Thrombotic microangiopathy presenting with stroke-like symptoms

Nasrollahi, Farrah; Eilbert, Wesley

doi:10.1016/j.ajem.2022.01.032

Cited by 2 publications

(3 citation statements)

References 7 publications

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“…The pathogenesis of TTP stems from low activity of ADAMTS13, a metalloproteinase involved in cleaving von Willebrand factor, resulting in large residual von Willebrand factor multimers causing platelet thrombi in the microvasculature and shearing of circulating red blood cells into schistocytes ( case 6-2 ). TTP may be hereditary (homozygous ADAMTS13 deficiency) or acquired from a range of systemic illnesses including infectious, toxic, metabolic, autoimmune, neoplastic, and pregnancy syndromes 41,42 . The classic pentad of TTP includes microangiopathic hemolytic anemia, thrombocytopenia, mild renal insufficiency, fever, and neurologic symptoms, although this full spectrum is seen less than 10% of the time 43 .…”

Section: Thrombotic Microangiopathies Including Thrombotic Thrombocyt...mentioning

confidence: 99%

“…43 In patients presenting within the clinical spectrum of TTP, the diagnosis is confirmed by the demonstration of microangiopathic hemolytic anemia and low plasma levels of ADAMTS13; the laboratory findings include schistocytes on peripheral blood smear (FIGURE , decreased serum haptoglobin, elevated serum lactate dehydrogenase, elevated serum reticulocytes, elevated serum unconjugated bilirubin, urobilinogen present on urinalysis, and blood present on urinalysis. 42 Prior to modern therapy, mortality for TTP was high, but now most instances of the disease are remittable with early recognition and treatment. Given the role of autoimmunity in most patients, treatment typically includes plasma exchange and corticosteroids.…”

Section: Key Pointsmentioning

confidence: 99%

“…TTP may be hereditary (homozygous ADAMTS13 deficiency) or acquired from a range of systemic illnesses including infectious, toxic, metabolic, autoimmune, neoplastic, and pregnancy syndromes. 41,42 The classic pentad of TTP includes microangiopathic hemolytic anemia, thrombocytopenia, mild renal insufficiency, fever, and neurologic symptoms, although this full spectrum is seen less than 10% of the time. 43 Nonetheless, TTP affects the nervous system in up to 60% to 70% of cases, manifesting with encephalopathy, headaches, seizures, or focal neurologic deficits.…”

Section: Key Pointsmentioning

confidence: 99%

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Hematologic Disorders and the Nervous System

Mauermann¹,

Southerland²

2023

CONTINUUM: Lifelong Learning in Neurology

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OBJECTIVE This article discusses the epidemiology, diagnosis, treatment, and prevention of neurologic complications of red blood cell, platelet, and plasma cell disorders. LATEST DEVELOPMENTS Cerebrovascular complications can occur in patients with blood cell and platelet disorders. Treatment strategies to prevent stroke are available for patients with sickle cell disease, polycythemia vera, and essential thrombocythemia. A diagnosis of thrombotic thrombocytopenic purpura should be considered in patients with neurologic symptoms, hemolytic anemia, thrombocytopenia, mild renal insufficiency, and fever. Plasma cell disorders can be associated with peripheral neuropathy, and classification of the monoclonal protein type and neuropathy aid in diagnosis. Patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) syndrome can present with arterial and venous neurologic events. ESSENTIAL POINTS This article discusses the neurologic complications of blood cell disorders and the most recent advances in prevention and treatment.

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Section: Thrombotic Microangiopathies Including Thrombotic Thrombocyt...mentioning

confidence: 99%

Section: Key Pointsmentioning

confidence: 99%

Section: Key Pointsmentioning

confidence: 99%

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Hematologic Disorders and the Nervous System

Mauermann¹,

Southerland²

2023

CONTINUUM: Lifelong Learning in Neurology

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Adult Vitamin B12 Deficiency-Associated Pseudo-Thrombotic Microangiopathy: A Systematic Review of Case Reports

Ganipisetti,

Maringanti,

Lingas

et al. 2024

Cureus

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Cobalamin-deficient thrombotic microangiopathy or vitamin B12 deficiency presenting as pseudo-thrombotic microangiopathy is a rare disorder that can be misdiagnosed as thrombotic thrombocytopenic purpura. Patients with this condition are at risk of receiving unnecessary plasmapheresis with a potential delay in appropriate therapy with vitamin B12 supplementation. There are no established diagnostic criteria for this condition in clinical practice. We performed a systematic review of case reports published between January 2018 and January 2023 to analyze the clinical characteristics, risk factors, and patterns of laboratory markers to improve the diagnostic criteria for this condition.

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Thrombotic microangiopathy presenting with stroke-like symptoms

Cited by 2 publications

References 7 publications

Hematologic Disorders and the Nervous System

Hematologic Disorders and the Nervous System

Adult Vitamin B12 Deficiency-Associated Pseudo-Thrombotic Microangiopathy: A Systematic Review of Case Reports

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