Thrombotic Thrombocytopenic Purpura Associated with Human Immunodeficiency Virus Type 1 (HIV-1) Infection

Leaf, Andrea

doi:10.7326/0003-4819-109-3-194

Cited by 129 publications

(46 citation statements)

References 12 publications

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“…Many TTP cases are idiopathic. However, some cases may occur in association with autoimmune diseases [3], infection [4], pregnancy [5], bone marrow transplantation [6], and certain drugs such as cyclosporine A, tacrolimus, mitomycin, or ticlopidine [7], which were not suggested in this patient's history. TTP is commonly associated with a deficiency of ADAMTS13, the von Willebrand factor-cleaving plasma metalloprotease that cleaves the von Willebrand factor at the Tyr1605-Met1606 peptide bond in the central A2 subunit [8,9].…”

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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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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“…Plasma exchange therapy reduces the mortality rate to approximately 20%. 4 Although many cases are idiopathic, TTP sometimes occurs in association with pregnancy 5 ; autoimmune diseases 6 ; infection 7 ; drugs such as cyclosporine A, FK506, mitomycin, or ticlopidine 8 ; and bone marrow transplantation. 9 This clinical heterogeneity poses a challenge for understanding the pathogenesis of TTP and selecting appropriate therapies.…”

Section: Introductionmentioning

confidence: 99%

Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura

Zheng

Kaufman

Goodnough

et al. 2004

Blood

427

407

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Therapeutic plasma exchange is an effective empiric treatment for thrombotic thrombocytopenic purpura (TTP), but how therapy affects the level of a disintegrin and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) or inhibitor has not been reported in many patients. We prospectively analyzed ADAMTS13 activity and inhibitor levels in 37 adults with TTP. ADAMTS13 level at presentation was lower than 5% in 16 of 20 patients with idiopathic TTP and in none of 17 patients with TTP associated with hematopoietic stem cell transplantation, cancer, drugs, or pregnancy (P < .00001). Seven of the 16 patients with ADAMTS13 activity lower than 5% (Ϸ 44%) had inhibitors. For 8 patients followed serially with ADAMTS13 activity lower than 5% but no inhibitor at presentation, plasma exchange led to complete clinical remission and a rise in ADAMTS13 level. In contrast, 4 patients with low ADAMTS13 activity but high-titer inhibitor (> 5 units/mL) had neither a rise in ADAMTS13 activity nor a reduction in the inhibitor titer: 3 had recurrent disease and 1 died. Among 17 patients with AD-AMTS13 activity at presentation higher than 25%, 10 died. Mortality rate for idiopathic TTP was 15%, whereas mortality for nonidiopathic TTP was 59% (P < .02). We conclude that assays of ADAMTS13 activity and inhibitors in addition to the clinical categories (idiopathic TTP and nonidiopathic TTP) are predictive of outcome and may be useful to tailor patient treatment. (Blood. 2004;103:4043-4049)

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“…Thrombocytopenia can follow HIV seroconversion, resulting in intracranial hemorrhage [15]. During the course of AIDS, thrombocytopenia may result from thrombotic thrombocytopenic purpura or disseminated intravascular coagulation [16]. Hemorrhagic infarcts are reported to be less frequent than nonhemorrhagic infarcts, but when they do occur, it is usually in association with thrombocytopenia [4].…”

Section: Discussionmentioning

confidence: 99%

Stroke and Pediatric Human Immunodeficiency Virus Infection

Narayan¹,

Samuels²,

Barrow³

2002

Pediatr Neurosurg

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Stroke is an uncommon complication in pediatric patients with acquired immunodeficiency syndrome (AIDS). However, with the increasing life span of this patient population, more cases of stroke are being reported. We report the case of a 13-year-old girl with a known history of AIDS who presented with new-onset seizures and right hemiparesis. Serial imaging studies revealed progressive occlusion of the left middle cerebral artery and both anterior cerebral arteries with development of collateral circulation. The workup for other etiologies of stroke was negative. This nonatherosclerotic occlusive disease is most likely secondary to vasculopathy caused by the human immunodeficiency virus (HIV). HIV infection should be included in the differential diagnosis of children who present with seizures, mental status change or focal neurological deficits. Treatment options are limited. The different prognoses associated with the etiologies of stroke in this patient population mandate a careful and thorough evaluation.

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Thrombotic Thrombocytopenic Purpura Associated with Human Immunodeficiency Virus Type 1 (HIV-1) Infection

Cited by 129 publications

References 12 publications

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

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

Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura

Stroke and Pediatric Human Immunodeficiency Virus Infection

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