image by: HWN

HWN Suggests

Tasty Morsels of EM 070 – TTP

For us in ED it’s all about recognising it as a potential differential. It’s a disease of microthrombi formation (platelets are low from consumption) which causes the end organ damage and haemolysis as the red cells get damaged squeezing past the microthrombi. There’s something called ADAMSTS13 that does something to von willebrand’s factor but honestly I couldn’t follow it and couldn’t see the relevance to what we do…

read full article

Featured

 Thrombotic Thrombocytopenic Purpura: Pearls and Pitfalls

In any patient with MAHA and thrombocytopenia, consider TTP and consult hematology. This is the most important aspect of this disease! Lab findings will include low hemoglobin, low platelets, elevated LDH, potentially a decrease in renal function and electrolyte abnormalities, and elevated bilirubin (indirect). A peripheral smear is vital for diagnosis, as 100% of patients will have schistocytes present during the course of the disease.

 Treat TTP Within Eight Hours

Acquired TTP usually presents as severe microangiopathic hemolytic anemia (MAHA) and thrombocytopenia in a previously healthy individual. The classic pentad of MAHA, thrombocytopenia, fever, acute renal failure, and severe neurologic findings is rare.

 TTP-The Treatment Pathway

In view of the high risk of preventable, early deaths in TTP, treatment with plasma exchange (PEX) should be initiated as soon as possible, preferably within 4-8 hours, regardless of the time of day at presentation, if a patient presents with a MAHA and thrombocytopenia in the absence of any other identifiable clinical cause.

Articles of Interest

Best Case Ever 53 – TTP

Thrombotic Thrombocytopenic Purpura, that rare but often elusive clotting disorder that picks off multiple organs and has a near 100% mortality rate without treatment…

Episode 45: Thrombotic Thrombocytopenic Purpura

TTP is a clinical diagnosis: The classic pentad is rare; suspect TTP in a sick-appearing patient with MAHA and thrombocytopenia. Elevated LDH is also common.

Thrombocytopenia – TTP Or DIC – Help From Dr. DeLoughery + EmCrit

In this post I’m going to link to two outstanding vidcasts from Dr. Tom DeLoughery and EmCrit on thrombocytopenia...

Thrombotic microangiopathies (including TTP, ST-HUS, and C-HUS)

Fresh frozen plasma contains ADAMTS13, so this may be beneficial. If plasma exchange is going to be delayed (e.g., >6-8 hours), it may be helpful to give 2 units of fresh frozen plasma, followed by ~1 unit every four hours. Consider administration of a diuretic along with plasma, to avoid volume overload.

Resources

Life in the Fastlane

Thrombotic Thrombocytopenic Purpura (TTP) is a rare life-threatening condition that resembles HUS, the distinction is important because TTP can be treated with plasmapheresis.

Kings County EM

TTP is a rare diagnosis and a hard diagnosis to make. The mainstay of treatment is plasmapheresis.