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.

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.

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.

Acute Thrombotic Microangiopathy and Cortical Necrosis Following Administration of Alemtuzumab: A Case Report.

Alemtuzumab, a humanized monoclonal antibody that targets CD52 antigens on lymphocytes and monocytes, has shown efficacy in preventing relapse in relapsing-remitting multiple sclerosis. Despite known severe (yet rare) renal side effects such as anti-glomerular basement membrane disease and membranous glomerulopathy, to our knowledge, alemtuzumab has never been documented to cause drug-induced thrombotic microangiopathy. We describe a 39-year-old woman with relapsing-remitting multiple sclerosis who developed acute kidney injury requiring renal replacement therapy after 1 dose of alemtuzumab, as well as microangiopathic hemolytic anemia and thrombocytopenia. Pathologic examination of a kidney biopsy specimen demonstrated extensive cortical necrosis and arteriolar fibrin thrombi with nonspecific immunofluorescence staining of immunoglobulin M and C3 and absence of immune deposits on electron microscopy. These findings were consistent with the diagnosis of acute thrombotic microangiopathy. She received dexamethasone and underwent plasmapheresis, which was unsuccessful at removing alemtuzumab. The patient received renal replacement therapy for approximately 7 weeks, followed by slow recovery of kidney function that returned close to her baseline.

Syndromes of Thrombotic Microangiopathy.

Thrombotic thrombocytopenia purpura (TTP) and the hemolytic uremic syndrome (HUS) are rare thrombotic microangiopathies that can be rapidly fatal. Although the acquired versions of TTP and HUS are generally highest on this broad differential, multiple rarer entities can produce a clinical picture similar to TTP/HUS, including microangiopathic hemolysis, renal failure, and neurologic compromise. More recent analysis has discovered a host of genetic factors that can produce microangiopathic hemolytic syndromes. This article discusses the current understanding of thrombotic microangiopathy and outlines the pathophysiology and causative agents associated with each distinct syndrome as well as the most accepted treatments.

Microangiopathic hemolytic anemia associated with metastatic breast cancer: case report and literature review.

INTRODUCTION: Microangiopathic hemolytic anemia (MAHA) is a mechanical hemolytic anemia characterized by the emergence of fragmented red cells in peripheral blood. Here, we report a case of breast cancer associated with cancer-related (CR)-MAHA along with a literature review. CASE DESCRIPTION: The patient was a 54-year-old woman who made an emergency visit to our hospital because of low back pain, shoulder pain, visual impairment, and anemia. She was diagnosed with stage IV, ER-positive, PgR-positive, HER2-negative left breast cancer (invasive lobular carcinoma), with left axillary adenopathy, metastasis to the soft tissue of the orbital region, multiple bone metastases, pleural dissemination, and metastasis to the stomach and para-aortic lymph nodes. Chemotherapy was initiated successfully; tumor marker levels normalized and the visceral metastases almost disappeared. Hormone therapy was administered for maintenance. Two and a half years later, rapid elevation in tumor marker levels and severe anemia were noted, and fragmented red cells and poikilocytes emerged in the peripheral blood. Positron emission tomography-computed tomography and bone scintigraphy revealed multiple bone metastases, but no evidence of visceral metastasis. CR-MAHA associated with multiple bone metastases was diagnosed, and Paclitaxel chemotherapy was initiated with frequent blood transfusions. Her anemia gradually improved, with a decrease in tumor marker levels and the number of blood transfusions. Three months later, tumor marker levels increased again. Because the anemia was also exacerbated, chemotherapy was changed to eribulin. Tumor marker levels temporally decreased, and the anemia tended to improve, but 3 months later, the levels were elevated again and the anemia was exacerbated. A switch to another regimen was planned, but best supportive care was chosen instead because of rapid deterioration of liver function. The patient died a month later. DISCUSSION AND EVALUATION: CR-MAHA is thought to have a different pathologic mechanism from TTP or HUS. Although CR-MAHA is a clinical condition associated with a very poor prognosis, we consider it controllable for long period by rapid introduction of chemotherapy in many cases. CONCLUSIONS: CR-MAHA is a nearly oncologic emergency that medical oncologists need to be able to recognize even though it rarely occurs in breast cancer.

Pathology Consultation on the Diagnosis and Treatment of Thrombotic Microangiopathies (TMAs).

OBJECTIVES: Pathologists specializing in transfusion medicine, apheresis medicine, and/or coagulation are often consulted by clinicians to reach a diagnosis for patients with thrombotic microangiopathy (TMA), so that disease-specific, often life-saving therapy can be initiated as promptly as possible. METHODS: This article describes how to proceed when treating a patient with TMA. The differential diagnosis is broad and potentially very challenging. Thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS), and typical hemolytic uremic syndrome (HUS) are three such TMAs that require timely diagnosis and treatment. RESULTS: TTP is treated with daily therapeutic plasma exchange (TPE) and commonly with adjunctive immunosuppressive therapy, while aHUS may initially be managed with TPE but is best controlled with eculizumab once a presumptive diagnosis is made. TPE has no proven role in typical HUS, which is most commonly treated with supportive measures only. CONCLUSIONS: Prompt and accurate diagnosis of TMA subtypes optimizes treatment and improves patient outcomes.

Disseminated gastric carcinoma in disguise-presentation as microangiopathic haemolytic anemia with bone marrow necrosis.

Cancer related microangiopathic hemolytic anemia (MAHA) and bone marrow necrosis (BMN) are two rare and distinct clinicopathological entities reported in patients with disseminated malignancy. When they emerge as initial findings in a previously undetected case of malignancy, the diagnosis is often missed resulting in inappropriate management. We report the extremely rare association of cancer related MAHA and BMN as the first presentation of unsuspected disseminated gastric carcinoma in a 63 years old male. Early institution of chemotherapy is the only effective option for both these conditions, though with little improvement in outcome. This emphasises the need for increased awareness of such presentations in advanced malignancies.