Atypical presentations of thrombotic thrombocytopenic purpura: A review

Sarode, Ravindra

doi:10.1002/jca.20182

Cited by 42 publications

(39 citation statements)

References 37 publications

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“…In our patient, in addition to the methylprednisolone, plasma exchange was started when the diagnosis of TTP was done, and as previously reported by other authors [9,10], a rapid improvement of haemolysis and a normalization of platelet count were seen.…”

Section: Discussionsupporting

confidence: 81%

Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

Aridon¹,

Ragonese²,

Mazzola³

et al. 2011

Neurol Sci

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Thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterised by fever, microangiopathic haemolytic anaemia, renal insufficiency, and thrombocytopenia. Neurological involvement, a prominent component of TTP, is characterised by a variety of brain lesions which include reversible cerebral oedema or magnetic resonance imaging (MRI) features of reversible posterior leukoencephalopathy syndrome (RPLS). TTP is frequently associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13.Here, we report a case of TTP with severe acute encephalopathy. Posterior leukoencephalopathy and brainstem oedema with triventricular hydrocephalus were observed on MRI. The low activity of ADAMTS13 was not observed and ADAMTS-13 antibodies were absent. Neurological symptoms and patient's condition were completely resolved by plasma exchange therapy in addition to high dose of methylprednisolone.

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Section: Discussionsupporting

confidence: 81%

Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

Aridon¹,

Ragonese²,

Mazzola³

et al. 2011

Neurol Sci

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“…This induces systemic platelet aggregation, microvascular thrombosis, intravascular hemolysis from fibrin deposition, and mechanical trauma. 8 Signs of end-organ damage Acute ischemic brain infarct is demonstrated by sagittal views of diffusion-weighted MRI sequence corresponding to a conspicuous area of restricted diffusion in the right insular cortex. Punctate foci of restricted diffusion were also seen in the right medial temporal lobe and right occipital cortex.…”

Section: Oy-stermentioning

confidence: 99%

Pearls & Oy-sters: Acute ischemic stroke caused by atypical thrombotic thrombocytopenic purpura

et al. 2013

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“…In recent literature, atypical TTP has been described in patients presenting with strokes or myocardial infarction with normal or near normal, laboratory values, and a low ADAMTS13 activity [16][17][18][19][20]. Downes et al [16] reported two interesting cases of atypical TTP.…”

Section: Commentarymentioning

confidence: 99%

Atypical thrombotic thrombocytopenic purpura in a middle‐aged woman who presented with a recurrent stroke

Idowu

Reddy

2012

American J Hematol

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A 48-year-old African American woman was brought to the emergency department by ambulance for evaluation of acute onset of altered mental status and neurological deficits. She was found leaning over the side of her bathtub with aphasia, left-sided facial droop, and left hemiparesis, suggestive of a stroke. Prior to this event, she was able to walk and perform daily activities. At baseline, she had mild dysarthria, minimal expressive aphasia, and right upper extremity weakness and spasticity. These were residual neurological deficits from the cerebrovascular accident (CVA) she had 6 years ago. Her past medical history included ischemic strokes 8 years ago associated with post-CVA seizures, another ischemic stroke 6 years ago, and thrombotic thrombocytopenic purpura (TTP). On physical examination, she appeared lethargic, disoriented to person, place, and time; she was unable to name objects or repeat words; and she was unable to follow commands. She was found to have a left facial droop, partial right-sided gaze preference, left hemianopia, and spastic left hemiparesis. A CT scan of the head without contrast showed two previous middle cerebral artery (MCA) territory cerebral infarctions with an acute right MCA occlusion at the origin of the M2 segment. An additional thrombus was present in a branch of the right MCA along the distribution of the posterior insular cortex. An MRI of her brain revealed a large area of acute ischemia within the distribution of the anterior division of right MCA, small foci of petechial hemorrhage in the right insula around the area of ischemia, and small foci of acute ischemia in the right cerebellar hemisphere. A CT angiogram confirmed these findings.This patient presented with acute stroke involving the distribution of the right MCA. Her vital signs on admission were within normal limits. Given that her clinical presentation was indicative of a stroke and having arrived within 3 hr of the onset of her symptoms, she was given pharmaceutical tissue plasminogen activator (tPA). Intra-arterial thrombolysis and thrombectomy were performed; however, her neurological symptoms did not improve.Her family history was negative for premature strokes or thrombotic disorders. The patient did not smoke, drink alcohol, or use illicit drugs. Her home medications included Keppra and Dilantin for seizure prophylaxis. A transesophageal echocardiogram performed on admission was unremarkable. Her lipid profile and HgbA1c were within normal limits. Admission laboratory data included hemoglobin (Hgb) 13.2 g/dl, hematocrit (Hct) 38.6%, and platelet (PLT) count of 113,000 per cubic mm of whole blood. Other laboratory values included lactate dehydrogenase (LDH) 238 U/l (normal range, 98-192 U/l), reticulocyte count 1.6%, international normalized ratio (INR) 1.22, prothrombin time (PT) 15.6 sec, activated partial thromboplastin time (aPTT) 26.7 sec, blood urea nitrogen (BUN) 6 mg/dl, and serum creatinine 0.8 mg/dl. Peripheral blood smear (PBS) did not show schistocytes. Patient's neurological deficits and alter...

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Atypical presentations of thrombotic thrombocytopenic purpura: A review

Cited by 42 publications

References 37 publications

Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

Pearls & Oy-sters: Acute ischemic stroke caused by atypical thrombotic thrombocytopenic purpura

Atypical thrombotic thrombocytopenic purpura in a middle‐aged woman who presented with a recurrent stroke

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