1. Which of the following is not part of the diagnostic criteria for TAMOF?
a) Plt count <100K
b) Organ failure index ≥ 2
c) PTT >1.5x normal
d) ADAMTS13 level <57%
2. Which drug is used to treat atypical HUS?
3. A 15 yo M is admitted to the PICU with a diagnosis of meningococcemia after presenting with fever, altered mental status, hypotension, and purpura. He is started on ceftriaxone and vancomycin, receives 60 cc/kg of NS as boluses, and a R IJ line is placed. The nurse calls you and states that he is oozing from his IV sites and the R IJ line. You suspect that he has DIC and want to send labs to evaluate. Which of the following labs is the most sensitive for DIC?
a) Decreased platelet count
c) prolonged PTT
d) low fibrinogen
ANSWERS & EXPLANATIONS
1. C. The PTT is not part of the diagnostic criteria for TAMOF. In general, an organ failure index ≥2 and a platelet count <100K are needed to define TAMOF. ADAMTS13 should be below 57% as this is thought to be one of the primary driving mechanisms behind TAMOF and a large part of the rationale for treatment with therapeutic plasma exchange.
2. A. Eculizumab is a terminal complement inhibitor and is used in the treatment of atypical HUS. Atypical HUS is a genetic disorder that leads to uncontrolled activation of the complement system with subsequent symptoms of systemic thrombotic microagniopathy.
3. While DIC can cause all of the above laboratory derangement, thrombocytopenia is the most sensitive indicator for DIC. Thrombocytopenia was followed by increased fibrin degradation products, prolonged PT, prolonged aPTT, and low fibrinogen in terms of sensitivity for DIC.