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.

Extensive Thrombosis in Catastrophic Antiphospholipid Syndrome in a Newly Diagnosed Systemic Lupus Erythematosus: A Case Report.

In this case report, we present the development of catastrophic antiphospholipid syndrome (CAPS), a rare and potentially fatal consequence of systemic lupus erythematosus (SLE), in a 33-year-old Micronesian woman. CAPS is characterized by extensive arterial thrombosis and multiorgan failure. The patient first showed signs of neuropsychiatric symptoms, brain infarctions on imaging, and severe hypoxic respiratory failure brought into the hospital by diffuse alveolar hemorrhage (DAH) along with lupus nephritis (LN). Blood urea nitrogen (BUN) and creatinine (Cr) were progressively elevated to 102/4.1 mg/dL, respectively. A urinalysis revealed microscopic hematuria and proteinuria with a urine protein/creatinine ratio of 6052 mg/g. She was also found to have had microangiopathic hemolytic anemia (MAHA) and severe venous thrombosis, both of which were indicative of CAPS. An aggressive approach, including immunosuppressive medication, therapeutic plasma exchange, and anticoagulation, had positive results, including renal recovery and the cessation of thrombotic episodes. This instance highlights how crucial it is to identify CAPS patients early and take appropriate action to improve patient outcomes for this difficult and sometimes deadly disorder.

Successful Management of Atypical Hemolytic-Uremic Syndrome in Pregnancy Using Eculizumab: A Case Review.

Hemolytic-uremic syndrome (HUS) is a rare thrombotic microangiopathy characterized by the triad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and acute kidney injury. The disease is pathologically marked by fibrinoid necrosis within renal arterioles and glomerular capillaries. HUS can be categorized into typical variants, often linked to Shiga toxin-producing Escherichia coli (STEC) infection, and atypical variants that stem from dysregulation in the alternative complement pathway. Pregnancy is a recognized predisposing condition for HUS due to the potential reduction in complement regulatory proteins and the possibility of heightened maternal immune response. This report illustrates the case of a 36-year-old woman who, at 36 weeks of gestation, faced a breech presentation and was diagnosed with atypical HUS (aHUS) after placental abruption. Following a cesarean section, she developed complications, including a pelvic hematoma and bilateral hydronephrosis. Despite initial suboptimal response to plasmapheresis, the patient exhibited marked clinical improvement with eculizumab treatment, with no evidence of disease relapse.

Hump-Nosed Viper Bite-Associated Thrombotic Thrombocytopenic Purpura: A Rare Complication.

Thrombotic thrombocytopenic purpura (TTP) is one of the rarely encountered complications of hump-nosed viper bites, which requires early detection and specific management. Hump-nosed viper bites are well known to affect multiple systems, and it is imperative to identify and manage each complication simultaneously. A 48-year-old patient presented to the hospital following a hump-nosed viper bite, where he subsequently developed local necrosis, acute kidney injury (AKI), and TTP. A diagnosis of TTP was made using the PLASMIC score (which refers to the score's seven components: platelet count; combined hemolysis variable; absence of active cancer; absence of stem-cell or solid organ transplant; mean corpuscular volume (MCV); international normalized ratio (INR); and creatinine) and supporting blood picture findings despite the diagnostic difficulties encountered due to the misleadingly normal automated platelet counts. The patient underwent multiple blood transfusions, 12 cycles of hemodialysis, and two cycles of therapeutic plasma exchange, the latter contributing to a significant improvement in his overall clinical and biochemical markers. In this case presentation, we report a rare case of TTP occurring after a hump-nosed viper bite, with the outcome of the report focusing on the diagnostic difficulties and available therapeutic modalities.

Hypertensive Emergency with Thrombotic Microangiopathy or TTP? A Case Series and Literature Review.

Thrombotic microangiopathy (TMA) is associated with both hypertensive emergency and primary thrombocytopenia purpura (TTP). However, their clinical management is vastly different, with the latter necessitating urgent plasma exchange (PEX). We report two cases of hypertension-associated TMA (HTN-TMA) and a literature review of the clinical management of malignant hypertension. We suggest that in patients presenting with hypertensive emergency associated with TMA, a clinical diagnosis of HTN-TMA should be made, with emergent treatment to lower blood pressure started immediately. Although TTP is a differential diagnosis for TMA, PEX should not be started concurrently in the absence of other supporting evidence for TTP.

Altered Mental Status in the Setting of Thrombotic Thrombocytopenic Purpura (TTP) and Spontaneous Coronary Artery Dissection (SCAD): A Case Report and Literature Review.

Altered mental status (AMS) is a common condition encountered in daily practice. Finding the cause is essential for treatment, but sometimes this may be challenging. Spontaneous coronary artery dissection (SCAD) is frequently underdiagnosed and is a potentially fatal cause of acute coronary syndrome. Clinical presentation depends on the extent of SCAD, ranging from unstable angina to sudden death. AMS has not been reported with this condition, but it may be possible in hypoperfusion states. Thrombotic thrombocytopenic purpura (TTP) is part of the microangiopathic hemolytic anemia (MAHA) spectrum, presenting with AMS as the cardinal symptom. TTP is a clinical emergency, and a high index of suspicion should be present as the mortality rate in untreated patients is extremely high and can be significantly reduced with proper treatment. We present a case of a 44-year-old female with a past medical history of antiphospholipid syndrome not on anticoagulation, peptic ulcer disease, chronic kidney disease, stroke, seizures, congestive heart failure with reduced ejection fraction (EF 40%), two non-ST-segment elevation myocardial infarctions not on dual antiplatelet therapy due to a history of gastrointestinal bleeding, and TTP, admitted to the hospital with AMS. The patient was diagnosed with two life-threatening pathologies with overlapping features but opposing management; TTP may have been caused by SCAD, even though this has never been reported. It is essential to recognize that while a single diagnosis frequently explains a patient's clinical manifestations, there are instances when various conditions may be present.

Thrombotic Microangiopathy and Multiple Organ Failure in Scleroderma Renal Crisis: A Case Report.

This case report can be considered a rare occurrence of scleroderma renal crisis (SRC) presenting with a severe clinical course and multiple organ failure. A patient diagnosed with systemic sclerosis four years earlier was admitted to the hospital because of severe malignant systolic-diastolic arterial hypertension and acute kidney injury (AKI). Exacerbating disease suggested thrombotic microangiopathy (TMA) and the PLASMIC (Platelet count; combined hemoLysis variable; absence of Active cancer; absence of Stem-cell or solid-organ transplant; MCV; INR; Creatinine) score was used in the differential diagnosis. Despite the timely initiation of therapy with ACE inhibitors (ACE-I), the progressive renal failure required hemodialysis treatment, but renal function never recovered. Disease duration, skin involvement, and previous specific pharmacological therapy represented multiple risk factors that determined a clinical course complicated by pericardial tamponade with acute heart failure, acute pancreatitis, and ischemic stroke, with fatal evolution. These complications presented a challenging clinical sequence of events requiring an interdisciplinary course of action. Timely ascertainment of the SRC is important given the possible severe organ involvement as well as mortality. A diagnosis of scleroderma renal crisis should be considered in cases of acute kidney injury associated with known risk factors. Early treatment and collaboration between rheumatology and renal physicians can improve patient outcomes.

Challenges in the diagnosis of thrombotic thrombocytopenic purpura.

INTRODUCTION: Immune-mediated TTP (iTTP) is a rare condition without pathognomonic signs and symptoms. For this reason, the diagnosis of iTTP may be delayed or even missed, with potentially catastrophic consequences. AREAS COVERED: The authors performed an extensive literature review on the diagnosis of iTTP and its challenges combined with their own experience in a referral center for patients with iTTP. EXPERT OPINION: Although a definitive diagnosis of iTTP depends on the ADAMTS13 activity result, timely testing is rarely available at many centers to which patients present. If less complex tests were to become available, they would decrease the chances of late and/or missed diagnoses of iTTP throughout the world. While clinical scores to estimate the likelihood of iTTP exist, they are not well known, and can be misleading if used in the wrong context. Furthermore, the three scoring systems (PLASMIC, Bentley, and French) only moderately correlate with each other, which further complicates the landscape. The existence of these scores and how they should be used in practice is but one opportunity that can be seized through more robust programs to educate nonspecialist clinicians on how to recognize and treat patients with iTTP.

Case report: Microangiopathic hemolytic anemia and thrombocytopenia in a child with Brucella infection.

Thrombotic thrombocytopenic purpura (TTP) is a diffused microvascular occlusive disorder characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and ischemic organ dysfunction. In addition, this condition has been linked to an absence or dysfunction of ADAMTS13. Although TTP can occur due to various factors, such as bacteria, viruses, autoimmune disorders, drugs, connective tissue conditions, and solid tumors, it is a rare hematological complication associated with brucellosis. We describe the first case of a 9-year-old boy with acquired TTP with undetectable ADAMTS-13 assay secondary to Brucella infection. After initiating antimicrobial therapy, symptoms and laboratory abnormalities improved dramatically, with no recurrence of TTP in subsequent follow-ups.

Imaging Plate Autoradiography for Ingested Anthropogenic Cesium-137 in Butterfly Bodies: Implications for the Biological Impacts of the Fukushima Nuclear Accident.

The Fukushima nuclear accident in March 2011 caused biological impacts on the pale grass blue butterfly Zizeeria maha. At least some of the impacts are likely mediated by the host plant, resulting in "field effects". However, to obtain the whole picture of the impacts, direct exposure effects should also be evaluated. Here, we examined the distribution of experimentally ingested anthropogenic cesium-137 (137Cs) in adult butterfly bodies using imaging plate autoradiography. We showed that 137Cs ingested by larvae was incorporated into adult bodies and was biased to females, although the majority of ingested 137Cs was excreted in the pupal cuticle and excretory material during eclosion. 137Cs accumulation in adult bodies was the highest in the abdomen, followed by the thorax and other organs. These results suggest that 137Cs accumulation in reproductive organs may cause adverse transgenerational or maternal effects mediated by reactive oxygen species (ROS) on germ cells. 137Cs accumulation was detected in field individuals collected in September 2011 and September 2016 but not in May 2011, which is consistent with the abnormality dynamics known from previous studies. Taken together, these results contribute to an integrative understanding of the multifaceted biological effects of the Fukushima nuclear accident in the field.

Protein Delivery to Insect Epithelial Cells In Vivo: Potential Application to Functional Molecular Analysis of Proteins in Butterfly Wing Development.

Protein delivery to cells in vivo has great potential for the functional analysis of proteins in nonmodel organisms. In this study, using the butterfly wing system, we investigated a method of protein delivery to insect epithelial cells that allows for easy access, treatment, and observation in real time in vivo. Topical and systemic applications (called the sandwich and injection methods, respectively) were tested. In both methods, green/orange fluorescent proteins (GFP/OFP) were naturally incorporated into intracellular vesicles and occasionally into the cytosol from the apical surface without any delivery reagent. However, the antibodies were not delivered by the sandwich method at all, and were delivered only into vesicles by the injection method. A membrane-lytic peptide, L17E, appeared to slightly improve the delivery of GFP/OFP and antibodies. A novel peptide reagent, ProteoCarry, successfully promoted the delivery of both GFP/OFP and antibodies into the cytosol via both the sandwich and injection methods. These protein delivery results will provide opportunities for the functional molecular analysis of proteins in butterfly wing development, and may offer a new way to deliver proteins into target cells in vivo in nonmodel organisms.

The Plastic Larval Body Color of the Pale Grass Blue Butterfly Zizeeria maha (Lepidoptera: Lycaenidae) in Response to the Host Plant Color: The Maternal Effect on Crypsis.

Many lepidopteran larvae show body color polyphenism, and their colors may be cryptic on the host plant leaves. To elucidate the effect of the host plant color on the plastic larval body color, we focused on the lycaenid butterfly Zizeeria maha, which shows various larval body colors ranging from green to red, even within a sibling group. We showed that oviposition was normally performed on both green and red leaves, despite a green preference and the fact that the larvae grew equally by consuming either green or red leaves. The number of red larvae decreased from the second instar stage to the fourth instar stage, demonstrating a stage-dependent variation. When the larvae were fed either green or red leaves across multiple generations of the lineages, the red larvae were significantly more abundant in the red leaf lineage than in the green leaf lineage. Moreover, the red-fed siblings showed a significantly higher red larval frequency than the green-fed siblings in the red-leaf lineage but not in the green-leaf lineage. These results suggest that, in this butterfly species, the plastic larval body color for crypsis may be affected not only by the color of the leaves that the larvae consume (single-generation effect) but also by the color of the leaves that their mothers consume (maternal effect), in addition to a stage-dependent color variation.

Recurrent stroke in an African female with idiopathic thrombotic thrombocytopenic purpura: A case report.

We report on a young Ghanaian female who was diagnosed with thrombotic thrombocytopenic purpura (TTP) but had an ischemic stroke as the initial presentation. She was successfully treated with therapeutic plasma exchange. This case illustrates how TTP can masquerade as ischemic stroke and the application of PLASMIC score without ADAMTS-13 assay in risk prediction.

A Case of Thrombotic Microangiopathy and Acute Sarcoidosis.

Although sarcoidosis is an established cause of multiorgan dysfunction, acute presentation with thrombotic microangiopathy resulting in severe renal and hematological sequelae has not been reported. We describe the case of a patient presenting with hypercalcemia, pancreatitis, and acute renal failure, followed by microangiopathic hemolytic anemia. Although there were no significant respiratory symptoms, thoracic radiology and mediastinal lymph node biopsy results were in keeping with sarcoidosis as the underlying cause of this multisystem presentation. Corticosteroids were commenced with clinical and biochemical improvement. This novel case highlights the need to consider sarcoidosis as part of the differential diagnosis for unusual multiorgan presentations and for early multidisciplinary involvement in such cases to permit optimal treatment.

Ingestional Toxicity of Radiation-Dependent Metabolites of the Host Plant for the Pale Grass Blue Butterfly: A Mechanism of Field Effects of Radioactive Pollution in Fukushima.

Biological effects of the Fukushima nuclear accident have been reported in various organisms, including the pale grass blue butterfly Zizeeria maha and its host plant Oxalis corniculata. This plant upregulates various secondary metabolites in response to low-dose radiation exposure, which may contribute to the high mortality and abnormality rates of the butterfly in Fukushima. However, this field effect hypothesis has not been experimentally tested. Here, using an artificial diet for larvae, we examined the ingestional toxicity of three radiation-dependent plant metabolites annotated in a previous metabolomic study: lauric acid (a saturated fatty acid), alfuzosin (an adrenergic receptor antagonist), and ikarugamycin (an antibiotic likely from endophytic bacteria). Ingestion of lauric acid or alfuzosin caused a significant decrease in the pupation, eclosion (survival), and normality rates, indicating toxicity of these compounds. Lauric acid made the egg-larval days significantly longer, indicating larval growth retardation. In contrast, ikarugamycin caused a significant increase in the pupation and eclosion rates, probably due to the protection of the diet from fungi and bacteria. These results suggest that at least some of the radiation-dependent plant metabolites, such as lauric acid, contribute to the deleterious effects of radioactive pollution on the butterfly in Fukushima, providing experimental evidence for the field effect hypothesis.

The second decade of the blue butterfly in Fukushima: Untangling the ecological field effects after the Fukushima nuclear accident.

Many field observations of the biological effects of the Fukushima nuclear accident have been reported in the first decade after the accident. A series of observational and experimental studies have demonstrated causal adverse effects on the pale grass blue butterfly even at the low-level radiation exposure in the "field," contrary to the dosimetric view that insects are generally tolerant of radiation exposure. However, it has been demonstrated that the pale grass blue butterfly is tolerant of high oral doses of anthropogenic radioactive cesium (137 Cs) under "laboratory" conditions. This field-laboratory paradox can be explained by ecological field effects; for example, radiation stress in the field causes physiological and biochemical changes in the host plant, which then trophically affects butterfly larvae. The second decade of butterfly-based Fukushima research will be devoted to demonstrating how such adverse field effects occur. Changes in the host plant's nutritional contents likely affect butterfly physiology. The host plant may also upregulate secondary metabolites that affect herbivorous insects. The plant may be affected by changes in endophytic soil microbes in radioactively contaminated areas. If demonstrated, these results will reveal that the delicate ecological balances among the butterfly, its host plant, and soil microbes have been affected by radioactive pollution in Fukushima, which has important implications for environmental policies and human health. Integr Environ Assess Manag 2022;18:1539-1550. © SETAC.

Thrombotic microangiopathy presenting with stroke-like symptoms.

Thrombotic microangiopathies are a diverse group of clinical syndromes characterized by microangiographic hemolytic anemia, thrombocytopenia, and organ dysfunction. We report a unique case of thrombotic microangiopathy in an adult caused by vitamin B12 deficiency that presented with stroke-like symptoms.

Post-partum occurrence of Wunderlich syndrome and microangiopathic haemolytic anaemia (MAHA): a case report.

This is a case of a 27-year-old primigravida with monochorionic diamniotic twin gestation who was admitted to the hospital for induction of labour. Her postpartum course was complicated by microangiopathic haemolytic anemia (MAHA). The etiology for the MAHA was initially thought to be secondary to pre-eclampsia and vitamin B12/folate deficiency. However, she had persistent anemia and further workup demonstrated that she had a left renal cell carcinoma (RCC) with perinephric haemorrhage consistent with Wunderlich syndrome. This case was intriguing because of its unusual presentation and the several diagnostic and therapeutic challenges along the way. Abbreviations: MAHA: microangiopathic haemolytic anaemia; RCC: renal cell carcinoma; BP: blood pressure; WS: Wunderlich syndrome; CT: computed tomography; LFTs: liver function tests; LDH: lactate dehydrogenase; HELLP: haemolysis elevated liver enzymes, low platelets; DIC: disseminated intravascular coagulation; PLASMIC: score for TTP - includes platelet count <30 x 109/L, evidence of haemolysis (reticulocyte count >2.5%, haptoglobin undetectable, or indirect bilirubin >2mg/dL), active cancer, history of solid organ transplant, mean corpuscular volume (MCV) <90fL, INR <1.5, creatinine <2mg/dL. Each item is sored as being present (YES) or not (NO). Absence of active cancer and solid organ transplant gets scored with a point each. The total points are added up to categorize the severity and risk of TTP. Low risk <4, Intermediate 5, high risk >6; TTP: thrombotic thrombocytopenic purpura; APLA- anti-phophospholipid antibody; BMI: body mass index; TMAs: thrombotic microangiopathies; HUS: haemolytic uremic syndrome; vWF: von Willebrand factor.

Roosting Site Usage, Gregarious Roosting and Behavioral Interactions During Roost-assembly of Two Lycaenidae Butterflies.

Lycaenidae is one of the larger of the world's butterfly families, based on number and diversity of species, but knowledge of roosting in this group is sparse. Zizina otis riukuensis and Zizeeria maha okinawana are two small lycaenids that are commonly found in urban settings and widely distributed across much of Asia. We conducted experiments on a university campus to determine the plant species and plant structures commonly used by these two blues when roosting. We also tested the hypothesis that gregarious roosting exists in these two blues by demonstrating the non-random distribution of roosting blues and the tight mapping of their roosts to the spatial distribution of specific plant species and/or specific plant structures, as well as by demonstrating behavioral interactions among individuals during roosting-assembly. We found that both Z. otis and Z. maha roosted primarily on flowers and fruits of Tridax procumbens and Vernonia cinerea. We also found that these blues formed conspicuous roosting aggregations with significant positive associations between the flowers and fruits of both T. procumbens and V. cinerea and the blues. Moreover, our behavioral observations showed that these blues expressed various levels of interaction during roosting gatherings. Based on these findings, we conclude that gregarious roosting exists in both Z. otis and Z. maha. To our knowledge, this paper represents one of the first demonstration of nocturnal gregarious roosting in lycaenids. This study also highlights the importance of institutional estates in providing roosting resources for butterflies in urban ecosystems.