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Blood Coagulation & Fibrinolysis : An... Jun 2024Immune-mediated thrombotic thrombocytopenia purpura (iTTP) is a rare microvascular disease characterized by severe disseminated microvascular thrombose-bleeding...
UNLABELLED
Immune-mediated thrombotic thrombocytopenia purpura (iTTP) is a rare microvascular disease characterized by severe disseminated microvascular thrombose-bleeding syndrome. Caplacizumab has been approved for the treatment of iTTP in combination with Plasma Exchange (PE) and immunosuppressive therapy, but its role in iTTP therapy remains uncertain. Therefore, we conducted a meta-analysis to investigate the safety and efficacy of caplacizumab for the treatment of patients with iTTP. We searched electronic databases (PubMed, Embase, Cochrane Library, and Scopus) and reference lists of relevant articles to find articles published from 2015 to 2022. The time to normalization of the platelet count of the group caplacizumab is shorter than the group placebo (SMD = -0.72; 95% CI -0.88 to -0.56; P < 0.05). Caplacizumab reduced the incidence of mortality (OR = 0.41; 95% CI 0.18-0.92; P < 0.05), exacerbations (OR = 0.10; 95% CI 0.05-0.18; P < 0.05), and recurrence (OR = 0.17; 95% CI 0.06-0.50; P < 0.05). However, the bleeding events in the caplacizumab group were higher than those in the placebo group, especially severe bleeding events. There was no difference in ADAMTS13 activity and thromboembolic events between the two groups. Our analysis indicated that caplacizumab is effective and well tolerated for the treatment of iTTP.
SYSTEMATIC REVIEW REGISTRATION
PROSPERO CRD42022362370.
PubMed: 38874905
DOI: 10.1097/MBC.0000000000001313 -
Cureus May 2024Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening condition characterized by fever, acute hemolysis, thrombocytopenia, renal...
Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening condition characterized by fever, acute hemolysis, thrombocytopenia, renal dysfunction, and CNS dysfunction. A peripheral smear shows schistocytes because of microangiopathy. It is extremely rare for TTP and systemic lupus erythematosus (SLE) to coexist. This report details an Indian female patient's uncommon clinical presentation of TTP linked to SLE. A 52-year-old woman presented to the emergency department with complaints of altered sensorium and weakness of the right side of the body. She was initially evaluated as a case of a cerebrovascular accident. CT brain imaging revealed multiple ischemic infarcts involving both cerebral hemispheres. MRI brain confirmed the same. During further evaluation, she was found to have hemolytic anemia, thrombocytopenia, and nephrotic range proteinuria. Immunological investigations confirmed SLE. A peripheral smear revealed schistocytes, and the PLASMIC score confirmed a high risk of TTP. The patient was treated with immunosuppressants, plasma exchange, and hemodialysis, along with other supportive measures. The patient showed a positive response to the therapy mentioned, with improved power and renal function. The patient denied a renal biopsy and was discharged after two weeks. This case report emphasizes the importance of the association between TTP and SLE.
PubMed: 38872677
DOI: 10.7759/cureus.60238 -
Cureus May 2024Thrombotic thrombocytopenic purpura (TTP) is clinical-pathological entity characterized by microangiopathic hemolytic anemia associated with end-organ...
Thrombotic thrombocytopenic purpura (TTP) is clinical-pathological entity characterized by microangiopathic hemolytic anemia associated with end-organ dysfunction. Traditionally, TTP was characterized by the classic pentad of fever, thrombocytopenia, hemolytic anemia, renal impairment, and neurological manifestations. However, this classic pentad is only observed in 40% of cases. We herein describe the case of a female patient who presented with epigastric pain and vomiting and found to have TTP without the classic pentad but with rapidly progressive renal dysfunction.
PubMed: 38872662
DOI: 10.7759/cureus.60259 -
Indian Pediatrics Jun 2024
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Child
PubMed: 38872289
DOI: No ID Found -
BMC Nephrology Jun 2024TAFRO syndrome is a rare disorder that causes thrombocytopenia, generalized oedema, fever, organ enlargement, and renal impairment. Few reports have suggested an...
BACKGROUND
TAFRO syndrome is a rare disorder that causes thrombocytopenia, generalized oedema, fever, organ enlargement, and renal impairment. Few reports have suggested an association with vaccines, and few cases have undergone renal biopsy. TAFRO syndrome is often severe and fatal, and its cause is unknown. We report a case of TAFRO syndrome that occurred after vaccination with the coronavirus disease 2019 (COVID-19) vaccine.
CASE PRESENTATION
An 82-year-old woman received two doses of the BNT162b2 mRNA vaccine 3 weeks apart. Two weeks later, she was admitted to the hospital with oedema, accompanied with renal failure and thrombocytopenia. After close examination, she was diagnosed with TAFRO syndrome. She was treated with steroids, cyclosporine, and thrombopoietin receptor agonists. The patient was discharged after several months in remission.
CONCLUSIONS
Although an incident of TAFRO syndrome after COVID-19 vaccination has been previously reported, this is a rare case in which the patient went into remission and was discharged. A renal biopsy was also performed in this case, which was consistent with previous reports. The favorable treatment course for TAFRO syndrome provides valuable insights.
Topics: Humans; Female; Cyclosporine; Aged, 80 and over; Thrombocytopenia; BNT162 Vaccine; Immunosuppressive Agents; COVID-19 Vaccines; Edema; COVID-19
PubMed: 38872134
DOI: 10.1186/s12882-024-03630-x -
Diving and Hyperbaric Medicine Jun 2024Hyperbaric oxygen treatment (HBOT) can be utilised for necrotising soft tissue infections, clostridial myonecrosis (gas gangrene), crush injuries, acute traumatic...
Hyperbaric oxygen treatment (HBOT) can be utilised for necrotising soft tissue infections, clostridial myonecrosis (gas gangrene), crush injuries, acute traumatic ischaemia, delayed wound healing, and compromised skin grafts. Our case was a 17-month-old male patient with Noonan syndrome, idiopathic thrombocytopenic purpura, and bilateral undescended testicles. Haematoma and oedema developed in the scrotum and penis the day after bilateral orchiopexy and circumcision. Ischaemic appearances were observed on the penile and scrotal skin on the second postoperative day. Enoxaparin sodium and fresh frozen plasma were started on the recommendation of haematology. Hyperbaric oxygen treatment was initiated considering the possibility of tissue necrosis. We observed rapid healing within five days. We present this case to emphasise that HBOT may be considered as an additional treatment option in patients with similar conditions. To our knowledge, no similar cases have been reported in the literature.
Topics: Humans; Male; Hyperbaric Oxygenation; Hematoma; Circumcision, Male; Noonan Syndrome; Infant; Orchiopexy; Cryptorchidism; Purpura, Thrombocytopenic, Idiopathic; Scrotum; Penile Diseases; Postoperative Complications; Enoxaparin; Plasma; Edema
PubMed: 38870956
DOI: 10.28920/dhm54.2.133-136 -
Clinical Practice and Cases in... May 2024A 21-year-old, otherwise healthy female presented to the emergency department with fever among other nonspecific symptoms after recently returning from Ghana. On...
CASE PRESENTATION
A 21-year-old, otherwise healthy female presented to the emergency department with fever among other nonspecific symptoms after recently returning from Ghana. On physical exam, she had a characteristic upper extremity rash, and a tourniquet test revealed numerous petechiae. The diagnosis of dengue was suspected and subsequently confirmed.
DISCUSSION
Dengue is one of many viral illnesses that should be considered in returning travelers presenting with fever and other nonspecific symptoms. Emergency physicians must keep a broad differential when evaluating fever in returned travelers and prioritize history and physical exam findings to help narrow the diagnosis and provide appropriate management and supportive care while awaiting further confirmatory testing.
PubMed: 38869348
DOI: 10.5811/cpcem.1911 -
Seminars in Thrombosis and Hemostasis Jun 2024Among the acquired coagulation factor deficiencies, autoimmune coagulation factor deficiencies (AiCFD) are rare and result from autoantibody production against...
Among the acquired coagulation factor deficiencies, autoimmune coagulation factor deficiencies (AiCFD) are rare and result from autoantibody production against coagulation factors. In Japan, a nationwide survey on AiCFD has been conducted since 2009. Autoimmune factor XIII, factor VIII, von Willebrand factor, factor V, and factor X deficiencies (AiF13D, AiF8D, AiVWFD, AiF5D, and AiF10D, respectively) have been enacted as "designated intractable disease-282." The incidence of AiF8D, AiF13D, and AiF5D was 1.83, 0.044, and 0.038 per million people/year, respectively, whereas that of AiVWFD and AiF10D was not calculable owing to the small number of patients. AiF13D and AiF8D were often idiopathic, whereas AiVWFD was often associated with plasma cell neoplasms. Epistaxis was a characteristic symptom of AiVWFD, intramuscular bleeding was frequent in AiF13D and AiF8D, and subcutaneous bleeding (purpura) was frequent in AiF13D and AiF10D, although none were specific to any one disease. Differential diagnosis cannot be made based on bleeding symptoms alone; therefore, rapid and accurate testing is mandatory. Definitive diagnosis of AiCFD necessitates identifying the presence of coagulation factor "inhibitors" and/or "autoantibodies." Therefore, these tests should be performed upon unexplained severe acquired coagulation factor deficiencies. The mainstay of treatment for AiCFD was hemostatic therapy and autoantibody eradication therapy, which included the replacement of coagulation factors or "bypass" agents and administration of immunosuppressants. The rate of hemorrhagic death was high in AiF13D (13%), followed by AiF5D (7%) and Ai10D (5%); therefore, early diagnosis and optimal treatment are essential for AiCFDs. Given the unknown long-term prognosis, "intractable disease platform registries" have begun to accumulate in Japan.
PubMed: 38866039
DOI: 10.1055/s-0044-1787188 -
La Tunisie Medicale Jun 2024Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal hematological disorder that requires urgent treatment. Once the diagnosis has been made, plasma...
INTRODUCTION
Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal hematological disorder that requires urgent treatment. Once the diagnosis has been made, plasma exchange (PE) must be started immediately and until a response is obtained.
AIM
Evaluate PE in terms of responses and complications in the treatment of TTP.
METHODS
This was a monocentric, descriptive, retrospective study including patients in whom TTP was diagnosed and treated with plasmapheresis in the clinical hematology department at Aziza Othmana Hospital, between January 2010 and December 2020.
RESULTS
Our study included 26 patients. PE was initiated within a median of 1 day. The rhythm of exchanges was daily in 22 patients. Twenty PE-related complications were noted, hypocalcemia being the most frequent (30%). CR was achieved in 15 patients after PE alone. Nine patients were refractory, and six received 2nd-line treatment, with CR achieved in five patients. Relapse was noted in six patients (40%). They were treated by PE and only one patient received rituximab. Four patients had a response. The overall response rate was 69% and overall mortality was 30%. OS at 2 years was 68,3% and RFS was 84,4%. Factors associated with the achievement of CR were the fall in LDH at D5 of treatment (p=0,027,OR=0,59 ;IC 95%[0,32-1,08]) and the daily rhythm of PE (p=0,005, OR=0,35; IC 95%[0,14-0,91]).
CONCLUSION
Our results were comparable to those of the literature, but the rate of refractory disease was higher. Rituximab may enhance our results.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Retrospective Studies; Plasma Exchange; Female; Male; Middle Aged; Adult; Young Adult; Treatment Outcome; Aged; Recurrence; Plasmapheresis; Adolescent; Rituximab
PubMed: 38864197
DOI: 10.62438/tunismed.v102i6.4614 -
Pediatrics International : Official... 2024Immumoglobulin A (IgA) vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP), is a self-limiting systemic vasculitis in children. Kidney involvement is...
BACKGROUND
Immumoglobulin A (IgA) vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP), is a self-limiting systemic vasculitis in children. Kidney involvement is associated with a long-term unfavorable outcome and can lead to significant morbidity. This study was conducted to describe the clinical and laboratory characteristics of childhood IgAV with kidney involvement and to identify risk factors associated with IgAV nephritis (IgAVN).
METHODS
This was an ambidirectional descriptive study of 77 children with IgAV. All demographic data, clinical features, and laboratory tests were collected from electronic medical records from January 2010 to December 2022. Risk factors for kidney involvement in IgAV were assessed using multivariate logistic regression. Kaplan-Meier survival analysis was used to calculate the time to commencement of kidney involvement.
RESULTS
Twenty-five children (32.4% of the IgAV patients) developed IgAVN. The common findings in IgAV with kidney involvement were microscopic hematuria (100%), nephrotic range proteinuria (44%), and non-nephrotic range proteinuria (40%). Multivariate logistic regression showed that age greater than 10 years (adjusted hazard ratio, AHR 4.66; 95% confidence interval, CI, 1.91-11.41; p = 0.001), obesity (body mass index, BMI, z-score ≥ +2 standard deviations, SDs) (AHR 3.59; 95% CI 1.41-9.17; p = 0.007), and hypertension at onset (AHR 4.78; 95% CI 1.76-12.95; p = 0.002) were associated significantly with kidney involvement. During follow up, most IgAV patients developed nephritis within the first 9 months.
CONCLUSION
Age greater than 10 years, obesity, and hypertension at presentation were predictive factors for IgAVN. Our study emphasized that IgAV patients with risk factors should be closely monitored for at least 1 year after the onset of the disease.
Topics: Humans; Male; Female; Child; Risk Factors; IgA Vasculitis; Child, Preschool; Adolescent; Retrospective Studies; Proteinuria; Kaplan-Meier Estimate; Hematuria; Logistic Models; Kidney; Glomerulonephritis, IGA
PubMed: 38863300
DOI: 10.1111/ped.15781