates that TTP may occur as a serious complication of cardiac and vascular surgeries, and early recognition of the diagnosis and institution of exchange plasmapheresis are of paramount importance for favorable outcome. © 1996 Wiley-Liss, Inc.     

Early treatment of rituximab combined with eltrombopag for secondary thrombocytopenic purpura in rheumatoid arthritis: A case report

Rationale:Secondary immune thrombocytopenic purpura (ITP) is also known as acquired thrombocytopenic purpura, autoimmune disease is usually one of the important causes. There are few reports about treatment of refractory thrombocytopenic purpura in rheumatoid arthritis (RA). We report a case of refractory ITP in which changes in platelet-related markers with therapeutic agents are worthy of the attention of clinicians.Patient concerns:A 69-year-old woman admitted for ecchymosis on the neck and arms for 15 days presented to our hospital. She was diagnosed with RA 5 years ago.Diagnosis:The diagnosis met the American College of Rheumatology/European League Against Rheumatism 2010 classification criteria. The disease activity score 28 (DAS-28) was 4.6, indicating that the disease activity was moderate.Interventions:Treatment with first-line therapies and second-line treatment--eltrombopag (EPAG) were ineffective. Therefore, we performed rituximab combined with a low dose of EPAG.Outcomes:The patient received 2 cycles of rituximab combined with EPAG, and reported no new petechiae on her buccal mucosa and limbs during follow-up.Lessons:This case suggests that early treatment of rituximab combined with EPAG is beneficial to patients with refractory ITP in RA. In terms of disease dynamic monitoring, immature platelet fraction (IPF) may be an auxiliary indicator for predicting efficacy, but its significance needs further study.     

Acquired Thrombotic Thrombocytopenic Purpura Following BNT162b2 mRNA Coronavirus Disease Vaccination in a Japanese Patient

A 57-year-old man without underlying diseases presented with fatigue, loss of appetite, and jaundice 1 week after receiving the first dose of the BNT162b2 mRNA coronavirus disease 2019 (COVID-19) vaccine and showed hemolytic anemia with fragmented erythrocytes and severe thrombocytopenia 2 weeks after receiving the vaccine. An a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) activity level of <10% and ADAMTS13 inhibitor positivity confirmed the diagnosis of acquired thrombotic thrombocytopenic purpura (TTP). Combination therapy with plasma exchange, corticosteroid, and rituximab improved the clinical outcome. We herein report the first Japanese case of TTP possibly associated with vaccination. Physicians should be alert for this rare but life-threatening hematological complication following COVID-19 vaccination.     

脾切除治疗特发性血小板减少性紫癜疗效及影响因素分析

目的 探讨特发性血小板减少性紫癜(idiopathic thromb-ocytopenic purpura,ITP)行脾切除治疗的效果并分析影响疗效的因素.方法 81例ITP行脾切除治疗,对其年龄、性别、术前对激素的反应、病程、术后血小板数量变化与脾切除疗效进行统计学分析.结果 81例中显效41例,良效27例,进步9例,无效4例,有效率(显效+良效)为84％.统计学分析显示术前对激素有反应者和术后血小板峰值较高者治疗效果较好(P＜0.05),其余各项指标差异均无统计学意义.结论 脾切除术对难治性的ITP患者安全有效,术前患者对激素治疗敏感程度可能对其手术效果的预判及指导临床治疗有一定的价值.     

COVID-19-induced immune thrombocytopenic purpura; Immunopathogenesis and clinical implications

Following the outbreak of the COVID-19 pandemic, millions of people around the world have been affected with SARS-CoV-2 infection. In addition to the typical symptoms, thrombotic events, lymphopenia, and thrombocytopenia have been reported in COVID-19 patients. Immune thrombocytopenic purpura (ITP) is one of the thrombotic events that occur in some COVID-19 patients. Hyperinflammation, cytokine storms, and immune dysregulation in some patients are the cause to the main COVID-19 complications such as ALI (acute lung injury), acute respiratory distress syndrome (ARDS), and multiple organ failure. Disruption in the differentiation of T-cells, enhanced differentiation of Th17 and Th1, cell death (pyroptosis), hyper-inflammation and dysfunction of inflammatory neutrophils and macrophages, and hyperactivity of NLRP3-inflammasome are among the important factors that may be the cause to COVID-19-induced ITP. This study aimed to give an overview of the findings on the immunopathogenesis of ITP and COVID-19-induced ITP. Further studies are required to better understand the exact immunopathogenesis and effective treatments for ITP, especially in inflammatory disorders.     

Different clinical severity of first episodes and recurrences of thrombotic thrombocytopenic purpura

The clinical course of thrombotic thrombocytopenic purpura (TTP) is characterized by recurrent disease episodes in up to 50% of cases. The clinical presentation and severity of different TTP episodes have not been systematically compared. Laboratory and clinical information from 51 patients with recurrent disease, derived from 136 patients with TTP included in the Milan TTP registry (URL: http://www.ttpdatabase.org), were used to compare mortality, symptoms and disease-related laboratory measurements in different disease episodes. The prevalence of severe neurological symptoms (coma, seizures, and focal neurological defects) was significantly lower in recurrences than in the first episode. Platelet counts and haemoglobin levels at presentation were higher in recurrences than in the first disease episode, and lactate dehydrogenase levels were lower. Also, mortality tended to be lower in the second and third disease episodes than in the first. Recurrences of TTP are generally milder than first episodes. These differences in severity should be taken into account in clinical research on TTP and in patient management.     

部分性脾动脉栓塞术治疗难治性血小板减少性紫癜并发脑出血3例体会

目的:探讨部分性脾动脉栓塞术(Partial splenic embolization,PSE)治疗难治性血小板减少性紫癜(Refractory purpura idiopathic thrombocytopenic,R-ITP)合并脑出血的临床应有价值。方法:对3例R-ITP合并脑出血患者进化部分性脾动脉栓塞术治疗,观察疗效。结果:3例均显效,随访至今无复发。并发症:3例均出现发热及穿刺处瘀斑,2例出现脾区疼痛及消化道反应。结论:部分性脾动脉栓塞术治疗R-ITP合并脑出血的重症患者疗效明显,且手术创伤小,安全性好,并发症少,值得临床应用推