Today's case was a patient presenting with microangiopathic hemolytic anemia, most
likely thrombotic thrombocytopenic purpura (TTP) on the basis of either congenital/acquired
ADAMTS13 deficiency, or an experimental chemotherapy drug.
Important
learning points:
-Pathophysiologically,
TTP results from multimers of Von Willebrand Factor (I erroneously said
platelets) due to congenital or acquired deficiency of the enzymes responsible
for degrading said multimers, ADAMTS13
-The
classical clinical pentad of fever, altered mental status/seizures, renal
failure, thrombocytopenia, and hemolytic anemia is rarely seen all together.
-The
alterations in mentation are often fluctuating changes in LOC rather than
discrete focal neurologic symptoms.
-The
condition is 100% fatal if left untreated, and treatment consists of
plasmapheresis (PLEX) at a centre like ours, or steroids and fresh frozen
plasma while en route to a centre like ours.
-There
is a wide differential of thrombocytopenia, and we discussed immune
thrombocytopenic purpura as a potential diagnosis of exclusion among
other causes.
-Be
careful committing someone to a diagnosis of Evan’s syndrome (ITP and
autoimmune hemolytic anemia) without ruling out a microangiopathic cause such
as TTP/HUS.
-We
discussed blood film findings (fragments/Schistocytes) and laboratory findings
of hemolysis.
Further Reading:
George, J. N., & Nester, C. M. (2014). Syndromes of thrombotic microangiopathy. New England Journal of Medicine, 371(7), 654-666.
George, J. N., & Nester, C. M. (2014). Syndromes of thrombotic microangiopathy. New England Journal of Medicine, 371(7), 654-666.
No comments:
Post a Comment