a 32-year-old woman with chest Pain, hematemesis, and Thrombocytopenia

Journal of the Louisiana State Medical Society
clinical case oF the month
a 32-year-old woman with chest Pain,
hematemesis, and Thrombocytopenia
Brian C. Boulmay, MD; Robert Fitzgerald, BS; Brian Lewis, MD;
and Fred A. Lopez, MD (Section Editor)
case PResenTaTion
nL (<231). Review of the peripheral blood smear revealed
schistocytes (Figure 1). The findings of an elevated LDH,
A 32-year-old woman presented with a one day history
schistocytes, and cardiac ischemia were felt to be most
of substernal, constant, non-radiating chest pain with
consistent with a thrombotic microangiopathic hemolytic
associated abdominal discomfort, nausea, and vomiting
process, and a presumptive diagnosis of thrombotic
of small amounts of blood of two days duration. She also
thrombocytopenic purpura (TTP) was made. The patient
reported headaches and dizziness during the day prior to
was emergently started on daily 1.5x plasma volume plasma
presentation. Her past medical history was significant for
exchange. Intravenous methylprednisolone was started at
hypertension treated with hydrochlorothiazide. The patient
1mg/kg for three days.
worked in a restaurant as a cook, smoked one-half pack
Twelve hours after the first plasma exchange the LDH
of cigarettes daily, but did not use alcohol or illicit drugs.
dropped from 1,600 U/L to 479 U/L; cardiac troponin
There were no recent sick contacts and the patient had no
decreased to 0.34 ng/mL; platelets rose to 33,000/microliter;
known allergies.
and total bilirubin dropped from 2.5mg/dL to 0.9mg/dL.
Physical examination revealed a heart rate of 100
After four additional plasma exchanges, the LDH had
beats per minute and a blood pressure of 115/72 mmHg.
decreased to 254 U/L; hematocrit had risen to 31%; and
The patient was somnolent, but able to cooperate with
platelets had normalized at 179,000/microliter (130-400).
the physical examination. The conjunctiva were pale and
Schistocytes remained on the peripheral smear (Figure 2).
without icterus. She had several ecchymoses measuring 1-2
The serum creatinine and total bilirubin remained normal
centimeters scattered on her legs and forehead. There were
at 1.2mg/dL and 0.8mg/dL, respectively. An ADAMTS13
no focal neurologic deficits. Laboratory evaluation showed
enzyme activity from the time of admission returned one
a hemoglobin of 7g/dL (12-16), hematocrit of 22% (35-46),
week later at 5% (>25%) and an ADAMTS13 inhibitor was
a platelet count of 15,000/
positive at 0.5 inhibitor units
microliter (130,000-400,000),
(<0.4).
a serum creatinine of 1.3mg/
An echocardiogram was
dL (0.6-1.2), and a blood urea
performed and revealed
nitrogen of 19mg/dL (7-25).
preserved left ventricular
The prothrombin time was
function with mild tricuspid
12.5s (9.5-12.5), and the partial
r e g u r g i t a t i o n . P l a s m a
thromboplastin time was 25.8s
exchange volumes were
(24-36). Serum haptoglobin
decreased to 1.0x plasma
was 11mg/dL (30-195); serum
volume and tapered to every
lactate dehydrogenase (LDH)
other day with no subjective
was 1,600 U/L (<201) with a
or laboratory evidence for
total bilirubin of 2.5mg/dL
recurrent TTP over one week.
(<1.3); indirect bilirubin was
At discharge, aspirin, 325mg
not available; and the direct
orally daily, was started.
Coombs was negative. The
Subsequent outpatient clinic
cardiac troponin was 1.85ng/
visits showed continued
mL (<0.09), and quantitative
remission. At one month
D-dimer level was 5,257ng/ Figure 1. Peripheral blood smear at diagnosis.
follow-up, the LDH was 189
130 J La state Med soc VOL 161 May/June 2009

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Figure 2. Peripheral blood smear after four daily plasma
Figure 3. Peripheral smear at three-month follow-up.
exchanges.
U/L (<201); hematocrit, 36.5% (34%-46%); platelet count,
Von Willebrand factor (VWF), a glycoprotein produced
427,000/microliter (130-400); creatinine, 1.0mg/dL (0.6-1.2);
by platelets and endothelial cells, is integral to normal
and a total bilirubin, 0.5 mg/dL(<1.3). Prednisone has been
hemostasis, and it is essential for the formation of the
tapered to off, and her peripheral smear now demonstrates
initial platelet “plug” at sites of endothelial injury. VWF
no schistocytes (Figure 3).
is produced as a large molecule referred to as the ultra-
large VWF multimer (ULVWF) which has an enhanced
Discussion
ability to activate platelets. The ULVWF is cleaved by the
metalloproteinase ADAMTS13 to the less active VWF. If
First described by Moschcowitz in 1924,1 thrombotic
ADAMTS13 activity is diminished, ULVWF is allowed to
thrombocytopenic purpura (TTP) is a rare disorder often
persist in the circulation. The resulting unimpeded platelet
confused with other consumptive hematologic disorders
aggregation causes obstruction of capillary beds, widespread
such as disseminated intravascular coagulopathy (DIC),
tissue hypoxia and schistocyte formation.
idiopathic thrombocytopenic purpura (ITP), HELLP
Idiopathic TTP is caused by inhibitory IgG antibodies
(hemolysis, elevated liver enzymes and low platelets),
directed against ADAMTS13, resulting in activities of
and the hemolytic uremic syndrome (HUS) (Table 1).
approximately 5% of normal. As such, ADAMTS13 activity
Historically, mortality with TTP exceeded 90%.2 With the
has been proposed as an additional component of the
use of plasma infusion or plasma exchange, the prognosis
diagnostic pentad. However, this reduction in ADAMTS13
for TTP has improved significantly with long-term survivals
activity is not specific for TTP; it can be diminished in other
now approaching 90%. Because of the implementation of
hematologic disorders often confused with TTP such as
effective treatments, empiric TTP therapy is often indicated
systemic lupus erythematosus, immune thrombocytopenic
even in unclear clinical situations with hemolysis, anemia,
purpura, and DIC. Further, the assay is often not rapidly
and thrombocytopenia.
available, and treatment should not be delayed if TTP is
The diagnostic pentad for TTP of thrombocytopenia,
strongly suspected.
hemolytic anemia, renal failure, fever, and neurologic
abnormalities was described 40 years ago.3 Since that time
TReaTMenT
TTP has remained a clinical diagnosis though additional
signs, symptoms, and laboratory findings have been
Plasma contains small amounts of ADAMTS13 sufficient
suggested for inclusion as diagnostic criteria for TTP. For
for normal hemostasis, and simple infusion of plasma can
example, schistocytes on the peripheral smear are felt to
be used as initial treatment of TTP. Therapeutic plasma
be an essential condition for the diagnosis of TTP. This
exchange (TPE) is more advantageous in that it allows both
finding is not specific as blood from patients with malignant
replacement of ADAMTS13 and removal of the antibody
hypertension, pre-eclampsia, or mechanical heart valves, as
to ADAMTS13. TPE is performed by removing a patient’s
well as from normal subjects, may have small numbers of
plasma and replacing it with donor plasma, typically in the
schistocytes.4,5 The entire classic pentad is helpful, but not
form of fresh frozen plasma (FFP). TPE is differentiated from
required, to make the diagnosis of TTP; clinical judgment
apheresis (commonly used for organ transplant rejection
remains the key.
or hyperviscosity syndrome) in which plasma is removed
J La state Med soc VOL 161 May/June 2009 131

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Journal of the Louisiana State Medical Society
Table. Typical Findings in TTP, HUS, DIC, HELLP, ITP.
TTP
HUS
DIC
HELLP
ITP
Bleeding
None
None
Common
Rare
Petechial
Platelet count
10,000-50,000
50,000-100,000
>30,000
10,000-50,000
0-50,000
Elevated Bilirubin
++
++
—
—
—
Elevated PT/PTT
—
—
++
—
—
Elevated AST/ALT
—
—
—
+++
—
Renal Abnormalities
+/-
+++
—
—
—
Schistocytes
+++
+
+/-
+/-
—
Elevated LDH
+++
++
+
+
—
TTP=thrombotic thrombocytopenic purpura; HUS=hemolytic uremic syndrome; DIC=disseminated intravascular coagulopathy;
HELLP=hemolysis, elevated liver enzymes and low platelets; ITP=idiopathic thrombocytopenic purpura.
and replaced with albumin and saline; plasma is the critical
Cryoprecipitate-poor plasma (CPP) is a plasma product
component of TTP therapy. TPE results in long-term
that is deficient in the large VWF multimers that play
remission rates of 60%-80%.9
the key role in the pathophysiology of TTP. It has been
The superiority of TPE to simple plasma infusion was
proposed that CPP may be superior to FFP due to lack of
established by the Canadian Apheresis Study Group in
these multimers. One small randomized, prospective trial
which patients diagnosed with TTP received either daily
performed by the North American TTP Group directly
plasma infusion or TPE. Patients were treated daily until the
compared FFP to CPP as initial therapy for TTP. There
platelet count increased to 150,000/microliter and therapy
were no differences reported in overall survival or response
intensity was reduced to five treatments given over two
rates.13 Despite lack of efficacy in upfront therapy, CPP has
weeks. The TPE arm had higher initial response rates with
been demonstrated to be effective in the salvage setting after
a statistically superior overall survival of 78% versus 63%.
failure with FFP.14,15
Seventy-eight percent of patients treated on the TPE arm
The addition of corticosteroids to TPE may result in a
remained in remission at six-month follow-up.10
more durable remission of TTP ostensibly by suppressing
Remission of TTP is loosely defined and can be a
production of antibodies directed against ADAMTS13.16
source of debate for when to stop intensive therapeutic
As an example, one series has reported treating patients
interventions. Remission is usually defined as resolution
diagnosed with TTP with 200mg of prednisone per day only.
of thrombocytopenia, a normalization of serum LDH,
Fifty-one percent of patients achieved a durable remission
a stable hemoglobin, resolved neurologic deficits, and
despite withholding TPE and plasma infusion.17 (We
normalized renal function. (Schistocytes may remain on the
would caution that employing plasma infusion/exchange
peripheral smear even when other laboratory parameters
is currently the accepted standard of care.) The Canadian
have normalized.) Once these laboratory endpoints have
Apheresis Study Group trial, described previously, did not
been reached, TPE may be stopped or tapered to every other
use steroids and had outcomes similar to other reported
day initially. For the most part, the decision to withdraw
exchange trials with steroid containing treatment arms.
therapy requires a thorough and constant evaluation of the
Expert opinion recommends that steroids can be added
entire clinical and laboratory data. If a patient experiences a
when response to TPE alone is poor.18 Short pulses of 1
relapse after stopping TPE, therapy needs to be resumed.
gram methylprednisolone daily for three days can be used
It is important to note that TPE is not a benign therapy:
at the time of diagnosis concurrent with TPE in order to
patients require an indwelling central venous catheter; the
limit long-term steroid exposure. Steroid therapy should
exchange process is associated with hypocalcemia and
also be considered in emergent situations where TPE or
volume shifts; and patients are exposed to blood products
plasma infusions are not immediately available.
derived from multiple donors. A recent review of patients
Platelet adhesion onto VWF multimers serves as the
treated with TPE showed that one-quarter developed
cause of the development of microvascular thrombosis.
hypotension, bacteremia, or deep vein thrombosis with a
Some researchers have proposed blocking additional
2% incidence of treatment-related deaths.11 Expert opinion
platelet aggregation with the use of aspirin or aspirin
supports continuing daily TPE for a few days, typically 2-3,
containing compounds. While aspirin acts as a potent
after remission is achieved as “consolidation.” The decision
inhibitor of platelet aggregation not directly associated with
to continue TPE needs to be balanced against the potential
TTP pathophysiology (through inhibition of thromboxane-
complications.12
dependent pathways), early clinical research suggested that
132 J La state Med soc VOL 161 May/June 2009

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antiplatelet agents helped achieve disease remission.19 Many
high mortality rate. For example, quinine-associated TTP
later study protocols evaluating TPE for TTP have included
treated with plasma exchange has a 21% mortality rate,21
platelet inhibitors such as aspirin and/or dipyridamole.9,16
and ticlopidine-associated TTP has an overall mortality
Recently, a small prospective trial that directly compared
of 18%.22 TTP induced by mitomycin C (now used almost
TPE to TPE/aspirin, and there was no difference in remission
exclusively in the therapy of anal canal carcinoma) has been
rates or numbers of death.20 In addition, fatal hemorrhage is
treated with TPE, plasma infusion, or glucocorticosteroids,
of concern when using platelet inhibitors with concomitant
in various combinations; survival rates range from 0%-25%.27
thrombocytopenia. Prospective data show approximately
As it relates to mitomycin C, the high mortality rate stems, in
8% of patients will experience bleeding complications while
part, from the poor prognosis of the underlying malignancy
on aspirin and TPE, but none of these episodes result in
for which the drug was used.
death or interfere with TTP therapy.19 As most clinical trials
Stem cell transplant is employed for the cure of
evaluating TPE have included aspirin, many practitioners
many hematologic malignancies and is often associated
consider its use a standard component of therapy. A
with microangiopathic anemia, thrombocytopenia, fever,
consensus conference has suggested starting aspirin when
neurologic pathology, and renal failure, ie, signs and
the platelet count is greater than 50,000/microliter.11
symptoms routinely seen in the transplant setting and not
necessarily indicative of TTP.24 Patients with transplant-
oTheR eTioLoGies oF TTP
related TTP usually have normal level of ADAMTS13.28
Instead, the underlying mechanism of the microangiopathic
Microangiopathic hemolytic anemias of various
process is felt to be directly related to endothelial damage
etiologies can present with clinical features that are similar
from myeloablative chemotherapy. The incidence of TTP
to classic TTP. These scenarios include systemic lupus
associated with transplant is reported to range between 2%-
erythematosus (SLE), pregnancy, and a TTP syndrome
76%. The largest series describing this disorder reports an
induced by various drugs or stem cell transplantation.21-24
18% response rate to TPE, with a 42-month overall survival
Even though these TTP-like syndromes resemble idiopathic
of 6%.24 Patients who develop this TTP syndrome in the
TTP in all laboratory and clinical parameters, standard
transplant setting do poorly, and TPE is likely of limited
therapies are not always of clear benefit.
utility.
The association of TTP with SLE has been frequently
A rare congenital form of TTP, referred to as chronic
reported in the medical literature.25 Simultaneous
relapsing TTP, typically presents in infancy or early
development of these two diseases likely stems from
overlapping autoimmune processes, and distinguishing
TTP from SLE can often prove difficult. Active lupus may
State-of-the-Art NEUROSURGERY
present with the classic pentad seen in active TTP and often
is treated as such. In one large case series of patients with
TTP and SLE, treatment consisted of either TPE, plasma
infusion, steroids, or no TTP directed therapy. The overall
mortality was relatively high (34%) despite the use of
aggressive interventions. Specifically, the mortality rate
even with TPE was 32%.25
TTP occurring during pregnancy can be confused with
the HELLP syndrome or pre-eclampsia; often the distinction
• Most patients seen within two weeks, many not
is impossible to make. Potential complications from TTP
needing surgery
include death of the mother or loss of the fetus by placental
• Brain Surgery for Tumors, Epilepsy, Tremor, Parkinson’s
infarction. Most practitioners recommend delivery of a
Disease, Aneurysms, Bleeds, and more
viable fetus, as delivery will result in resolution of pre-
• Spine Surgery – minimal surgery = less pain, fast recovery
eclampsia and HELLP. Elective termination of non-viable
fetus or early delivery is not necessary when TTP is strongly
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TTP associated with commonly used drugs is
described extensively in the literature; quinine, ticlopidine
Dr. ravish PatwarDhan
and mitomycin-C are most frequently implicated. The
Located within Willis-Knighton, Pierremont
pathophysiology of drug-induced TTP differs from
8001 Youree Dr., Suite 970
idiopathic TTP in that most cases associated with drugs
phone 318.797.5543
do not have an ADAMTS13 inhibitor.26 Withdrawal of the
fax 318.797.7608
offending drug should be the first therapeutic intervention
but standard therapies such as TPE should also be
www.neurosurgery.ws
implemented. Drug-induced TTP is associated with a
CNS Ad_010909_gray.indd 1
1/11/09 9:01:48 PM
J La state Med soc VOL 161 May/June 2009 133

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Journal of the Louisiana State Medical Society
adulthood with episodes occurring at 3-4 week intervals.
are present. If other secondary causes are ruled out, therapy
It is inherited in an autosomal recessive manner. Deficient
should instituted as rapidly as possible. TPE remains the
ADAMTS13 activity results from missense mutations in the
standard of care for TTP. In emergent situations where TPE
gene encoding ADAMTS13.29,30 The course of the disease
is not immediately available, infusion of FFP at 15mL/kg
is variable with patients often requiring simple plasma
per day and initiation of glucocorticoids is acceptable.
infusions monthly; plasma exchange does not have a role in
treating this disorder.31,32 Patients who present for the first
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