Association of Sjögren's syndrome with immune-mediated thrombotic thrombocytopenic purpura and posterior reversible encephalopathy syndrome: A case report.

Background: A patient with Sjögren's syndrome (SS), immune-mediated thrombotic thrombocytopenic purpura (ITTP), and posterior reversible encephalopathy syndrome (PRES) was reported, and all published cases with thrombotic thrombocytopenic purpura (TTP), PRES, and SS were retrieved and analysed. The patient's clinical data and treatment procedure have been discussed. Case summary: A 45-year-old Chinese female was hospitalized with headache and low platelet count. She had previously presented to a local hospital with a 7-month history of epigastric discomfort and anorexia, and was diagnosed with SS and ITTP. Laboratory investigations after admission showed platelet (PLT) of 13*10^9/L, red blood cell (RBC) fragments of 6 %, ADAMTS13 Activity＜0.2 %, anti-ADAMTS13 IgG of 88.3U/mL. Brain magnetic resonance imaging (MRI) showed gyriform restricted diffusion along with increased T2-FLAIR signal in the left frontal cortex and bilateral parietal temporal cortex. She was diagnosed with SS, ITTP and PRES, and received the treatment of methylprednisolone, cyclosporine, plasma exchange, IVIG, and rituximab. This patient did not experience the recurrence during the 8-month follow-up period. Conclusion: ITTP and PRES are rare manifestations of SS. After a suspected or confirmed diagnosis of ITTP, plasma exchange and immunosuppressive therapy should be immediately administered. We suggest that rituximab could have additional therapeutic value for SS combined with ITTP and PRES.

Efficacy and safety of daratumumab in multiresistant immune-mediated thrombotic thrombocytopenic purpura.

The immunosuppressive treatment of immune-mediated thrombotic thrombocytopenic purpura (iTTP) in patients with intolerance or refractoriness to the B-cell depleting monoclonal antibody rituximab remains debated. Daratumumab, a plasma cell-directed monoclonal antibody targeting CD38, represents a therapeutic option, but data are scarce. The French Thrombotic Microangiopathies Reference Center conducted a nationwide survey on iTTP patients treated with daratumumab. Nine episodes from seven patients were identified. Treatment was administered for A Disintegrin And Metalloproteinase with ThromboSpondin-1 motifs, 13th member (ADAMTS13) relapses while patients were otherwise in clinical response (N = 8), or during the acute phase of the disease following rituximab intolerance (N = 1). Patients have received a median of three previous therapeutic lines. ADAMTS13 activity improved in eight cases following daratumumab administration, including three cases where ADAMTS13 normalized. ADAMTS13 relapses occurred in three patients; in two cases, retreatment with daratumumab was successful. Median ADAMTS13 relapse-free survival was not reached; 12-month ADAMTS13 relapse-free survival was 56%. Daratumumab-related adverse events occurred in five cases and were non-severe infusion-related reactions in all cases. These results suggest that daratumumab may be an effective treatment option for iTTP patients with intolerance or refractoriness to rituximab.

SARS-CoV-2 as a trigger of IgA vasculitis: a clinical case and literature review.

Coronavirus Disease 2019 (COVID-19), caused by SARS-CoV-2, has negatively affected global health. COVID-19 has been associated with a variety of autoimmune and inflammatory disorders, complicating its respiratory manifestations. SARS-CoV-2 triggers inflammatory reactions which may involve multiple organs and systems. The proof for IgA involvement in the immune reactions to coronavirus infection is growing, particularly in the case of IgA immune complex deposition diseases such as IgA vasculitis (IgAV) and IgA nephropathy.This report presents a case of IgAV caused by SARS-CoV-2 in a 53-year-old man. His symptoms included papillomatous, bright red rashes, urticaria throughout the body, aphthous stomatitis, pain in all joints and muscles, weakness, malaise, abdominal pain, face swelling, and arterial hypertension (160/100 mmHg). He received intravenous methylprednisolone (250 mg) and then oral methylprednisolone (16 mg) treatment, which improved his condition. This improvement included the disappearance of abdominal and joint pain and skin rashes.This article also provides an overview of published cases of IgAV after SARS-CoV-2. It may alert rheumatologists and allied specialists of clinical features of IgAV and guide them how to diagnose and treat this disease.

A comparative study of anti-ADAMTS-13 antibody dynamics in immune-mediated thrombotic thrombocytopenic purpura.

Background: Thrombotic thrombocytopenic purpura, particularly its immune-mediated variant (iTTP), necessitates accurate diagnostic approaches for effective management. Objectives: To compare a chemiluminescence immunoassay (CLIA) and an enzyme-linked immunosorbent assay (ELISA) for testing ADAMTS-13 activity and detecting anti-ADAMTS-13 autoantibodies (AAbs) in patients with iTTP. Methods: This study involved 31 paired samples from 12 iTTP patients. ADAMTS-13 activity was measured using the HemosIL AcuStar (Instrumentation Laboratory, CLIA) and Technozym (Technoclone) activity assay (ELISA). The presence of AAbs was assessed using Technozym ADAMTS-13-INH assay (ELISA) and HemosIL AcuStar activity (CLIA) within a Bethesda assay following mixing with normal pool plasma. von Willebrand factor (VWF) multimers were analyzed using the HYDRASYS-2 SCAN system and the HYDRAGEL 5- or 11-VW Multimer kits (Sebia). VWF activity levels were measured with the HemosIL AcuStar VWF:GPIbR on the ACL AcuStar Analyzer (IL). Results: For ADAMTS-13 activity, a strong linear relationship and no bias between CLIA and ELISA were confirmed (slope = 1.01 [0.91, 1.11], intercept = 0.00 [-0.47, 0]). However, significant discrepancies were found in AAb detection during remission phases with ADAMTS-13 activity between 10% and 50%, with CLIA and ELISA showing significant divergence (P < .001, Cohen's g = 0.34). Consistently, VWF multimers and activity levels exhibited significantly different values between remission samples with ADAMTS-13 activity below 50% and above 50%. In longitudinal analysis of patients with multiple iTTP relapses, positivity to CLIA appears to precede ELISA in predicting exacerbations. Conclusion: While CLIA and ELISA might be interchangeable for assessing ADAMTS-13 activity, they are not equivalent for detecting AAbs, particularly in patients in clinical remission with ADAMTS-13 activity between 10% and 50%.

Caplacizumab improves clinical outcomes and is well tolerated across clinically relevant subgroups of patients with immune-mediated thrombotic thrombocytopenic purpura.

Background: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) may lead to microvascular thrombosis and mortality, despite patients receiving appropriate standard of care treatment (immunosuppressive therapy and therapeutic plasma exchange). Caplacizumab directly inhibits von Willebrand factor-platelet interaction and consequently prevents microthrombi formation. Objectives: This study aimed to determine the efficacy and safety of caplacizumab in diverse, clinically relevant patient subgroups. Methods: In this post hoc analysis of phase 3 HERCULES study (NCT02553317), patients were categorized by clinically relevant subgroups (prior iTTP history, iTTP severity at presentation, and initial immunosuppression regimen). Results: In patients with previous acute iTTP episodes, less severe disease at presentation, or those who received a corticosteroid-only initial immunosuppression regimen, time to platelet count response was shorter with caplacizumab vs placebo. Across all subgroups, fewer patients experienced a composite outcome of iTTP-related death, exacerbation, or major thromboembolic event on caplacizumab vs placebo. Placebo-treated patients remained at risk of exacerbations and refractoriness on either initial immunosuppression regimen (ie, corticosteroids only or corticosteroids plus rituximab). In the corticosteroids plus rituximab group, no exacerbations were reported in caplacizumab-treated patients, but 8 of the 16 (50%) patients experienced exacerbations in the placebo group. Safety outcomes were consistent with the findings of the main HERCULES study. Conclusion: Caplacizumab treatment of acute iTTP, in combination with therapeutic plasma exchange and immunosuppression, was safe and effective regardless of prior iTTP history, severity, or initial immunosuppression regimen and improved patient outcomes across clinically diverse subgroups. These findings emphasize the need for treatments with rapid onset of action that can reduce mortality and iTTP-related complications.

Literature review and case report of septic arthritis and purpura fulminans leading to a child limb amputation as chickenpox complications.

Introduction and significance: Chickenpox, induced by the varicella-zoster virus (VZV), generally presents with an itchy rash and fluid-filled blisters. While complications such as pneumonia and sepsis are well-documented, occurrences of septic arthritis and purpura fulminans are exceedingly rare. Septic arthritis following varicella infection is infrequently reported and often attributed to Staphylococcus aureus. Purpura fulminans encompasses disorders characterized by rapidly progressing purpuric lesions, often fatal and associated with consumptive coagulopathy. Case presentation: The authors present the case of an 8-year-old boy diagnosed with chickenpox who concurrently developed severe left knee pain, erythema, and swelling indicative of septic arthritis, along with a single pustular lesion on his right foot that progressed to purpura fulminans. Laboratory investigations revealed elevated inflammatory markers. Knee ultrasound findings were consistent with septic arthritis, corroborated by synovial fluid analysis. Immediate initiation of empiric antibiotics was undertaken. Further investigation disclosed unusual coagulation parameters, positive autoantibodies, and reduced protein S levels. Treatment included anticoagulation, immunomodulation, and ultimately, amputation. Clinical discussion: This rare case underscores the complexity of varicella-related complications, representing the first documented instance of simultaneous septic arthritis and purpura fulminans in a pediatric patient. It highlights the necessity of a multidisciplinary approach for accurate diagnosis and management, emphasizing the importance of recognizing rare complications to improve patient outcomes. Conclusion: This case exemplifies the complexity of varicella-associated complications, showcasing a rare simultaneous occurrence of septic arthritis and purpura fulminans in a pediatric patient. It underscores the importance of a thorough understanding and collaborative management approaches for timely intervention and enhanced clinical outcomes.

Chlorogenic acid compounds from sweetpotato (Ipomoea batatas L.) leaves facilitate megakaryocyte differentiation and thrombocytopoiesis via PI3K/AKT pathway.

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by antiplatelet autoantibodies, thrombocytopenia, and bleeding, however, its treatment options are limited. In this study, a kind of active component, chlorogenic acid compounds (CGAs) from sweetpotato leaves was extracted out to explore its medicinal value and provide novel therapeutic strategies for the treatment of ITP. CGAs was isolated by ionic liquids-ultrasound (IL-UAE), which contains six isomers of chlorogenic acid with total purity of 95.69%. The thrombopoietic effect and mechanism of CGAs were investigated using in silico prediction and experimental validation. The changes of HEL cells morphology in volume and the increase in the total cell percentage of polyploid cells indicated that CGAs could promote megakaryocyte differentiation. Meanwhile, CGAs could promote platelet formation in a murine model of ITP, which was established by injection of antiplatelet antibody. Further quantitative proteomics analysis and Western blot verification revealed that CGAs could activate PI3K/AKT signaling pathway, which confirmed the mechanism prediction. It suggested that CGAs may provide a novel therapeutic strategy that relies on the PI3K/AKT pathway to facilitate megakaryocyte differentiation and platelet production.

Caplacizumab as an add-on therapy in a 7-year-old girl with exacerbated immune-mediated thrombotic thrombocytopenic purpura, a case report and literature review.

The cornerstone treatment for immune-mediated thrombotic thrombocytopenic purpura (iTTP) in children is a combination of therapeutic plasma exchange (TPE), corticosteroids, and rituximab. Caplacizumab is an anti-von Willebrand factor (VWF) NANOBODY molecule approved as a frontline therapy of iTTP for adults and children aged ≥12 years. Using caplacizumab in children aged <12 years remains a gray area based on recommendations but with no marketing authorization. We report the first case of a pediatric patient with iTTP successfully treated with a caplacizumab dose adjustment of 5 mg daily based on ADAMTS13 activity. We also review all published cases of iTTP in children aged <12 years treated with caplacizumab. This is a 7-year-old girl with clinical thrombotic microangiopathy, in the absence of diarrhea and kidney injury. With a French score of 2 and a PLASMIC score of 7 (high risk), the diagnosis of TTP was suspected and later confirmed by severely low ADAMTS13 activity (<5%). Immune-mediated TTP was distinguished from the congenital one due to the presence of a functional ADAMTS13 inhibitor. Daily TPE and intravenous corticosteroids were started on day 0 (D0). Rituximab was added on D4, and due to refractoriness under daily TPE, we considered off-label administration of caplacizumab from D12. A clinical answer, with a significant increase in the platelet count, was observed within 48 h. A complete ADAMTS13 recovery was reached on D62. No major adverse events were observed during the treatment. She was discharged from the hospital over 3 months ago with a platelet count still within normal ranges. In the literature, we identified a total of four case reports describing five iTTP patients aged <12 years treated with caplacizumab, with a 100% success and tolerability rate. These published data attest to the efficacy and safety of the systematic use of caplacizumab and rituximab as frontline therapy in pediatric iTTP under 12 years of age. Therefore, prospective data are needed to support commercial authorization of caplacizumab in this subpopulation. Close monitoring of ADAMTS13 activity is particularly of interest among children to limit the number of caplacizumab injections.

Evaluating the Effectiveness of Laparoscopic Removal of an Accessory Spleen After a Failed Splenectomy for Immune Thrombocytopenia.

Immune thrombocytopenic purpura (ITP) is a challenging condition to manage especially when conventional treatment methods, including splenectomy, fail. This report evaluates the effectiveness of laparoscopic removal of accessory spleen for chronic refractory ITP after an initial splenectomy. A 73-year-old African American male with a history of ITP, previously treated with laparoscopic splenectomy nine years ago, presented with severe thrombocytopenia that was found to be refractory to medical therapies. Platelet counts were monitored, and the absence of Howell-Jolly bodies was noted in the peripheral blood smear. Imaging studies over the past eight years indicated the growth of a mass in the left upper abdomen, suggesting a possible accessory spleen. Given the overwhelming evidence of a splenule in refractory thrombocytopenia, laparoscopic exploration and mass removal were conducted. Histologic analysis of the removed mass confirmed a splenule. Despite the complete removal of the mass, postoperative platelet counts remained consistently low and unresponsive to the resumption of medical therapies. This study emphasizes the limitations of accessory splenectomy for refractory ITP and highlights the need for further research to clarify the long-term effectiveness of this surgical procedure in these patients.

Legionella Pneumophila Presenting as a Rare Cause of Acute Thrombocytopenia: A Case Report and Review of Literature.

Introduction: Legionella pneumophila can cause a wide spectrum of clinical manifestations, ranging from a mild flu-like illness to fulminant multi-organ involvement, characterised by severe pneumonia, diarrhoea, encephalopathy, shock, hepatic dysfunction and renal failure. Very rarely, it can be associated with haematologic conditions such as thrombotic thrombocytopenic purpura (TTP), haemolytic uraemic syndrome (HUS) and immune thrombocytopenic purpura (ITP). We report a rare case of L. pneumophila causing ITP and review previously published cases of thrombocytopenia associated with Legionellosis in the literature. Case description: A 53-year-old male presented with fevers, chills, a productive cough and severe haemoptysis. Blood work was remarkable for leukocytosis, severe thrombocytopenia and hyponatraemia. Computed tomography (CT) imaging showed left lower lobe lung consolidation, and a peripheral blood smear showed giant platelets consistent with ITP. Legionella urine antigen testing returned positive. He was treated with intravenous immunoglobin, steroid taper and a ten-day course of azithromycin, which led to normalisation of his platelet count and resolution of the pneumonia. Discussion: L. pneumophila can lead to complement-mediated destruction of platelets resulting in ITP. Antibodies against L. pneumophila can also cross-react with the enzyme ADAMTS13, inhibiting its function and resulting in TTP and HUS. Additionally, L. pneumophila can infect vascular endothelial cells causing their death and stimulating release of von Willebrand factor (vWF) multimers into the bloodstream, promoting thrombosis and platelet consumption. Conclusion: It is important for internists to consider L. pneumophila in the differential for any patient presenting with pneumonia and severe thrombocytopenia. Earlier detection and intervention can lead to prevention of critical bleeding and better outcomes. LEARNING POINTS: Legionella pneumophila is rarely associated with different haematologic disorders resulting in severe bleeding diathesis as well as thrombosis.It is important for internists to consider Legionella pneumophila in the differential diagnosis for any patient presenting with pneumonia and severe thrombocytopenia.Earlier detection and intervention can lead to prevention of critical bleeding and better outcomes.

Practical Considerations for the Use of the Rapid AcuStar® ADAMTS13 Activity Assay in the Diagnosis of Acute Thrombotic Thrombocytopenic Purpura (TTP).

Introduction: Conventional practice in the management of acute TTP entails empirical treatment of suspected cases whilst awaiting confirmatory ADAMTS13 deficiency testing. Rapid ADAMTS13 assays offer increased accessibility and rapid diagnostics. The new automated HemosIL AcuStar® ADAMTS13 assay has seen increasing use among UK TTP Specialist Centres alongside the traditional ELISA method to confirm severe ADAMTS13 deficiency. Methods: A multi-centre retrospective case-control study was performed to review patients demonstrating discrepant ADAMTS13 activity results measured using rapid (AcuStar®) and ELISA assays in parallel from September 2019 to December 2021. Cases were compared with a cohort of suspected TTP patients exhibiting no difference in assay results and in relation to their presenting characteristics and pre-test probability of a diagnosis of TTP. Results: Where the clinical index of suspicion for TTP was high at presentation, acute TTP was confirmed using the AcuStar® assay < 0.2 IU/dL and subsequently < 10 IU/dL by ELISA with zero incidence of discrepancy. For patients with low clinical suspicion of acute TTP, a discrepancy between the AcuStar® and ELISA assay results was observed in 2% of cases; 5-10 IU/dL in AcuStar®, confirmed as >20 IU/dL by ELISA. A concurrent cancer diagnosis or sepsis was observed in 40% of discrepant cases. Conclusions: Where acute TTP is strongly suspected, there is a good correlation between the rapid AcuStar® ADAMTS13 assay and the conventional ELISA assay. Where the clinical suspicion of acute TTP is low, caution should be exercised in the interpretation of the ADAMTS13 activity using the AcuStar® assay. Accurate interpretation requires robust ADAMTS13 testing algorithms to be incorporated into diagnostic pathways.

Vitamin B12 deficiency misdiagnosed as TTP: What can we learn from it?

The case report by Dwyre et al. shows that vitamin B12 deficiency may be misdiagnosed as acute thrombotic thrombocytopenic purpura. Together with similar observations, this underlines that acquired vitamin B12 deficiency-besides the inherited disorder of intracellular cobalamin metabolism, cbl C disease-should be listed as a separate entity of the thrombotic microangiopathies. Commentary on: Dwyre et al. Microangiopathic thrombocytopenia caused by vitamin B12 deficiency responding to plasma exchange. Br J Haematol 2024 (Online ahead of print). doi: 10.1111/bjh.19625.

External validation of a predictive model for aged-related skin tears in Fitzpatrick skin types I-IV cohort.

Older Australians are at increased risk of skin tears with the risk not always recognised or the injury able to be prevented. This study externally validated Rayner et al. (2019) Skin Tear Risk Prediction Model in an independent aged cohort with a Fitzpatrick skin types I-IV from across multiple residential-care sites, over a 6-month period. A total of 362 individuals aged between 65 and 102.5 years completed the study. In all, 165-residents sustained one or more skin tears. Logistic regression analysis was conducted of the five variables (gender, previous history of skin tears, previous history of falls, purpura and solar elastosis) identified in the skin tear model. The skin tear model provided 'good' to nearly 'very good discrimination' for correctly classifying residents at-risk or not-at-risk (area under the curve of 0.799 [95% confidence interval, CI: 0.75-0.84]). The skin tear model correctly predicted 75.8% (sensitivity) of participants with skin tears and 71.6% (specificity) of residents without skin tears. The model demonstrated it could work as a screening tool to identify older individuals at risk of skin tears and would benefit clinical practice as it was easy to use, was reproducible, and had good accuracy across aged-care residents with a Fitzpatrick skin type I-IV.

Pulmonary Tuberculosis Complicated by Thrombotic Thrombocytopenic Purpura: A Case Report and Literature Review.

Thrombotic thrombocytopenic purpura is a rare but life-threatening emergency. Tuberculosis can have hematologic complications. However, concurrent tuberculosis and thrombotic thrombocytopenic purpura are extremely rare. In this study, we report a 53-year-old man who was initially treated for pulmonary tuberculosis but later developed weakness and an altered mental status. Laboratory tests revealed evidence of thrombocytopenia, acute renal insufficiency, and microangiopathic hemolytic anemia. Brain imaging identified intracranial hemorrhage. Further testing revealed low ADAMTS13 activity (1.8%) and positive anti-ADAMTS13 antibody, confirming the diagnosis of thrombotic thrombocytopenic purpura. The patient had a full recovery after anti-tuberculosis treatment, plasma exchange, and supportive care. We present this rare case and review previous relevant studies to remind clinicians about the potential connections between tuberculosis and thrombotic thrombocytopenic purpura. In patients with signs of severe thrombocytopenia and microangiopathic hemolysis, necessary diagnostic tests should be performed to eliminate the possibility of thrombotic thrombocytopenic purpura occurring concurrently with tuberculosis.

Longitudinal characterization of symptoms, healthcare resource utilization, and costs among people with thrombotic thrombocytopenic purpura compared with non-thrombotic thrombocytopenic purpura controls.

AIMS: Thrombotic thrombocytopenic purpura (TTP) is an ultra-rare blood disorder, characterized by severe ADAMTS13 deficiency. Affected individuals present with potentially life-threatening acute events and may experience sub-acute and chronic TTP manifestations often resulting in long-term organ damage. Incremental symptom prevalence before, during, and after an acute event as well as healthcare resource utilization (HCRU) and costs during and after an acute event were compared between people with TTP and matched non-TTP controls. METHODS: This retrospective, matched study used data from Merative MarketScan Commercial Database and Medicare Supplemental Database (from January 1, 2008, through September 30, 2021) to identify people with TTP (inpatient diagnosis for "thrombotic microangiopathy (TMA)" or "congenital TTP," and ≥1 claim for plasma exchange or infusion). People with TTP were matched (1:2) with non-TTP controls on age, sex, geographic region, index year, and select Elixhauser comorbidities. RESULTS: 255 people with TTP were matched with 510 non-TTP controls. Both cohorts had a mean age of 43.9 years; 71% were female. Overall, more people with TTP reported symptoms compared with non-TTP controls prior to (51% vs 43%), during (99% vs 52%), and after an acute event (85% vs 50%; p < 0.05 for all periods). Symptom prevalence decreased following an acute event compared with during an acute event, but remained high-85% of people with TTP experienced symptoms compared with 50% of non-TTP controls. HCRU and mean costs per patient per month were significantly higher in all care settings among people with TTP compared with non-TTP controls (p < 0.05). LIMITATIONS: Identification of patient populations may have been limited due to coding errors, as the data were obtained from an administrative claims database. CONCLUSIONS: TTP is associated with a substantial symptom burden and increased costs and HCRU during and up to almost a year after acute events, demonstrating the longitudinal burden of this disease.

Development of Palpable Purpura in a Patient With Infective Endocarditis: A Case Report.

Infective endocarditis (IE) can present with a variety of signs and symptoms, including skin lesions. The few papers describing a relationship between IE and vasculitis are split between IE being able to mimic vasculitis and between IE indeed being associated with a vasculitis involving the skin, kidney, gastrointestinal tract, or peripheral nerves. It is important for clinicians to distinguish between an isolated vasculitis, infective endocarditis, and IE-associated vasculitis because the treatments and outcomes are different. We report a case of a patient with a history of intravenous (IV) drug use who initially presented with chest pain, was started on vancomycin following diagnosis of methicillin-resistant Staphylococcus aureus (MRSA) IE, left against medical advice (AMA), and then returned to the hospital due to development of a purpuric rash. We contend that while he did not have a skin biopsy due to time delay, his symmetrically distributed purpura was consistent with cutaneous vasculitis. His symptoms, including his rash and an acute kidney injury (AKI), improved with antibiotics to treat the endocarditis.

Recurrent, atraumatic acute compartment syndrome secondary to IgA vasculitis: A case report.

Acute compartment syndrome is a surgical emergency requiring rapid recognition in the emergency department to minimize morbidity and mortality. It is most commonly caused by traumatic extremity fractures, which account for about 75% of cases. Atraumatic acute compartment syndrome is substantially less common with current evidence mostly limited to case reports, and diagnosis is made more challenging by the absence of an obvious traumatic injury. We present the case of a young adult female patient with IgA vasculitis who developed recurrent, atraumatic acute compartment syndrome and was successfully managed with prompt fasciotomy. This is the first case of spontaneous intramuscular hemorrhage, a rare sequela of IgA vasculitis, leading to recurrent, atraumatic acute compartment syndrome. This case highlights the importance of both a thorough physical exam and maintaining a high suspicion for acute compartment syndrome in the absence of injury to ensure patients receive prompt surgical evaluation for definitive care.

Effect of Immune Thrombocytopenic Purpura Medications on Depression Risk: An Analysis of NHANES Data.

BACKGROUND: Immune thrombocytopenic purpura (ITP) in adults typically develops slowly and insidiously. The ITP medications might be linked to psychological disorders, but the connection is not well-understood. OBJECTIVE: This study aimed to examine the association between ITP medication use and the risk of depression among participants in the National Health and Nutrition Examination Survey (NHANES) from 2005 to 2018. METHODS: Using data from 70 190 NHANES participants, we conducted a cross-sectional study, excluding individuals under 18 years, with hypertension, HIV, hepatitis C, and various comorbidities. A total of 17 299 individuals were included in the analysis of this study. We identified 2 populations within this study: those using ITP medications, including prednisone, dexamethasone, and rituximab and those not using ITP drugs. Depression status was assessed using the Patient Health Questionnaire-9 (PHQ-9), and the relationship between ITP medication use and depression was analyzed through multivariate logistic regression. RESULTS: There was no significant association between ITP medication use and an increased risk of depression after adjusting for demographic and health-related variables. Notably, among the study participants, 1.8% of the non-depressed population were on ITP medication compared with 0.3% in the depressed population. The analysis revealed varying depression risks associated with different sociodemographic factors. For instance, the correlation between ITP medication and depression risk was influenced by a combination of age, race, income, and smoking status. CONCLUSION AND RELEVANCE: The study suggests that ITP medication use does not independently increase the risk of depression. This finding is crucial for guiding clinical decisions and managing patient expectations regarding ITP treatment and its psychological impacts.

Relationship between response to first-line steroid treatment in adult immune thrombocytopenic purpura and the course of the disease.

BACKGROUND: Immune thrombocytopenic purpura, a recurrent autoimmune disease, is characterized by thrombocytopenia, purpura and hemorrhagic episodes with the main factor in the pathogenesis of this disease being autoantibodies against platelets. Since the 1950s, first-line treatment has been glucocorticoids that have indirect and direct effects on thrombocytopenia. Although the characteristics associated with the chronicization of immune thrombocytopenic purpura at the time of diagnosis have been investigated in previous studies, no study was found in the literature investigating the relationship between the response to first-line steroid treatment and the course of the disease, the aim of this study. MATERIALS AND METHODS: This retrospective, single center study revisited electronic files of patients with a diagnosis of immune thrombocytopenic purpura between September 2012 and September at the Department of Clinical Hematology, Selcuk University Faculty of Medicine 2022. The platelet count had been confirmed by peripheral blood smears of patients with a platelet count ≤30 × 109/L. The bleeding status of patients at the time of diagnosis was evaluated according to the immune thrombocytopenic purpura bleeding score. Patient responses to treatment were categorized in three groups: a platelet count ≤30 × 109/L was defined as no-response, a platelet count of 30-100 × 109/L was defined as partial response, and a platelet count >100 × 109/L was defined as complete response. Subsequently, patients in the partial or complete response groups were divided into two subgroups: patients who remained in remission for less than or more than six months. RESULTS: A total of 100 patients were included in the study; 73 % were in the young (19-65 years old) and 27 % in the old (>65 years old) age group. Most of the patients were female (69 %). Forty-one patients were hospitalized without bleeding. The complete response rate to first-line treatment was 61 %. There was no significant difference between the agents given in first-line treatment in terms of response and length of remission. CONCLUSION: The main purpose of immune thrombocytopenic purpura treatment is to prevent severe bleeding rather than bringing the platelet count to normal values. Glucocorticoids, the first step of treatment, provide high response rates. There is no significant difference between glucocorticoid agents in terms of response to treatment and long-term remission. The points to be considered in the selection of glucocorticoid agents are the side effect profiles, ease of administration and individualization of treatment.