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

Rojas, Julio C.; Banerjee, Chirantan; Siddiqui, Fazeel M.; Nourbakhsh, Bardia; Powell, Craig M.

doi:10.1212/wnl.0b013e318294b423

Cited by 21 publications

(17 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…While PLT transfusions could themselves be associated with thrombotic events, arterial thrombosis and ischemic stroke continue to remain significant independent predictors of mortality in TTP patients. There is recent evidence suggesting that cryptogenic stroke with vascular distributions suggesting a distal microembolic pattern and other neurovascular complications of TTP may actually develop before the onset of profound hematologic abnormalities . CNS bleeding is not commonly reported in TTP subjects.…”

Section: Discussionmentioning

confidence: 99%

Prognostic risk‐stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012

Goel

King

Takemoto³

et al. 2016

Transfusion

View full text Add to dashboard Cite

Background Despite proven efficacy and increased availability of therapeutic plasma exchange (TPE), mortality for patients with thrombotic thrombocytopenic purpura (TTP) remains high with a limited understanding of those at highest risk of death. Study Design and Methods This study utilized the Nationwide Inpatient Sample (2007–2012) to derive a prognostic score for mortality in hospitalized TTP patients. Odds ratios of death with various putative risk factors adjusted for age, gender and race were calculated (adjOR). Weighted average of adjOR estimates were incorporated in a risk stratified score. Results Among 8203 hospitalizations with TTP as primary admission diagnosis who underwent TPE, 613 deaths were identified (all-cause mortality 7.5%, median time-to-death 9 days with interquartile range 4–14 days). In multivariable logistic regression, arterial thrombosis (adjOR 6.7, 95%CI=1.1–40.9), intra-cranial hemorrhage (adjOR 6.1, 95%CI=1.6–23.2), age >=60 years (adjOR 3.5, 95%CI=2.1–5.6), renal failure (adjOR 2.6, 95%CI=1.5–4.5), ischemic stroke (adjOR 2.4, 95%CI=1.2–5.0), platelet transfusions (adjOR 2.2, 95%CI=1.2–4.1) and myocardial infarction (adjOR 2.3, 95%CI=1.2–4.6) were significant independent predictors of mortality in TTP patients who underwent TPE. A prognostic weighted mortality prediction scoring system incorporating arterial thrombosis, intracranial hemorrhage, age, renal failure, ischemic stroke, platelet transfusion and myocardial infarction showed very good discrimination and was predictive of 78.6% deaths. Conclusions Early and targeted therapy for high risk individuals should be used to guide management of TTP patients for improved survival outcomes.

show abstract

Section: Discussionmentioning

confidence: 99%

Prognostic risk‐stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012

Goel

King

Takemoto³

et al. 2016

Transfusion

View full text Add to dashboard Cite

show abstract

“…These correlate with the petechial cerebral hemorrhages observed on autopsy [13]. While occasional punctate foci of diffusion restriction may also be seen in TMAs, they are not as numerous as those seen in the starfield pattern of FES [14]. Medium and large vascular distributions are more likely to be infarcted during TMA [15][16][17], including that of the cerebral gray matter, brainstem, cerebellum [18] or the deep white matter [19].…”

Section: Discussionmentioning

confidence: 87%

Cerebral fat embolism syndrome mimicking thrombotic thrombocytopenic purpura in a patient with hemoglobin SC disease

2016

View full text Add to dashboard Cite

A 54-year-old man with hemoglobin SC disease and a history of substance abuse presented to the Emergency Department from a nursing home with 2 days of progressive weakness, shortness of breath, and lower back pain. He developed a fever, hypoxia, and tachycardia on the day of admission. He did not have any recent changes in medications, and his family as well as the nursing home staff denied any access to illicit drugs.The patient's clinical presentation raises concern for a vaso-occlusive crisis, specifically acute chest syndrome (ACS.) ACS should be suspected when a patient with a sickle cell disorder (SCD) experiences fever, chest pain, and dyspnea, with laboratory testing demonstrating the presence of a leukocytosis and an associated decrease in hemoglobin and platelets [1]. Supportive care with hydration, oxygen, pain control, and broad-spectrum antibiotics should be initiated while awaiting hematologic, metabolic, infectious, and radiographic workups.On arrival to the hospital, his vital signs demonstrated a temperature of 39.48C, a heart rate of 140 beats per minute, and a blood pressure of 159/120 mmHg. He was hypoxic and required supplemental oxygen. Physical exam was remarkable for altered mental status (AMS) and a rapidly decreasing level of consciousness, dry mucous membranes, dilated, slightly irregular, and nonreactive pupils, and a torsional nystagmus. A later review of records from his ophthalmologist documented the presence of iris atrophy as a complication of his sickle cell disease. Upon evaluation, the patient was intubated for airway protection.ACS and the systemic inflammatory response syndrome (SIRS) remain atop the differential diagnosis. Other important considerations include severe sepsis secondary to pneumonia or meningitis, illicit drug use, metabolic disturbances, heart failure, hypertensive emergency, and embolic diseases. In general, neurologic complications are seen in 11% of patients presenting with ACS, with 31% of those patients also developing a relative thrombocytopenia [2]. While there are many etiologies that might contribute to this clinical picture, one finding that stands out is his torsional nystagmus, indicating a dysfunction in his cerebellum or brainstem. With such a finding, a metabolic or ischemic cause for his acute encephalopathy becomes more likely.Initial complete blood count showed a decrease in hemoglobin from a baseline of 13-14 to 12.4 g/dl, a decrease in platelets from a baseline of 222 to 92 k/mm 3 , and serum WBCs of 11.8 k/ml. Peripheral blood smears showed moderate amount of target cells and sickle cells, a markedly elevated number of nucleated red blood cells (65 nucleated RBCs/100 WBCs), and a slight number of schistocytes.There also was evidence of neutrophil precursors with 3% neutrophil bands, 2% metamyelocytes, and 1% myelocytes. A comprehensive metabolic panel showed an elevation of serum creatinine from a baseline of 1.2 to 1.6 mg/dl and a total bilirubin of 3.4 mg/dl. His troponin I level was 0.96 ng/ml. Further hematological work-up demonstrat...

show abstract

“…7 As TTP is a thrombotic disease, the most common and feared causes of death are cerebral, renal and myocardial infarction. 4 Fifty% of cases will have central nervous complications, such as seizures, 8 and some cases have even reported neural sequelae preceding hematological findings 9 . Renal injury (indicated by elevated creatinine) occurs in 33% of patients.…”

Section: Discussion Epidemiologymentioning

confidence: 99%

“…7 With regards to laboratory findings, 95% of TTP cases will have thrombocytopenia with platelet counts <60,000. 9 Evidence of hemolysis should be found in the form of hyperbilirubinemia, elevated LDH, schistocytes on peripheral smear, and anemia.…”

Section: Discussion Epidemiologymentioning

confidence: 99%

Untitled

2016

HTIJ

View full text Add to dashboard Cite

Microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fever, renal failure, and neurologic symptoms comprise the cardinal features of thrombotic thrombocytopenic purpura (TTP). Etiologies can include medications, infections, cancers, or transplantation. However, recognition of thrombotic thrombocytopenic purpura can be difficult because of the variety of presentations and lack of specific diagnostic criteria. We are presenting a case of acute TTP following a bout of splenic infarction. This rare case reminds us that splenic infarction can be an atypical presentation of TTP. Prompt recognition of TTP is important because the disease responds well to plasma-exchange treatment which improves patient`s prognosis, and is associated with a high mortality rate when untreated.

show abstract

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

Cited by 21 publications

References 11 publications

Prognostic risk‐stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012

Prognostic risk‐stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012

Cerebral fat embolism syndrome mimicking thrombotic thrombocytopenic purpura in a patient with hemoglobin SC disease

Untitled

Contact Info

Product

Resources

About