Implementation of a rapid assay of ADAMTS13 activity was associated with improved 30‐day survival rate in patients with acquired primary thrombotic thrombocytopenic purpura who received platelet transfusions
Abstract:Our results indicate that PLT transfusions are harmful for aTTP patients when the definite diagnosis of severe ADAMTS13 deficiency is delayed. If it can be done as soon as possible, PLT transfusions for severe bleeding or surgical interventions might be allowed with subsequent plasmapheresis.
“…Thus, TTP is a medical emergency and rapid diagnosis and prompt treatment is critical 4 . Reduced ADAMTS13 activity of < 10% confirms the diagnosis of TTP; 1,5 however, this is a send‐out test at most institutions and results may not be available for several days 6–9 . In this setting, several clinical screening tools have been developed to guide clinical decisions and rapid recognition of TTP when ADAMTS13 tests are not immediately available.…”
Section: Introductionmentioning
confidence: 99%
“…4 Reduced ADAMTS13 activity of < 10% confirms the diagnosis of TTP; 1,5 however, this is a send-out test at most institutions and results may not be available for several days. [6][7][8][9] In this setting, several clinical screening tools have been developed to guide clinical decisions and rapid recognition of TTP when ADAMTS13 tests are not immediately available. Of these, the PLASMIC and French TTP scores are the best known and most widely applied.…”
Background: Thrombotic thrombocytopenic purpura (TTP) is a lifethreatening disorder characterized by thrombocytopenia, microangiopathic hemolysis, and ischemic organ failure. The PLASMIC and French TTP scores can help guide clinical decisions when ADAMTS13 testing is not immediately available. Older individuals often present atypically, but the impact of age on these tools is not known. Study Design and Methods: We calculated the sensitivity and specificity of the PLASMIC and French TTP scores in patients enrolled in the Johns Hopkins thrombotic microangiopathy (TMA) registry. Results: Of 257 patients with TMA enrolled in the registry, we excluded patients less than 18 years of age (n = 19), with prior TMA (n = 81) or who initially presented at another hospital (n = 25). The remaining 132 patients (75 with TTP and 57 with other TMA) were analyzed. Sensitivity of a French score of 2 decreased with age and was 72.2%, 61.5%, and 46.2% for ages 18 to 39, 40 to 59, and ≥ 60 years old, respectively. A PLASMIC score ≥ 5 had higher sensitivity than the French score but this also decreased with age; sensitivity was 91.4% (95% confidence interval [CI], 76.9-98.2), 78.3% (95% CI, 56.3-92.5), and 76.9% (95% CI, 46.2-95.0) for patients 18 to 39, 40 to 59, and ≥ 60 years old, respectively. Older patients had higher platelet counts and serum creatinine than the youngest group, contributing to the loss in sensitivity. Conclusion: The PLASMIC and French TTP scores have reduced sensitivity at age ≥ 60 years and are less reliable in identifying TTP in older patients. A high index of suspicion and availability of rapid ADAMTS13 assays is required to correctly diagnose all patients with TTP.
“…Thus, TTP is a medical emergency and rapid diagnosis and prompt treatment is critical 4 . Reduced ADAMTS13 activity of < 10% confirms the diagnosis of TTP; 1,5 however, this is a send‐out test at most institutions and results may not be available for several days 6–9 . In this setting, several clinical screening tools have been developed to guide clinical decisions and rapid recognition of TTP when ADAMTS13 tests are not immediately available.…”
Section: Introductionmentioning
confidence: 99%
“…4 Reduced ADAMTS13 activity of < 10% confirms the diagnosis of TTP; 1,5 however, this is a send-out test at most institutions and results may not be available for several days. [6][7][8][9] In this setting, several clinical screening tools have been developed to guide clinical decisions and rapid recognition of TTP when ADAMTS13 tests are not immediately available. Of these, the PLASMIC and French TTP scores are the best known and most widely applied.…”
Background: Thrombotic thrombocytopenic purpura (TTP) is a lifethreatening disorder characterized by thrombocytopenia, microangiopathic hemolysis, and ischemic organ failure. The PLASMIC and French TTP scores can help guide clinical decisions when ADAMTS13 testing is not immediately available. Older individuals often present atypically, but the impact of age on these tools is not known. Study Design and Methods: We calculated the sensitivity and specificity of the PLASMIC and French TTP scores in patients enrolled in the Johns Hopkins thrombotic microangiopathy (TMA) registry. Results: Of 257 patients with TMA enrolled in the registry, we excluded patients less than 18 years of age (n = 19), with prior TMA (n = 81) or who initially presented at another hospital (n = 25). The remaining 132 patients (75 with TTP and 57 with other TMA) were analyzed. Sensitivity of a French score of 2 decreased with age and was 72.2%, 61.5%, and 46.2% for ages 18 to 39, 40 to 59, and ≥ 60 years old, respectively. A PLASMIC score ≥ 5 had higher sensitivity than the French score but this also decreased with age; sensitivity was 91.4% (95% confidence interval [CI], 76.9-98.2), 78.3% (95% CI, 56.3-92.5), and 76.9% (95% CI, 46.2-95.0) for patients 18 to 39, 40 to 59, and ≥ 60 years old, respectively. Older patients had higher platelet counts and serum creatinine than the youngest group, contributing to the loss in sensitivity. Conclusion: The PLASMIC and French TTP scores have reduced sensitivity at age ≥ 60 years and are less reliable in identifying TTP in older patients. A high index of suspicion and availability of rapid ADAMTS13 assays is required to correctly diagnose all patients with TTP.
“…Regarding the definition of TMA, TMA presents with microangiopathic hemolytic anemia (MHA), including hemolytic anemia, thrombocytopenia and organ failure in the kidney, central nervous system, and other organs [ 3 , 4 ]. These findings suggest that marked elevation of FRMs is required in DIC while MHA is required in TMA; the diagnosis of TTP among TMA requires a markedly decreased ADAMTS13 level [ 14 ], that of STEC-HUS requires the detection of a STEC infection [ 15 ] and that of aHUS requires the detection of abnormalities in the complement system [ 16 ]. Fig.…”
IntroductionBoth disseminated intravascular coagulation (DIC) and thrombotic microangiopathy (TMA) cause microvascular thrombosis associated with thrombocytopenia, bleeding tendency and organ failure.Reports and discussionThe frequency of DIC is higher than that of thrombotic thrombocytopenic purpura (TTP). Many patients with TMA are diagnosed with DIC, but only about 15% of DIC patients are diagnosed with TMA. Hyperfibrinolysis is observed in most patients with DIC, and microangiopathic hemolytic anemia is observed in most patients with TMA. Markedly decreased ADAMTS13 activity, the presence of Shiga-toxin-producing Escherichia coli (STEC) and abnormality of the complement system are useful for the diagnosis of TTP, STEC-hemolytic uremic syndrome (HUS)and atypical HUS, respectively. However, there are no specific biomarkers for the diagnosis of DIC.ConclusionAlthough DIC and TMA are similar appearances, all coagulation, fibrinolysis and platelet systems are activated in DIC, and only platelets are markedly activated in TMA.
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