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.

Uncommon Manifestation: Thrombotic Thrombocytopenic Purpura Presenting Solely as Petechial Rash.

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder typically presenting with a classic pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal dysfunction, and fever. This report explores an unusual presentation of TTP in a 47-year-old female with a medical history of hypertension, hyperlipidemia, and chronic TTP, who exhibited only petechial rashes, generalized weakness, and headache. Notably, the petechial rash, a less common manifestation of TTP, became a pivotal clue for the diagnosis, underscoring the necessity for vigilance even when classic symptoms are absent. This case reinforces the imperative of a high suspicion index for TTP, especially in patients with thrombocytopenia and hemolytic anemia, irrespective of other traditional signs. Plasmapheresis remains the treatment cornerstone, removing autoantibodies and replenishing ADAMTS13, as evidenced by the patient's initial response. The administration of rituximab, targeting B cells to mitigate autoantibody production against ADAMTS13, featured prominently in her management, aligning with its recognized role in refractory or relapsing TTP cases. Despite an encouraging response to rituximab, a subsequent decline in platelet count indicated the unpredictable nature of TTP and the necessity for multi-pronged therapeutic strategies. The patient's medical background and persistently low ADAMTS13 levels hinted at a chronic relapsing trajectory associated with increased morbidity and mortality. This necessitates ongoing vigilance and treatment flexibility. Highlighting this atypical TTP presentation, the report calls for immediate, robust intervention, serving as a critical reminder of the heterogeneity of TTP manifestations and the complexities in its management, thereby contributing to broader clinical awareness and improved patient prognoses.

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.

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 in patients with sickle cell disease / Microangiopatia trombótica em pacientes com doença falciforme

ABSTRACT Objective: To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD). Case description: Both patients started with a painful crisis and had acute chest syndrome during hospitalization. They showed significant worsening of hemolytic anemia, with very high levels of lactate dehydrogenase, thrombocytopenia, lowered level of consciousness, organ damage and the presence of schistocytes in peripheral blood. Due to the possibility of TMA, despite the very rare association with SCD, they were treated with fresh frozen plasma replacement and plasmapheresis, with good response. Comments: TMA is a serious, life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. The association of SCD and TMA is difficult to diagnose, since they can share a similar clinical presentation. Recognizing this association and promptly instituting treatment may impact the survival of these patients.

RESUMO Objetivo: Descrever dois casos de pacientes que apresentaram microangiopatia trombótica (MAT) associada à doença falciforme (DF). Descrição do caso: Ambos os pacientes iniciaram com crise dolorosa e apresentaram síndrome torácica aguda durante a internação. Eles apresentaram piora significativa da anemia hemolítica, com níveis muito elevados de lactato desidrogenase, trombocitopenia, rebaixamento do nível de consciência, lesão de órgãos e presença de esquistócitos no sangue periférico. Diante da possibilidade de MAT, apesar da associação muito rara com DF, eles foram tratados com reposição de plasma fresco congelado e plasmaférese, com boa resposta. Comentários: A MAT é uma doença grave e com risco de vida, caracterizada por anemia hemolítica microangiopática, trombocitopenia e danos a órgãos. A associação de DF e MAT é de difícil diagnóstico, pois as duas podem ter apresentação clínica semelhante, portanto reconhecer essa associação e instituir o tratamento prontamente pode ter grande impacto na sobrevida desses pacientes.

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.

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.

A case of infection-induced thrombotic thrombocytopenic purpura.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) are a group of microvascular thrombohemorrhagic syndromes with low incidence and high mortality, which are characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neuropsychiatric disorders, and renal involvement. In addition, TTP has a high rate of misdiagnosis and underdiagnosis due to the lack of specific clinical manifestations. CASE REPORT: A male patient aged 47 years was admitted to our hospital with complaints of dizziness and nausea for 2 days and soy-colored urine for 1 day. The patient had caught a cold and suffered from fever, dizziness, and nausea 2 days before admission. These symptoms were relieved by self-administration of berberine 1 day before admission. Later, the patient found that the urine was scanty and soy-colored. Physical examination on admission showed that the patient developed apathy, with occasional babbling, yellowing skin and sclera, and scattered bleeding spots on the anterior chest area. Based on auxiliary tests combined with clinical manifestations, the patient was diagnosed with TTP and administered plasma exchange, hemofiltration, hormone, and anti-platelet therapies. The patient recovered and was discharged after 3 weeks. The patient regularly took aspirin and was followed up one year later with no recurrence. CONCLUSION: TTP is an acute severe disease with complex etiology, abrupt onset, and dangerous conditions. In this patient with TTP, an important cause of the disease may have been an acute gastrointestinal infection. The plasma examination in another hospital revealed positive results for ADAMTS13 inhibitors, providing strong evidence for the diagnosis of this case. Multiple plasma exchanges and glucocorticoids yielded favorable treatment results and were critical measures of successful treatment of TTP.

Thrombotic thrombocytopenic purpura following ChAdOx1 nCov-19 vaccination: A case report.

Vaccine-associated thrombotic thrombocytopenic purpura (TTP) is a rare type of acquired TTP recently reported after COVID-19 vaccination. Merely four cases are ascribed to the ChAdOx1 nCoV-19 vaccine in the medical literature till the preparation of this study. In this case report, we describe a 43-year-old man who developed symptoms of TTP four days after receiving the second dose of the ChAdOx1 nCoV-19 vaccine. Peripheral blood smear demonstrated multiple schistocytes. Given a high plasmic score, he received plasma exchange, corticosteroids, and rituximab, and later, low ADAMTS 13 activity and high-titer ADAMTS inhibition antibody confirmed the diagnosis of COVID-19 vaccine-associated TTP. COVID-19 vaccine-associated TTP is an infrequent consequence of SARS-CoV-2 vaccination but with a substantial mortality rate which must be considered as one of the crucial differential diagnoses of post-COVID-19 vaccine thrombocytopenia besides vaccine-induced immune thrombotic thrombocytopenia and Immune thrombocytopenic purpura.

Thrombotic Thrombocytopenic Purpura Without Evidence of Microangiopathic Hemolytic Anemia: A Case Report.

Diagnosis of thrombotic thrombocytopenic purpura (TTP) is challenging due to varied clinical presentations and is primarily based on ADAMTS13 activity assay, however clinical suspicion to include TTP as a potential diagnosis relies on multiple scoring systems all involving hemolysis as a prime feature. Here, we report a case of TTP without any evidence of microangiopathic hemolytic anemia (MAHA). A 65-year-old male admitted with a Glasglow come scale of 3 was intubated and sedated on admission. Complete blood count was concerning for a hemoglobin (Hb) of 5.8 g/dL, and a platelet count of 76 k/µL. The patient had a bleeding episode while placing a central line; the repeat platelet count was found to be 35 k/µL, further dropping to 14 k/µL the next day. Coagulation studies now reflected PT of 19.8 sec, aPTT of 38.7 sec, and fibrinogen of 212 mg/dL. The peripheral smear showed no evidence of hemolysis. TTP was kept low on the differential and haematological anomalies were attributed to possible disseminated intravascular coagulation (DIC) from sepsis and liver disease. ADAMSTS13 was incidentally checked upon admission, later resulting in <5% activity with a Bethesda titer inhibitor of 3.2. The patient was immediately initiated on plasmapheresis along with prednisone. Additionally, rituximab and caplacizumab were added. Plasmapheresis was continued for ten sessions until the platelet count reached 167 k/µL. At the time of discharge, laboratory values revealed platelets of 251 k/µL and hemoglobin of 8.8 g/dL. We recognize that the diagnosis of TTP is challenging because of its diverse clinical manifestations and constrained availability of ADAMTS13 testing. Clinical prediction scores have been developed to estimate the pretest probability of severe ADAMTS13 deficiency, however, they all include the presence of MAHA. Atypical presentation of TTP has been previously acknowledged however continues to remain under-recognized.

The Need for Primary Care Beyond the Postnatal Period: Uncontrolled Hypertension-Induced Thrombotic Microangiopathy in a 32-Year-Old With a History of Preeclampsia Presenting Three Months Postpartum.

A 32-year-old African American female with a past medical history of uncontrolled hypertension and preeclampsia with severe features presented to the emergency department with three days of shortness of breath, chest pain, bloody cough, and non-bloody diarrhea without any known prior viral syndrome. On presentation, she was diagnosed with a hypertensive emergency with renal and cardiac dysfunction. Laboratory workup revealed leukocytosis, normocytic anemia, and thrombocytopenia. The remainder of the laboratory data were significant for hemolysis. Differential diagnosis included thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome (HUS); therefore, the patient was initiated on TTP treatment of pulsed dose steroids and plasma exchange. However, once the ADAMTS13 test returned negative, plasma exchange was stopped and the patient's profile returned to normal with supportive care and aggressive blood pressure control, as she had hypertension-induced thrombotic microangiopathy.

Semi-selective plasma filtration applied to the treatment of acquired thrombotic thrombocytopenic purpura following bnt162b2 administration.

Following the widespread use of anti SARS-CoV-2 vaccines, there have been reports of thrombocytopenia developing after the administration of different types of vaccine. We report a case of a 63-year-old male who developed neurological symptoms after receiving the second dose of the bnt162b2 vaccine. Blood tests performed upon admission to the Emergency Department revealed severe thrombocytopenia and microangiopathic hemolytic anemia. ADAMTS13 activity was undetectable and antibody titer was high. Due to the rapid neurological deterioration, steroid therapy with prednisone was started at an initial dose of 1 mg/kg/day. Rituximab therapy was started to prevent the formation of new antibodies. Given the slow response to this therapy, we added Caplacizumab, (a monoclonal antibody anti-Von Willebrand factor) in order to inhibit platelet hyperaggregation, combined with standard plasma exchange. The patient experienced repeated episodes of intolerance to fresh frozen plasma (FFP). Switching from plasma exchange to plasma filtration, remission was attained in this unusual case of vaccine-related thrombocytopenia with microangiopathic hemolytic anemia.

Targeting the innate repair receptor axis via erythropoietin or pyroglutamate helix B surface peptide attenuates hemolytic-uremic syndrome in mice.

Hemolytic-uremic syndrome (HUS) can occur as a systemic complication of infections with Shiga toxin (Stx)-producing Escherichia coli and is characterized by microangiopathic hemolytic anemia and acute kidney injury. Hitherto, therapy has been limited to organ-supportive strategies. Erythropoietin (EPO) stimulates erythropoiesis and is approved for the treatment of certain forms of anemia, but not for HUS-associated hemolytic anemia. EPO and its non-hematopoietic analog pyroglutamate helix B surface peptide (pHBSP) have been shown to mediate tissue protection via an innate repair receptor (IRR) that is pharmacologically distinct from the erythropoiesis-mediating receptor (EPO-R). Here, we investigated the changes in endogenous EPO levels in patients with HUS and in piglets and mice subjected to preclinical HUS models. We found that endogenous EPO was elevated in plasma of humans, piglets, and mice with HUS, regardless of species and degree of anemia, suggesting that EPO signaling plays a role in HUS pathology. Therefore, we aimed to examine the therapeutic potential of EPO and pHBSP in mice with Stx-induced HUS. Administration of EPO or pHBSP improved 7-day survival and attenuated renal oxidative stress but did not significantly reduce renal dysfunction and injury in the employed model. pHBSP, but not EPO, attenuated renal nitrosative stress and reduced tubular dedifferentiation. In conclusion, targeting the EPO-R/IRR axis reduced mortality and renal oxidative stress in murine HUS without occurrence of thromboembolic complications or other adverse side effects. We therefore suggest that repurposing EPO for the treatment of patients with hemolytic anemia in HUS should be systematically investigated in future clinical trials.

Refractory Thrombotic Thrombocytopenic Purpura to Therapeutic Plasma Exchange.

Thrombotic thrombocytopenic purpura (TTP) is a rare, potentially fatal hematologic disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and varying signs of visceral ischemia secondary to microvascular thrombosis. TTP is caused by a severe deficiency of ADAMTS13, a protease enzyme responsible for cleaving von Willebrand-factor (vWF) multimers. First-line therapy with plasmapheresis has increased survival rates immensely; however, there are few reported cases that are refractory to standardized treatment. We describe two cases of refractory TTP successfully managed with the addition of caplacizumab, an anti-von Willebrand factor immunoglobulin fragment that inhibits the interaction of vWF multimers with platelets.

Therapeutic plasma exchange in gastric signet ring cell carcinoma presenting as microangiopathic hemolytic anemia: A rare case report.

Microangiopathic hemolytic anemia (MAHA) defines a group of disorders characterized by the formation of microthrombi in capillaries and arterioles and the fragmentation of erythrocytes that pass through. Cancer-related MAHA is a rare but serious condition that is encountered in patients diagnosed with a malignancy. This clinical picture is thought to be linked to certain tumor characteristics; particularly, adenocarcinoma histology, vascular invasion, and bone marrow infiltration. MAHA is most commonly associated with tumors of gastric, prostate, and breast origin. The optimal treatment is not clear; however, there is evidence for the importance of promptly starting an effective antineoplastic regimen and it was also reported that administering therapeutic plasma exchange (TPE) therapy for immunocomplex removal could be beneficial for patients with symptoms of bleeding and thrombosis. Here, we present a case that presented a picture of MAHA secondary to gastric signet-ring cell adenocarcinoma (SRCC). The clinical picture was initially evaluated as thrombotic thrombocytopenic purpura and the patient benefited significantly from the TPE treatment administered before the adenocarcinoma diagnosis was confirmed. In this period, epistaxis stopped, platelet count increased from 25 × 109 /L to 162 × 109 /L, fragmented erythrocyte rate in the peripheral smear decreased by more than 75% and other laboratory findings of hemolysis (LDH, bilirubin, etc.) significantly improved.

The Challenges in Diagnosis and Management of Acquired Thrombotic Thrombocytopenic Purpura: Consensus Report from Three Gulf Countries.

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare hematological emergency characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, and multiorgan failure due to autoimmune-mediated deficiency in ADAMTS-13 activity. Currently, plasma exchange, with or without steroids, is the frontline option for the management of aTTP. The treatment should be started promptly once the disorder is clinically suspected. Besides, immunomodulators were studied in patients with aTTP to achieve stable remission and reduce the risk of relapse in patients with suboptimal response to plasma exchange; however, clinical trials showed equivocal results. Published data on early diagnosis, referral, and treatment patterns of aTTP patients in the member nations of the Arabian Gulf Cooperation Council (GCC) are still lacking. Therefore, the present consensus report aimed to present an overview of aTTP situation in GCC by bringing together a panel of experts from three GCC nations, to share their views on current trends and practices regarding aTTP. The experts discussed challenges including the lack of reliable data regarding the incidence of aTTP in GCC and delayed results of ADAMTS-13 activity testing. Limited patient access to tertiary centers and low level of awareness about the aTTP clinical spectrum among general practitioners are other challenges. The experts agreed that there is a need for national and regional consensus regarding the diagnosis and treatment of aTTP in the Gulf region.

Recurrent microangiopathic hemolysis after recovery from complement-mediated hemolytic uremia syndrome during chemotherapy for a CFH-mutated patient with T-lymphoblastic lymphoma.

Complement-mediated hemolytic uremic syndrome (CM-HUS) following chemotherapy for pediatric acute lymphoid neoplasms has rarely been reported. We report the case of an 8-year-old boy with T-lymphoblastic lymphoma (T-LBL) who developed CM-HUS with complement factor H (CFH) mutations (S1191L, V1197A) during induction therapy. Safe administration of chemotherapy after CM-HUS recovery was challenging. By closely monitoring hemolytic and renal parameters during the 2-year treatment period, we observed four episodes of microangiopathic hemolytic anemia (MAHA) with hypocomplementemia and low haptoglobin but no renal dysfunction or thrombocytopenia. Here, we describe the MAHA and CM-HUS episodes in the hopes of elucidating the complex pathophysiology of disorders associated with CFH mutation.