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Background: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a microangiopathy often characterized by acute neurological involvement including ischemic stroke (IS). The characteristics of IS in iTTP remain largely unknown., Aims: To evaluate the epidemiology, neuroimaging patterns and risk factors of IS in iTTP patients., Methods: We performed a cross-sectional study of patients enrolled in the Milan TTP Registry presenting with neurological signs/symptoms and underwent neuroimaging evaluation during their first acute iTTP episode., Results: Seventy-eight patients were enrolled, the majority of patients were female (72 %), with a median age of 46 years. Computed tomography (CT) was performed in all patients, and magnetic resonance (MRI) was performed in 38 % of patients. IS was confirmed in 18 out of 78 patients (23 %), most of whom (70 %) showed a non-lacunar pattern with multifocal involvement. In the subgroup of patients who had MRI (n = 30), IS was identified in 12 patients (40 %) and of them 6 (50 %) had a false negative result with CT scan. Patients with IS were slightly older than those without, whereas the prevalence of cardiovascular risk factors and iTTP-related parameters were comparable between the two groups., Conclusion: 23 % of patients presenting with neurological manifestations at their first acute TTP episode, showed brain IS. As expected, MRI showed higher sensitivity in detecting ischemic lesions underscoring its usefulness over CT in this setting. An unexpected prevalence of non-lacunar and multifocal stroke patterns warrants further investigation. Cardiovascular risk factors and iTTP-related clinical and laboratory parameters were similarly distributed in patients with and without IS., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: P. Agosti, A. Artoni, and B. Ferrari received honoraria for participating as a speaker at educational meetings organized by Sanofi; I. Mancini received honoraria for participating as a speaker at educational meetings organized by Werfen and Sanofi; F. Peyvandi has received honoraria for participating as a speaker in education meetings organized by Sanofi, Spark and Takeda and she is member of scientific advisory boards of CSL Behring, Biomarin, Roche, Sanofi, Sobi. The other authors do not have any conflict of interests to disclose., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare and fatal thrombotic microangiopathy-based hematologic disease. Stroke has been reported as atypical neurological manifestations of TTP in some cases., Case Presentation: A 65-year-old male presented with typical acute ischemic stroke symptoms including sudden-onset dysarthria, right-sided facial paralysis and hemiplegia. However, his CT and MRI scans were negative without showing any new ischemic lesions. He was diagnosed with TTP with severe thrombocytopenia, mild anemia, increased LDH, and low ADAMTS-13 activity. The symptoms and positive signs were rapidly resolved after administrating the plasma exchange therapy., Conclusion: Clinicians should consider the possibility of TTP when a patient presents with acute stroke-like symptoms and thrombocytopenia, especially in an emergency room, either with or without new stroke lesions on the brain CT and MRI., (© 2023. The Author(s).)

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombomicroangiopathy caused by deficiency of ADAMTS13. Acute neurological involvement is well described, but its long-term impact requires evaluation. One-hundred thirty-one patients, following an acute TTP event, with severe headache or neurological symptoms had a cerebral MRI. Fifty-six percent had abnormal imaging, more commonly in patients with neurological symptoms than headaches only (80% vs. 18%, P < 0·0001). In remission, 27% (n = 35) reported persistent cognitive symptoms: specifically, impaired memory (66%), difficulty concentrating (26%), and word-finding difficulties not secondary to an acute stroke (26%). Sixty-five percent also reported depression and 55% reported anxiety, regardless of presenting neurology. The frontal lobe was disproportionally affected in patients with marked intellectual impairment, seen in 67% of patients compared to 19% of patients without intellectual impairment (P = 0·002). The primary MRI finding in these patients was hyperintense white matter lesions. An abnormal MRI was associated with a lower median verbal IQ (85 vs. 99, P = 0·02) and performance IQ (83 vs. 100, P = 0·02). In conclusion, neurological symptoms are frequently associated with an abnormal cerebral MRI scan, and white matter frontal lobe lesions are particularly significant, leading to marked intellectual impairment. Anxiety and depression were evident in over half of patients, regardless of neurological involvement at presentation., (© 2020 British Society for Haematology and John Wiley & Sons Ltd.)

Thrombotic thrombocytopenic purpura (TTP) is a microangiopatic thrombotic state associated with a deficiency on the cleavage function of the Von Willebrand factor polymers by a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13. We report a patient with relapsing TTP successfully treated with N-acetylcysteine (NAC) after failure of plasma exchange (PE) with steroids, rituximab, cyclophosphamide, vincristine, and azathioprine. A 51-year-old male who had an altered mental status while he was on rehabilitation for a previously treated TTP with a subsequent neurologic deficit. He was treated 7 days ago with PE plus steroids and subsequently discharged to our facility for rehabilitation. He was found to have a platelet level of 153,000/mm, hemoglobin decreased from 9.2 to 6.2 g/dL, creatinine raised from 1.0 to 2.4 mg/dL, and the peripheral smear showed schistocytes. A brain computed tomography showed a subacute infarction in the left frontal lobe and an abdominal-pelvic computed tomography disclosed a retroperitoneal hematoma. PE and steroids were started for 14 days. On day 15th, rituximab was added weekly for 10 cycles. A disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13 activity level was 95% without platelet count improvement. We started cyclophosphamide, then vincristine, and finally azathioprine. His platelet were maintained above 150,000/mm for a few days. He had several episodes of sepsis after every chemotherapeutic drug. On day 135th, NAC was commenced at 150 mg/kg for 10 days along with PE and low-dose steroids for 10 days. Complete recover of platelet count was achieved and the patient was successfully discharged. Relapsing TTP is often difficult to manage and may last longer than expected carrying several comorbidities and complications. PE plus steroids are the mainstay of TTP treatment and Rituximab is the drug of choice after they have failed. The patient had a complete remission after NAC therapy. Hence, NAC likely can be considered an earlier choice of treatment after rituximab, before the use of chemotherapeutic agents, considering its toxic and adverse effects.

Introduction: Multicentric Castleman's disease can mimic adult-onset Still disease. It is exceptionally associated with anasarca, thrombotic microangiopathy and dysautonomia., Case Report: We report a 32-year-old woman with an association of oligoanuria, anasarca, thrombotic microangiopathy with features compatible with adult-onset Still disease. The outcome was initially favorable with corticosteroids, immunoglobulins and plasmapheresis but with the persistence of relapses marked by severe autonomic syndrome and necessity of high dose corticosteroids. The diagnosis of mixed type Castleman's disease, HHV8 and HIV negative, was obtained four years after the onset of symptoms by a lymph node biopsy. The outcome was favorable after tocilizumab and corticosteroids but tocilizumab had to be switched to anakinra to ensure a proper and long-lasting control of the disease., Conclusion: Our patient partially fits the description of TAFRO syndrome (Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly), a MCM rare variant, recently described in Japanese patients., (Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Thrombotic thrombocytopenic purpura is an acute syndrome with abnormalities in multiple organ systems, which becomes manifest with microangiopathic hemolytic anemia and thrombocytopenia. The hereditary or acquired deficiency of ADAMTS-13 activity leads to an excess of high molecular weight von Willebrand factor multimers in plasma, leading to platelet aggregation and diffuse intravascular thrombus formation, resulting in thrombotic thrombocytopenic purpura. Thrombotic lesions occurring in TTP leads to ischemia and convulsion. Depending on the properties of the bony tissue, fractures are divided into three groups as traumatic, pathological, and stress fractures. A pathologic fracture is a broken bone caused by disease leading to weakness of the bone. This process is most commonly due to osteoporosis, but may also be due to other pathologies such as cancer, infections, inherited bone disorders, or a bone cyst. We herein report a case with a pathologic fracture due to convulsion secondary to thrombotic thrombocytopenic pupura. Thrombotic lesions occurring in TTP may lead to ischemia and convulsion, as in our patient and pathological fractures presented in our case report may occur as a result of severe muscle contractions associated with convulsive activity. Thrombotic thrombocytopenic pupura is a disease that involves many organ systems and thus may have a very wide spectrum of clinical presentations., (Copyright © 2014. Published by Elsevier Ltd.)

We report a case of thrombotic thrombocytopenic purpura (TTP) with cardiac involvement, imaged with Tc-99m depreotide. A 56-year-old man presented with fever, hematuria, and chest pain. Laboratory findings (angiopathic hemolytic anemia, thrombocytopenia, and uremia) were suggestive of TTP. Cardiac enzymes were elevated and diffuse left ventricular hypokinesis was demonstrated by echocardiography. Serum rheumatologic and virologic analysis were negative. A Tc-99m depreotide SPECT/CT study showed diffuse uptake in the myocardium, indicating inflammatory reaction to thrombotic/hemorrhagic myocardial damage. We suggest that Tc-99m depreotide imaging may reveal myocardial involvement in TTP; this could prompt further investigation for potential applications in myocarditis of other etiologies.

There are so few true haematological emergencies that they are often way down the list as a differential diagnosis. Those that do exist, however, can prove devastatingly fatal if left untreated and a case is presented of one such possible diagnosis, thrombotic thrombocytopenic purpura, which falls into the remit of many possible presenting conditions. Particularly prevalent in young adults, this condition must always be thought of in any patient presenting with confusion, renal impairment, fever or other neurological abnormalities.

We report on a 43-year-old woman who developed a fulminant thrombotic thrombocytopenic purpura (TTP) in the early course of an oral ciprofloxacin therapy. An acute bacterial meningo-encephalitis with disseminated intravascular coagulation was first suspected. She also presented with haemolytic anaemia and a severe thrombocytopenia. The lumbar puncture was delayed and only performed after platelets transfusions. CSF examination ruled out meningo-encephalitis. A diagnosis of TTP was made and she was given plasma exchanges. However, her neurological status worsened and the cranial CT revealed a subarachnoidal hemorrhage in left sylvian valley with diffuse oedema predominating in the left cerebral hemisphere and multiple ischemic lacunes. She died four days after admission.

We describe an extraordinary patient with overlap syndrome (systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis) having positive autoantibodies against Sm, double stranded DNA, DNA topoisomerase I, and centromere, together with rheumatoid factor. The patient had multiple organ involvement resulting from thrombotic microangiopathy that mimicked so-called normotensive scleroderma renal crisis, and died mainly of massive pulmonary hemorrhage caused by thrombotic thrombocytopenic purpura. The clinical presentations of the case support the concept of strong associations between disease-specific autoantibodies and clinical features.

Thrombotic thrombocytopenic purpura (TTP) is a rare complication of scleroderma (systemic sclerosis, SSc). In the 5 reports documenting the association of TTP and SSc, the TTP syndrome developed on a background of well established SSc. We describe a 51-year-old woman with a 5 month history of an evolving connective tissue disease syndrome who presented initially with TTP, followed 4 months later by limited cutaneous SSc and Raynaud's phenomenon.

Clinical-neuroimaging analysis of 12 thrombotic thrombocytopenic purpura (TTP) patients revealed a variety of brain lesions. These included reversible cerebral edema lesions with MRI features of reversible posterior leukoencephalopathy syndrome (RPLS). Most of the RPLS patients had hypertension and renal dysfunction, suggesting RPLS due to hypertensive encephalopathy. Prompt treatment usually led to neurologic recovery and disappearance of edematous lesions. Those with infarcts or hematomas had a poorer outcome. TTP should be added to the expanding spectrum of RPLS and hypertensive encephalopathy.

A patient with active thrombotic thrombocytopenic purpura underwent examination with In-111 troponolate labeled autologous platelets. A mean platelet survival of 1.2 days was found, comparable to formerly described studies using other methods. Furthermore, remarkable renal uptake of radioactivity consistent with abnormal platelet trapping was demonstrated. In the absence of any other signs of renal involvement, the conclusion was reached that the platelet scan is a sensitive and direct method for showing renal involvement in thrombotic thrombocytopenic purpura.

We studied the prognostic value of computed tomography (CT) of the brain for neurologic morbidity in patients with thrombotic thrombocytopenic purpura. On review of Mayo Clinic records for 1975 through 1985, we found 32 patients with thrombotic thrombocytopenic purpura, 20 of whom had undergone CT of the brain during their hospitalization. Despite major neurologic symptoms and signs, normal CT findings were associated with complete neurologic recovery. Seventy percent of patients with normal results of CT of the brain recovered and had no neurologic deficits, whereas 80% of patients with CT abnormalities died or had permanent neurologic sequelae. A review of the literature supports these conclusions. Thus, we suggest that CT of the brain be done in any patient with thrombotic thrombocytopenic purpura and neurologic deficits. Regardless of the severity of neurologic involvement, normal CT findings should encourage continued vigorous treatment of the patient because a normal scan supports the possibility of full clinical recovery.

Computed tomographic (CT) brain scan findings in a patient with severe thrombotic thrombocytopenic purpura (TTP) are described. Multiple scattered lucencies were noted which correlated with thrombotic occlusions found at autopsy. In this patient, the neurologic manifestations of TTP preceded the haemolytic anaemia. In the correct clinical setting, multiple areas of lucency should suggest multiple infarcts secondary to thrombotic vessel occlusions as a diagnostic possibility.

A 34-year old woman presented with the clinical and laboratory features of thrombotic thrombocytopenic purpura (TTP). Studies with isologous platelets labelled with 111In-oxine revealed a short half-life of circulating platelets (18,5 hours) and destruction of the transfused platelets in the spleen, liver and bone marrow. There was no scientigraphic evidence of deposition of labelled platelets in the vasculature. The patient was treated with daily fresh frozen plasma transfusions, but no improvement in platelet count or serum urea level was noted. Although there was no clinical evidence of a bleeding tendency at the time, the patient had a fatal cerebrovascular haemorrhage. The findings in this case suggest that an immune type destruction of platelets may occur in TTP.

Optimal labelling conditions of human platelets with 111In-oxine were determined in vitro. Based on this optimized technique, platelet mean life span (MLS) and platelet sequestration site were comparatively evaluated in 79 patients with two labels, 51Cr (n = 26) and 111In (n = 53). Patients were subgrouped according to clinical criteria as autoimmune thrombocytopenic purpura (AITP) (group 1; n = 49), hypersplenism (2; n = 12), impaired thrombopoiesis (3; n = 3), unclassified thrombocytopenia (4; n = 6), and nonthrombocytopenic patients (5; n = 9). In patients with AITP and hypersplenism the mean values for the MLS determined either with 51Cr or with 111In were lowered but the difference was not statistically significant, neither for group 1 (18.6 h vs 17.3 h; P greater than 0.2) nor for group 2 (94.7 vs 122.3 h; P greater than 0.2). The correlation between MLS and platelet counts in patients with AITP was significant for both labels (P less than 0.001). The 15 min recovery tended to be higher with 111In in all groups, but the difference was significant (P less than 0.05) only for group 1. The sequestration sites were similar with both labels. We conclude that, contrary to previous reports, platelet survival studies yield similar results with both the 51Cr and 111In methods. Due to its distinct advantages 111In is the label of choice for investigation of platelet kinetics.