Acquired Thrombotic Thrombocytopenic Purpura (TTP) Presenting With Synthetic Cannabinoid Use.

Synthetic cannabinoids (SCs) have become commercially available throughout the United States as manufacturers circumvent regulations with labels stating "not for human consumption" with misleading advertisements, resulting in the consumption of products that are not safe or regulated. We present a case report of a middle-aged woman exhibiting altered mental status secondary to SC use who was found to have severe thrombocytopenia and hemolytic anemia. She was later confirmed to have thrombotic thrombocytopenic purpura (TTP) through ADAMTS13 testing. TTP is one of several platelet-related disorders presenting with findings of hemolytic anemia and thrombocytopenia. The presence of altered mental status is typically used as a symptomatic differentiator between hemolytic uremic syndrome, immune thrombocytopenic purpura, and TTP. SCs can cause superimposed altered mental status, which, in the setting of a concomitant platelet disorder, can complicate the standard workup and prolong the time to a final diagnosis. This case serves as an essential reminder that collecting detailed social history and promptly recognizing laboratory abnormalities is critical for early recognition of TTP, as the diagnosis is time-sensitive and delays in recognition can lead to significant morbidity and mortality.

VE-Cadherin is involved in endothelial cell detachment during Thrombotic Thrombocytopenic Purpura.

BACKGROUND AND OBJECTIVES: Immune Thrombotic thrombocytopenic purpura (i-TTP) is a life-threatening thrombotic microangiopathie linked to ADAMTS13 deficiency. It has long been assumed that the activation of endothelial cells is the triggering factor for the TTP crisis. Circulating endothelial cells (CEC) have been shown to be a biomarker of vascular damage and are associated with the clinical severity of i-TTP. However, the mechanisms leading to endothelial cell detachment remain unclear. OBJECTIVES: We have investigated junctional destabilization and the mechanisms underlying cell detachment in TTP. METHODS AND RESULTS: In plasma from i-TTP patients, we show that CEC count is associated with severity and correlated to intracellular calcium influx (p<0.01). In vitro, serum from i-TTP patients induced stronger detachment of human umbilical vein endothelial cells (HUVECs) than serum from control patients (p<0.001). Plasma from i-TTP patients induced a higher calcium-dependent phosphorylation (p<0.05) and internalization (p<0.05) of VE-cadherin compared to plasma from control patients. This effect could be reproduced by IgG fraction isolated from patient plasma and in particular by the F(ab)'2 fragments of the corresponding IgG. In addition, subcutaneous injection of i-TTP plasma into mice resulted in higher vascular permeability than plasma from control patients. An inhibitor of endothelial calcium influx, ITF1697, normalized this increase in permeability. CONCLUSIONS: Our results suggest that plasma-induced endothelial activation also leads to an increase in vascular permeability. They contribute to the understanding of the mechanisms behind the presence of elevated CECs in patients' blood by linking endothelial activation to endothelial injury.

Rapid, Simple and Low-Cost Analytical Method Development for Quantification of Eltrombopag Olamine in Tablet Dosage by UV Spectroscopy Method.

BACKGROUND: Eltrombopag Olamine is a drug used to treat thrombocytopenia, a disorder where blood platelet counts get lower and severe aplastic anemia. It serves as a thrombopoietin receptor agonist, which give rise to platelet production in the bone marrow. OBJECTIVES: The objective of this study is to develop a simple, specific, accurate, precise and economical Ultraviolet spectroscopy method to estimate the amount of Eltrombopag Olamine in bulk and tablet dosage form. METHODS: The developed method was performed using methanol for identification and physicochemical characterization of the drug. The validation parameters like linearity, precision, accuracy, robustness limits of detection and quantitation, and specificity were assessed as per ICH Q2 (R2). RESULTS: The maximum absorbance wavelength (λmax) of the drug was found at 247 nm in methanol. The linearity was found in the concentration range of 2-14 µg/ml with regression equation y = 0.0619x - 0.0123 and r² = 0.999. The standard addition method was used to determine the accuracy of the developed method. The result was found in the % recovery range of 98-99%. The precision was done on λmax with respect to the parameters such as repeatability, intraday, and interday. The method was found to be precise as the % RSD value was found to be <2%. The detection limit value (LOD) and quantitation limit value (LOQ) were 0.0524 µg/ml and 0.1588 µg/ml, respectively. CONCLUSION: The developed method is simple, economical, accurate and selective. The developed method was adaptable for the estimation of Eltrombopag Olamine analysis in pharmaceutical dosage form and routine quality control laboratory.

Acute hemorrhagic edema of infancy: 20-year experience from an Italian tertiary referral center.

PURPOSE: Data on acute hemorrhagic edema of infancy (AHEI) are derived from small case series or case reports. We report a 20-year experience at a national referral center. METHODS: We performed a single-center retrospective study including patients who were diagnosed with AHEI from January 1, 2004, to June 30, 2023. RESULTS: We identified 21 patients (57.1% females) with a median age of 18 months (range 7-33 months). Thirteen (61.9%) patients were admitted to the pediatric ward, the remaining eight (38.1%) presented to the emergency department and were discharged for outpatient management. The median length of hospitalization was 5 days (range 3-9 days). Twenty patients (95.2%) had prodromal symptoms. The most common cutaneous findings were targetoid purpuric plaques. The lesions were most localized on the face (13, 61.9%) and on the upper limbs (18 patients, 85.7%). Sixteen (76%) patients presented with nonpitting and tender edema, localized on the feet (9/16, 56%) and hands (6/16, 37.5%). Systemic involvement was rare, and no patients experienced complications or sequelae. Twelve (57.1%) patients underwent infectious disease investigations, with positive results in only four (33.3%). None of the patients diagnosed after the SARS-CoV-2 outbreak (March 2020) had positive nasopharyngeal swabs for the virus. For the 13 patients who were admitted to the pediatric ward, the median length of hospitalization was five days (3-9 days). CONCLUSIONS: The 21-patient single-center cohort of children affected by AHEI confirmed a generally benign course of AHEI, despite a 62% rate of hospitalization.

The Clinical Effect and Safety of Dihuang Decoction in Henoch-Schönlein Purpura Compared With Different Traditional Programs: A Network Meta-Analysis.

This systematic review aims to evaluate the therapeutic efficacy of Dihuang decoction (DD), anti-inflammatory drugs (AIDs), blood circulation improvement drugs (BCIDs), and conventional therapy (CT) in the management of Henoch-Schönlein purpura (HSP) and to establish their comparative effectiveness rankings. Using the Population, Intervention, Comparison, Outcome, Study (PICOS) design framework, we developed a detailed search strategy. The literature search included databases such as PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, Wanfang Data, Weipu Journal Data, and the Chinese Biomedical Database, covering studies published up to June 2024. We included randomized controlled trials that featured the DD as the experimental intervention, with three remaining treatments as comparators. Our analysis encompassed 63 studies with 5,435 participants, divided into 2,817 in the experimental group and 2,618 in the control group. The network meta-analysis suggested that the DD potentially surpasses AIDs, BCIDs, and CT in the management of HSP. This conclusion is supported by its superior SUCRA (Surface Under the Cumulative Ranking) scores across various measures, including the overall effective rate of medication, time to relief or disappearance of the rash, incidence of adverse reactions, time to relief or disappearance of abdominal pain, time to relief or disappearance of arthritic swelling or pain, IgA levels, and the relapse rate within six months (SUCRA scores: 100.0%, 88.3%, 79.8%, 94.4%, 99.9%, 88.3%, and 95.4%, respectively). In terms of overall effectiveness rate, the SUCRA efficacy rankings are as follows: DD > AIDs > AIDs+BCID > BCID > CT. Regarding rash relief and regression time, the SUCRA efficacy rankings are as follows: DD > CT > AIDs+BCID > AIDs. For the incidence rate of adverse reactions, the SUCRA efficacy rankings are as follows: DD > CT > AIDs > BCID > AIDs+BCID. For the relief and disappearance of abdominal pain, the SUCRA efficacy rankings are as follows: DD > CT > AIDs+BCID > AIDs. In terms of relief and disappearance of joint swelling and pain, the SUCRA efficacy rankings are as follows: DD > AIDs+BCID > AIDs. Regarding IgA changes, the SUCRA efficacy rankings are as follows: DD > CT > BCID > AIDs+BCID > AIDs. For the six-month recurrence rate, the SUCRA efficacy rankings are as follows: DD > AIDs > AIDs+BCID > CT. The DD appears to be a more effective alternative for treating HSP compared to AIDs, BCIDs, and CT. We hope that this study will provide better assistance to clinical practice.

Eosinophilic granulomatosis with polyangiitis developed during treatment with benralizmab for severe asthma: A case report and literature review.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune disorder characterized by necrotizing vasculitis, asthma, and eosinophilia. We report a case of EGPA that developed during benralizumab treatment for severe asthma and provide a literature review. A 79-year-old Japanese male with severe asthma presented with generalized purpura 4 months after initiating benralizumab treatment. He had reduced his oral prednisolone dose from 7.5 to 2 mg/day. Laboratory tests revealed eosinophilia, and skin biopsy showed vasculitis with eosinophilic infiltration. He was diagnosed with EGPA and treated with corticosteroids, azathioprine, and mepolizumab, which led to rapid improvement and sustained remission. Five cases of EGPA developing during benralizumab treatment have been reported, with onset ranging from 14 to 36 weeks after initiation. Clinicians should monitor for EGPA development in patients receiving benralizumab, particularly during oral corticosteroid reduction.

Safety and efficacy of danazol in immune thrombocytopenia: a systematic review.

Background: The treatment landscape for relapsed or refractory immune thrombocytopenia (ITP) after corticosteroids is complex. Objectives: We aimed to assess the efficacy of danazol in treating ITP and evaluate the safety and adverse events following its administration. Methods: We searched the databases PubMed, EMBASE, and ClinicalTrials.gov for all published studies assessing danazol's efficacy and safety in treating ITP. The retrieved studies were screened by title and abstract, followed by full-text screening based on the eligibility requirements. The quality assessment was performed using a set of questionnaires. The data were extracted on the descriptive characteristics of the studies and participants, drug dosage, efficacy measures, and adverse effects, and the data were synthesized. Results: A total of 17 studies consisting of 901 participants were included. The overall response rate is around 61% in this analysis. Among the participants, 315 (34.9%) were men. The age of participants ranged from 16 to 86 years. Danazol combined with other pharmacologic interventions, including all-trans-retinoic acid or glucocorticoids, generated better results. The most common side effects appear to be liver injury and elevation of liver enzymes, weight gain, oligomenorrhea, amenorrhea, and myalgia. Conclusion: Danazol at low-to-medium doses was well tolerated and succeeded in improving ITP. Danazol therapy may be helpful in the treatment of chronic ITP that is corticosteroid refractory and when corticosteroids or splenectomy (or both) is contraindicated. Danazol can be considered for further research and development in treating primary immune thrombocytopenia.

Immune Thrombocytopenic Purpura and Ganglionic Tuberculosis: An Unlikely Link.

Tuberculosis (TB) is one of the leading infectious causes of morbidity and mortality worldwide. Either in its pulmonary (PTB) or extrapulmonary forms (EPTB), TB has a wide variety of manifestations, including hematological ones like thrombocytosis (especially in PTB) and thrombocytopenia (mainly with disseminated or miliary TB). Hematological manifestations are infrequently presenting features of TB, and within them, immune thrombocytopenic purpura (ITP)-associated TB is one of the rarest presenting features. We report a case of a 22-year-old woman with a diagnosis of ganglionic tuberculosis (GTB) presenting with ITP. The therapeutic approach was challenging and included the use, originally, of intravenous immunoglobulin 30 mg/day for five days and, posteriorly, of high-dose corticosteroids (dexamethasone 40 mg/day) and anti-tubercular therapy with satisfactory outcomes.

Clinical and economic burden of suspected thrombotic thrombocytopenic purpura in US hospitals.

DISCLAIMER: In an effort to expedite the publication of articles, AJHP is posting manuscripts online as soon as possible after acceptance. Accepted manuscripts have been peer-reviewed and copyedited, but are posted online before technical formatting and author proofing. These manuscripts are not the final version of record and will be replaced with the final article (formatted per AJHP style and proofed by the authors) at a later time. PURPOSE: Severe ADAMTS13 deficiency (activity <10%) is the diagnostic threshold for thrombotic thrombocytopenic purpura (TTP) and is associated with various clinical symptoms, abnormal laboratory results, and long-term complications. METHODS: This retrospective, noninterventional cohort study used the Premier Healthcare Database to identify patients with ADAMTS13 activity of <10% in US hospitals from January 1, 2016, through March 31, 2020. The objective was to describe patient characteristics, laboratory results, comorbidities (as measured by the Elixhauser comorbidity index), symptoms, length of stay, treatment patterns, mortality, inpatient costs, and readmission rates (summarized descriptively). Inpatient costs were calculated as total cost to the hospital. RESULTS: There were 211 patients with severe ADAMTS13 deficiency; 89% of patients had a TTP-related diagnosis, of whom 62% had a primary diagnosis of thrombotic microangiopathy. Over 80% of patients with available data had a decreased platelet count and elevated lactate dehydrogenase; schistocytes were detected in 99%. The most prevalent symptoms/complications were neurological, bleeding, and pain. Most patients (86%) had 2 or more Elixhauser comorbidities. Over 80% of patients received 1 or more TTP-related treatments, mostly plasma exchange. The mean length of stay was 11.5 days; 5% of patients died during their stay. Readmission rates at 30, 60, and 90 days were 20%, 26%, and 28%, respectively. The median (interquartile range) total inpatient cost to the hospital throughout the index admission was $33,221 ($19,431-$64,901). CONCLUSION: Patients with severe ADAMTS13 deficiency have substantial clinical burden, have high mortality and readmission rates, and generate high costs for hospitals. There is a high need for a therapy that replaces ADAMTS13, thus addressing the root cause of the symptoms and complications caused by this deficiency.

Primary immune thrombocytopenic purpura with exuberant oral manifestations in a patient with Turner syndrome.

BACKGROUND: Primary Immune Thrombocytopenic Purpura (ITP) is an autoimmune hematological condition characterized by isolated thrombocytopenia and frequently presents with oral manifestations. However, reports of primary ITP in patients with Turner Syndrome (TS) are exceptionally rare, with few cases documented in the literature. Herein, we describe an unusual case of primary ITP with exuberant oral manifestations in a patient with TS. CASE REPORT: A 29-year-old woman was referred to an oral diagnostic service with complaints of "blood blisters and gum bleeding" lasting 8 h. On extraoral physical examination, multiple petechiae were observed in the upper and lower limbs, in addition to hemorrhagic extravasation in the right ocular sclera (hyposphagma). On intraoral examination, multiple vesicles and blisters filled with blood were identified on the lower lip, back of the tongue, and buccal mucosa, along with spontaneous gingival bleeding and hemorrhagic petechiae on the palate. Laboratory tests revealed thrombocytopenia (5000/mm3), whereas the blood count showed normality in the red and white series. After excluding other etiological factors or associated diseases, the patient was diagnosed with severe ITP and began treatment with systemic corticosteroids in the intensive care unit, resulting in a successful increase in platelets. After a 2-year follow-up, the patient remains free of ITP recurrences. CONCLUSION: Oral manifestations may be one of the first signs of ITP. Therefore, it is essential that dentists are familiar with the condition and, when faced with unusual oral bleeding, consider the possibility of a hematological disorder such as ITP, ensuring a correct and early diagnosis. Moreover, the presence of ITP can further exacerbate complications associated with TS. Therefore, rigorous follow-up of these patients is crucial, considering the high incidence of cardiovascular and autoimmune diseases and the reduced life expectancy of these patients.

Immune Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, Therapy and Open Issues.

Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and ischemic end-organ injury due to microvascular platelet-rich thrombi. iTTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (vWF)-cleaving protease, due to anti-ADAMTS13 autoantibodies. Early diagnosis and treatment reduce the mortality. Frontline therapy includes daily plasma exchange (PEX) with fresh frozen plasma replacement and immunosuppression with corticosteroids. Caplacizumab has recently been added to frontline therapy. Caplacizumab is a nanobody that binds to the A1 domain of vWF, blocking the interaction of ultra-large vWF multimers with the platelet and thereby preventing the formation of platelet-rich thrombi. Caplacizumab reduces mortality due to ischemic events, refractoriness, and exacerbations after PEX discontinuation. Until now, the criteria for response to treatment mainly took into account the normalization of platelet count and discontinuation of PEX; with the use of caplacizumab leading to rapid normalization of platelet count, it has been necessary to redefine the response criteria, taking into account also the underlying autoimmune disease. Monitoring of ADAMTS13 activity is important to identify cases with a low value of activity (<10IU/L), requiring the optimization of immunosuppressive therapy with the addition of Rituximab. Rituximab is effective in patients with refractory disease or relapsing disease. Currently, the use of Rituximab has expanded, both in frontline treatment and during follow-up, as a pre-emptive approach. Some patients do not achieve ADAMTS13 remission following the acute phase despite steroids and rituximab treatment, requiring an individualized immunosuppressive approach to prevent clinical relapse. In iTTP, there is an increased risk of venous thrombotic events (VTEs) as well as arterial thrombotic events, and most occur after platelet normalization. Until now, there has been no consensus on the use of pharmacological thromboprophylaxis in patients on caplacizumab because the drug is known to increase bleeding risk.

A Case Series of Unusual IgA Vasculitis.

Introduction: Immunoglobulin A vasculitis (IgA)is a rare condition characterized by palpable purpura, often involving the skin, gastrointestinal tract, joints, and kidneys. Presentation is usually acute and is more common in children and adolescents of Southeast Asian and European descent. In the adult population, it is less common and therapies are not as well-established. Case Presentation: Disease prevalence of IgA vasculitis outside Southeast Asian and European populations is not well-documented. In this case series, we present 2 cases of IgA vasculitis in 2 older adult males, one of Native American descent and one of African American descent. Conclusion: IgA vasculitis must be considered within the adult population, and it is not limited to certain ethnic groups. Further research is needed to give clarity on the best treatment options for adults with IgA vasculitis. We believe that patients presenting with IgA vasculitis are best managed in a multidisciplinary approach, especially those patients with limited improvement despite the initiation of corticosteroids. Our 2 cases should raise awareness of IgA vasculitis in patients with skin rashes and elevated creatinine levels.

A Complicated Case of Thrombotic Thrombocytopenic Purpura in Pregnancy with Superimposed Preeclampsia.

A 32-year-old woman at 26 weeks gestation presented with severe unexplained thrombocytopenia with signs and symptoms of severe pregnancy-induced hypertension and what appeared to be hemolysis, elevated liver enzymes, and low platelet count syndrome, leading to a severe diagnostic challenge. The multidisciplinary team identified her condition as thrombotic thrombocytopenic purpura. Timely management decisions and multidisciplinary care ensured a safe delivery.

Large-scale bidirectional Mendelian randomization study identifies new gut microbiome significantly associated with immune thrombocytopenic purpura.

Introduction: A variety of studies have shown a link between the gut microbiota and autoimmune diseases, but the causal relationship with Henoch-Schönlein purpura (HSP) and immune thrombocytopenic purpura (ITP) is unknown. Methods: This study investigated the bidirectional causality between gut microbiota and HSP and ITP using Mendelian randomization (MR). Large-scale genetic data of gut microbiota at phylum to species level from the MiBioGen consortium and the Dutch Microbiome Project were utilized. Genome-wide association studies (GWAS) summary statistics for HSP and ITP came from FinnGen R10. Various MR methods were applied to infer causal relationships, including inverse variance weighted (IVW), maximum likelihood (ML), cML-MA, MR-Egger, weighted median, weighted model, and MR-PRESSO. Multiple sensitivity analyses and Bonferroni correction were conducted to enhance robustness and reliability. Results: Based on the IVW estimates, 23 bacterial taxa were identified to have suggestive associations with HSP and ITP. Remarkably, after Bonferroni correction, family Alcaligenaceae (OR = 2.86, 95% CI = 1.52-5.37; IVW, p = 1.10 × 10-3, ML, p = 1.40 × 10-3) was significantly associated with ITP as a risk factor, while family Bacteroidales S24 7group (OR = 0.46, 95% CI = 0.29-0.74; IVW, p = 1.40 × 10-3) was significantly associated with ITP as a protective factor. No significant associations between HSP and ITP and gut microbiota were found in reverse analyses. Conclusion: Our study provides evidence of causal effects of gut microbiota on HSP and ITP, highlighting the importance of further research to clarify the underlying mechanisms and develop targeted therapeutic interventions for these autoimmune diseases.

Role of plasma exchange in a post-partum case of severe thrombotic thrombocytopenic purpura with acute kidney injury.

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease present with the classic pentad of microangiopathic hemolytic anemia (MAHA), fever, neurologic changes, thrombocytopenia, and renal dysfunction. In a diagnostic dilemma, therapeutic plasma exchange (TPE) is a choice of life-saving intervention. In this, we assess the efficacy of TPE in a suspected case of post-partum TTP. A 27 years old female was admitted in an emergency on day 8 after a lower segment cesarian section (LSCS) with unresponsive behavior for 3 days and with TTP. She was normal 32 days back with her second, 7-month pregnancy. Ultrasonography (USG) showed an umbilical cord around the neck of the baby. On the fifth post-operative day, she was shifted to emergency with fever, generalized anasarca, gastrointestinal tract (GI) bleeding, low platelet count, and low Hb, with a poor Glasgow coma scale (GCS) of 6. On the bases of serum urea and serum creatinine, she presented acute kidney injury with encephalopathy. At emergency, she was unresponsive to mechanical ventilation and supportive treatment; hence, therapeutic plasma exchange was performed. After eight TPE cycles, the patient presented with an improved hematological and renal profile with good GCS. TPE is helpful and life-saving for suspected TTP patients with AKI.

Practical approach to thrombocytopenia in patients with sepsis: a narrative review.

Thrombocytopenia frequently occurs in patients with sepsis. Disseminated intravascular coagulation (DIC) may be a possible cause of thrombocytopenia owing to its high prevalence and association with poor outcomes; however, it is important to keep the presence of other diseases in mind in sepsis practice. Thrombotic microangiopathy (TMA), which is characterized by thrombotic thrombocytopenic purpura, Shiga toxin-producing Escherichia coli hemolytic uremic syndrome (HUS), and complement-mediated HUS, is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ damage. TMA has become widely recognized in recent years because of the development of specific treatments. Previous studies have reported a remarkably lower prevalence of TMA than DIC; however, its epidemiology is not well defined, and there may be cases in which TMA is not correctly diagnosed, resulting in poor outcomes. Therefore, it is important to differentiate DIC from TMA. Nevertheless, differentiating between DIC and TMA remains a challenge as indicated by previous reports that most patients with TMA can be diagnosed as DIC using the universal coagulation scoring system. Several algorithms to differentiate sepsis-related DIC from TMA have been suggested, contributing to improving the care of septic patients with thrombocytopenia; however, it may be difficult to apply these algorithms to patients with coexisting DIC and TMA, which has recently been reported. This review describes the disease characteristics, including epidemiology, pathophysiology, and treatment, of DIC, TMA, and other diseases with thrombocytopenia and proposes a novel practical approach flow, which is characterized by the initiation of the diagnosis of TMA in parallel with the diagnosis of DIC. This practical flow also refers to the longitudinal diagnosis and treatment flow with TMA in mind and real clinical timeframes. In conclusion, we aim to widely disseminate the results of this review that emphasize the importance of incorporating consideration of TMA in the management of septic DIC. We anticipate that this practical new approach for the diagnostic and treatment flow will lead to the appropriate diagnosis and treatment of complex cases, improve patient outcomes, and generate new epidemiological evidence regarding TMA.

Severe Thrombocytopenia Secondary to Systemic Lupus Erythematosus With Antiphospholipid Antibodies in a Middle-Aged Woman.

Thrombocytopenia is a common hematological complication of systemic lupus erythematosus (SLE). However, severe thrombocytopenia is a relatively rare presentation, accounting for only 3-10% of cases. A 52-year-old woman was being treated with 4 mg/day of prednisolone for 12 years for SLE-induced autoimmune hemolytic anemia. She presented to her family physician with nasal bleeding and purpura, which required more than two hours to control. She had bruises on her legs and mild multiple arthralgia. The platelet count was 19,000/µL. She was suspected to have developed immune thrombocytopenia as an exacerbation of SLE. Thus, she was referred to our hospital. Laboratory examination revealed thrombocytopenia, hypocomplementemia, and a positive result for anti-cardiolipin (CL) and anti-ß2-glycoprotein (GP) I IgG antibodies. The patient was diagnosed with thrombocytopenic purpura, complicated by SLE. Methylprednisolone pulse therapy, followed by 60 mg/day of prednisolone and 200/400 mg of hydroxychloroquine on alternate days, was initiated. The platelet count increased from 5,000/µl to 50,000/µl, and the immature platelet fraction (IPF) decreased from 14.9% to 6.3%. Anti-CL and anti-ß2-GPI IgG antibodies were considered to be associated with thrombocytopenia and a risk of thrombotic events after normalization of her platelet counts. Therefore, aspirin therapy was initiated to prevent thrombosis. As an episode of acute thrombocytopenia occurred without other clinical findings indicating active SLE, it was important to determine the exact cause of thrombocytopenia in this situation. Immediate recovery of thrombocytopenia with high-dose prednisolone reduced the risk of bleeding that could have otherwise been fatal.

The CARMEN-France registry of adult patients with immune thrombocytopenia and autoimmune hemolytic anemia in France.

The CARMEN-France registry is a prospective, multicenter registry in France including adult patients with a new diagnosis of immune thrombocytopenia or of autoimmune immune hemolytic anemia (2402 patients included in December 31, 2023). The recording of clinical, biological and treatment data allows detailed epidemiological and pharmacoepidemiological real-world studies. This review summarizes the CARMEN-France registry protocol, gives examples of studies conducted in the registry, and indicates future directions such as inclusion of patient reported outcomes, linkage with the French national health insurance database and linkage with other registries in Europe.

Acquired immune thrombotic thrombocytopenic purpura (TTP) associated with inactivated COVID-19 vaccine CoronaVac.

Corona virus disease 2019 (COVID-19) due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has affected the whole world. Acquired thrombotic thrombocytopenic purpura (TTP) has been reported after administration of mRNA- or adenoviral vector-based COVID-19 vaccines, including Ad26.COV2-S, BNT162b2, mRNA-1273, and ChAdOx1 nCov-19. However, whether inactivated vaccines, such as CoronaVac, could cause TTP and whether the symptoms in TTPs caused by inactivated vaccines are different from previously reported cases are unknown. In this study, two cases were reported. Both cases developed TTP after the second CoronaVac vaccination shot, but not the first. They demonstrated symptoms of fever, neurological abnormalities, renal dysfunction, thrombocytopenia, and hemolysis. Both patients achieved complete remission through several sessions of plasma exchanges and immune suppression. The incidence of TTP in Nanjing area was analyzed. The number of patients with TTP was 12 in 2019, 6 in 2020, 16 in 2021, and 19 in 2022. To the authors' knowledge, this report is the first report of TTP associated with inactivated COVID-19 vaccine (CoronaVac). The rarity and delayed onset may be due to the relatively milder immune response caused by the inactivated vaccines than mRNA-based ones. Timely plasma exchange is a vital treatment for CoronaVac-related TTP, similar to activated vaccine-related TTP.