A 52 year old African American female presented to the Emergency Department for the evaluation of left sided hand and
facial weakness. She was noted to have a normal non-contrast head CT; however she did have thrombocytopenia on her
complete blood count. Thus, initially the patient had microangiopathic hemolytic anemia (MAHA), thrombocytopenia and
a neurological change consistent with thrombotic thrombocytopenia purpura (TTP). Subsequently, the patient developed
the TTP pentad of MAHA, renal failure, thrombocytopenia, fever and neurological involvement. Thus the initial presentation
was incomplete for the pentad of TTP. This case illustrates that the emergency physician must be on high alert for
a patient who presents with neurological symptoms and thrombocytopenia despite not having the complete pentad for TTP.
TTP is a rare condition which carries a mortality rate of > 90%
if left untreated. Early treatment with plasma exchange can reduce
the mortality to 10-20% if instituted within 4-8 hours of
presentation [1]. Plasma exchange should be started if there is
evidence of microangiopathic hemolytic anemia (MAHA) and
thrombocytopenia with no other known cause.A 52 year old African American female presented to the ED for
evaluation of left sided hand and facial weakness which started
approximately 30 minutes prior to her arrival in the ED. A
stroke alert was immediately called. She was immediately taken
down for a non-contrast head CT scan. The CT scan showed
no acute pathology. On further evaluation, the patient report
ed that she had a history of multiple strokes in the past with a
recent stroke approximately 1.5 months prior to this ED visit.
Given this history of multiple strokes and a normal head CT
with no evidence of prior stroke this seemed abnormal. There
was no evidence of old infarcts that we could view. Neurology
recommended that she be admitted to the hospital and a
routine stroke workup should be undertaken.
The initial platelet
count was 74,000. Shortly thereafter, her platelet count
was noted to be 44,000. The laboratory was then called and
a smear was requested. The smear showed +2 schistocytes.
After obtaining that information LFTs, LDH and a reticulocyte
count were added to the requested labs. The BMP showed
normal creatinine of 0.99. She had no fever. A call was placed
to hematology to discuss the case. That opinion of the consul-tant was that TTP was the likely diagnosis, given evidence of
microangiopathic anemia as evidenced by schistocytes, along
with the elevated LDH and reticulocyte count, in association
with the neurologic complaints noted. Hematology recommended
that the patient be admitted to the ICU and that plasmaphoresis
should be initiated. The patient was admitted to
the ICU. She was also on Coumadin which she reported was
for “irregular heart beat.” Her INR was 2.1. Later the next afternoon
she was noted to have an acute change in mental status.
The neurologist then reevaluated her and recommended
that she be transferred to a Neuro ICU. There she had a MRI
which showed small infarcts in the left corona radiate and
left parietal cortex. No acute neurosurgical intervention was
needed. She then also developed a fever of 100.6 as well as
an acute increase in her creatinine. Her maximal creatinine
was 6.31.
Initially the patient only had a microangiopathic hemolytic
anemia and a neurological change consistent with TTP. Later
she developed the pentad of MAHA, renal failure, thrombocytopenia,
fever and neurological involvement. Plasmapheresis
was initiated early; approximately 14 hours after the patient
was admitted to the hospital. The patient required approximately
30 days in the hospital with multiple rounds of plasmaphoresis
and hemodialysis to clear her pathology. She was
then discharged to a rehabilitation facility, with minimal left
sided residual deficit but off hemodialysis. Shortly thereafter,
she presented to the hospital with a decreased platelet count
and required repeat plasmaphoresis. Lab testing was noted to
have a decreased ADAMTS13 level. The patient had no history
of recent diarrhea or of any new medications. It was felt that
the ADAMTS13 level was a mutation that was unmasked as
an adult.
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