BMC Neurology

(2023) 23:47
Jameie et al. BMC Neurology
https://doi.org/10.1186/s12883-023-03073-1

Open Access

CASE REPORT

Two ischemic stroke events within 48 h:
a case report of an unusual presentation
of thrombotic thrombocytopenic purpura
Melika Jameie1,2†, Sanaz Heydari3†, Mojdeh Ghabaee1 and Hamed Amirifard1*   

Abstract
Background Thrombotic thrombocytopenic purpura (TTP) considers a rare cause of ischemic stroke (IS). We
reported a case of a newly diagnosed patient with acquired immune-mediated TTP (iTTP), in whom two IS events
developed during 48 h.
Case presentation A 59-year-old diabetic male was presented to the hospital 24 h after symptoms onset, including
left hemiparesis, dysarthria, and decreased consciousness. A brain CT scan was performed with the suspicion of acute
IS, indicating infarct lesions in the right middle cerebral artery (MCA) territory. The patient was not eligible for thrombolytic therapy due to admission delay. Over the next 24 h, the patient’s neurological condition deteriorated, and the
second brain CT scan showed new ischemic lesions in the left MCA territory. Initial laboratory evaluation indicated
thrombocytopenia without evidence of anemia. However, in the following days, thrombocytopenia progressed, and
microangiopathic hemolytic anemia (MAHA) developed. The ADAMTS-13 (a disintegrin and metalloproteinase with a
thrombospondin type 1 motif, member 13) activity and inhibitors assay confirmed the diagnosis of iTTP. The patient
underwent plasma exchange activity and inhibitors assay confirmed the diagnosis of iTTP. The patient underwent and
pulse IV methylprednisolone. Rituximab was also added due to the refractory course of the disease. After a prolonged
hospital course, he had considerable neurologic recovery and was discharged.
Conclusions Clinicians should consider two points. First, TTP should be considered in any patient presenting with IS
and having thrombocytopenia or anemia without other symptoms of TTP. Second, worsening the patient’s condition
during hospitalization may indicate a new stroke and should be investigated immediately.
Keywords Purpura, Thrombotic thrombocytopenic, Ischemic stroke, Thrombotic microangiopathies, Anemia,
Hemolytic, ADAMTS13 protein, Case report

†

Melika Jameie and Sanaz Heydari contributed equally to this work.

*Correspondence:
Hamed Amirifard
Dr.amirifard@gmail.com
1
Iranian Center of Neurological Research, Neuroscience Institute, Tehran
University of Medical Sciences, Tehran, Iran
2
Neuroscience Research Center, Iran University of Medical Sciences,
Tehran, Iran
3
Neurology Department, Imam Khomeini Hospital Complex, Tehran
University of Medical Sciences, Tehran, Iran

Background
Coagulation disorders account for less than 5% of
ischemic strokes (IS) [1]. Thrombotic thrombocytopenic
purpura (TTP) is a rare, though severe, inherited or
acquired disease caused by severe deficiency of the Von
Willebrand factor-cleaving serine protease, ADAMTS-13
(a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) [2]. The exact incidence
and prevalence figures are not available [3]. However,
various numbers have been reported, from 2.2 cases
per million population per year to 3.7 [4, 5] or even up

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Jameie et al. BMC Neurology

(2023) 23:47

to 13 cases per million population, according to a French
national registry [6]. Patients with TTP might present
with severe thrombotic events such as IS, either initially
or during the treatment [7–10]. Stroke develops in 8.16%
of patients admitted with TTP, according to the findings
from the Nationwide Inpatient Sample (NIS), Healthcare
Cost and Utilization Project (HCUP) [11]. Classic clinical
manifestations include pentad of fever, microangiopathic
hemolytic anemia (MAHA), thrombocytopenic purpura,
renal dysfunction, and neurologic symptoms [3, 12]. Laboratory findings include thrombocytopenia and evidence
of MAHA, including schistocytes, reticulocytosis, indirect hyperbilirubinemia, undetectable haptoglobin, and
high lactate dehydrogenase (LDH) [12]. The diagnosis is
suspected based on the clinical and laboratory findings
and confirmed by very low ADAMTS-13 levels [12]. Of
note, in the absence of other obvious causes, evidence of
MAHA or thrombocytopenia without the presence of
the classic pentad is strongly suggestive of TTP, hence the
importance of immediate total plasma exchange (TPE)
[13].
We reported a case of a newly diagnosed patient with
acquired immune-mediated TTP (iTTP), in whom two IS
events developed during a period of 48 h. This circumstance is unique in several ways. To begin with, iTTP
is a rare disorder. Second, stroke is considered to be an
unusual inaugural presentation of TTP. Third, there are
currently very few reports of new ischemic lesions during hospitalization in individuals with iTTP following
the first ischemic stroke event [14, 15]. Notably, some
of these patients had the congenital type of the disease,
making our patient even more intriguing.

Case presentation
A 59-year-old diabetic male was referred to our emergency room following left-sided weakness, hemifacial
paresis, difficulty speaking, and drowsiness from one
night before (National Institute of Health Stroke Score
(NIHSS) at admission = 10). There was no history of
recent head trauma. Family history of premature stroke
or thrombotic events was also negative. The patient was
a non-smoker, albeit an opiate user. The initial physical
exam showed an elderly, confused male who could not
answer the questions or follow commands cooperatively.
Vitals were as follow: T = 37.4 °C, BP = 150/90 mmHG,
HR = 73 bpm (beats per minute), RR = 16/minute, and ­O2
saturation in the room air = 97%. An electrocardiogram
(ECG) showed normal sinus rhythm. On initial physical
examination, no signs of pallor, jaundice, petechiae, or
organomegaly were evident. Except for left facial paresis,
cranial nerves examination was normal. Motor examination revealed left hemiparesis, with a force of 3/5 in the
left upper extremity and 4/5 in the left lower extremity.

Page 2 of 7

The grimace to pinprick was reduced on the left side. The
plantar reflex showed upward movement in the left side
(Babinski sign).
Investigations and treatment

The patient underwent a brain CT scan with suspicion of
IS. Of note, since the patient’s symptoms had been initiated more than 24 h ago, he was not eligible to receive
intravenous thrombolysis (IVT) or thrombectomy [16].
The first brain CT scan showed an acute stroke in the
right middle cerebral artery (MCA) territory, including the right parietotemporal lobe infarct (Fig. 1). The
patient’s level of consciousness deteriorated over the
next 24 h, necessitating further evaluation; the second
brain CT scan revealed evidence of new ischemic lesions
in the left MCA territory, including the left frontal lobe
(Fig. 2). Progressive thrombocytopenia was found in
the patient (initial platelet count: 77*103/ µL reduced
to 21*103/ µL). Although the patient was not anemic at
first (initial Hgb: 15 g/dL), evidence of hemolytic anemia
gradually emerged, including a reticulocyte percentage
of 2.6%, reduced Hgb (from 15 g/dL to 11.8 g/dL on day
6), indirect hyperbilirubinemia (total bilirubin 1.9 mg/
dL increased up to 4.4 mg/dL, and indirect bilirubin
1.1 mg/dL increased up to 3.1 mg/dL), and elevated LDH
(1148 U/L). Additionally, acute phase reactants, including ESR, CRP, ferritin, fibrinogen, and D-dimer, were
also elevated. Troponin, coagulation tests, liver function

Fig. 1 Brain CT scan on admission showed the right parietotemporal
lobe infarct

Jameie et al. BMC Neurology

(2023) 23:47

Page 3 of 7

Table 1 The results of patients’ laboratory evaluation at
presentation, day 7 hospitalization, and discharge

Fig. 2 Brain CT scan 24 h after admission showed a new infarct in the
left frontal lobe

tests, lipid profile, and electrolytes were normal. Peripheral blood smear (PBS) showed occasional schistocytes.
Direct and indirect Coombs tests were negative. TTP
diagnosis was highly suspected based on the patient’s
clinical manifestations and progressive thrombocytopenia. Therefore further evaluation was performed, indicating an ADAMTS-13 activity level of 0.3 IU/mL (our
laboratory cut-off for a positive value was < 0.4 IU/mL)
and ADAMTS-13 inhibition screen of 18 U/mL (our
laboratory cut-off for a positive value was > 15 U/mL),
confirming the diagnosis of iTTP. Other Serologic evaluations (antinuclear antibody (ANA), anti-double strands
DNA antibody (anti-dsDNA Ab), perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA), cytoplasmic
anti-neutrophil cytoplasmic autoantibody (C-ANCA),
rheumatoid factor (RF), complement 3 ­(C3), complement
4 ­(C4), anticardiolipin IgM and IgG antibodies, antiphospholipid IgM and IgG antibodies, and anti–Sjögren’ssyndrome-related antigen A and B autoantibodies (SSA
and SSB) were within normal limits. HIV, hepatitis B
virus, and hepatitis C virus serology were also nonreactive (Table 1).
Although the cardioembolic source of stroke was suspected due to the bihemispheric infarcts, ECG Holter
monitoring, trans-thoracic, and trans-esophageal
echocardiography revealed no signs of arrhythmia,
clot, or vegetation, and the ejection fraction (EF) was
50–55%. Brain MRI on day 8 demonstrated evidence

Laboratory test
(unit)

Initial value Day 7
Discharge value
hospitalization

WBC (103/ µL)

10.8

Hgb (g/dL)

15

MCV (fL)

86.5

MCH (pg)

30.9

Reticulocyte (%)

3.2

4

Corrected reticulocyte (%)

1.7

2.6

PLT (103/ µL)

77

25

PT (sec)

13.1

13.1

PTT (sec)

28

26

INR

1.0

1.0

Glucose (mg/dL)

268

8
12.3

12

189

HbA1c (%)

> 14

BUN (mg/dL)

18

12.3

Cr (mg/dL)

0.9

Total Bilirubin (mg/
dL)

1.9

4.4

1.4

Direct Bilirubin (mg/
dL)

0.8

1.3

0.4

LDH (U/L)

1148

ESR (mm/h)

49

CRP (mg/l)

16

D-Dimer (µg/l)

1932

Fibrinogen (mg/dL)

609

Ferritin (ng/mL)

910

23
0.7

456

WBC White blood cells, Hgb hemoglobin, MCV Mean corpuscular volume,
MCH Mean corpuscular hemoglobin, PLT Platelet, PT Prothrombin time,
PTT Partial thromboplastin time, INR International normalized ratio, HbA1c
Glycosylated hemoglobin, BUN Blood urea nitrogen, Cr Creatinine, LDH Lactate
dehydrogenase, ESR Erythrocyte sedimentation rate, CRP, C reactive protein

in favor of subacute infarctions with restriction and
mild hemorrhagic transformation in the right parietotemporal, left frontal lobe, and left centrum semiovale
(Fig. 3). There was no evidence of venous thrombosis
or vascular lesions on magnetic resonance angiography (MRA) and magnetic resonance venography
(MRV). Since malignant bone marrow infiltration
is one of the main differential diagnoses in a patient
with MAHA and thrombocytopenia [14], hematology
was consulted. They asked for a bone marrow aspiration and biopsy (day 8), which showed normocellular marrow with increased erythroid series. Informed
consent was obtained, and the patient underwent therapeutic plasma exchange (TPE) on day 8 with iTTP
diagnosis. Consequently, the patient’s level of consciousness improved so that he was completely alert

Jameie et al. BMC Neurology

(2023) 23:47

Page 4 of 7

Fig. 3 Brain MRI on day 8 showed subacute infarctions with restriction and mild hemorrhagic transformation in the right parietotemporal, left
frontal lobe, and left centrum semiovale; A: Diffusion-weighted imaging (DWI); B: Fluid attenuated inversion recovery (FLAIR); C: T1-weighted

and responsive 9 days after TPE (day 17 hospitalization). The platelet count also gradually increased from
21*103/ µL up to 91*103/ µL a week after TPE initiation,
although it again started to decrease. Hence, pulse IV
methylprednisolone therapy was added on day 17 of
hospitalization for 3 days, followed by oral prednisolone (1 mg/kg). Rituximab (750 mg/m2) was also initiated on day 30 of hospitalization. The patient’s clinical
condition, as well as the MAHA, gradually improved

(NIHSS at discharge = 3), and the PLT count reached
189*103/ µL at discharge (48 days of hospitalization,
including 17 days at the stroke care unit (SCU), 5 days
at the neurology ward, and 26 days at the hematology
ward). During the follow-up, the neurological symptoms progressively improved, and he did not experience
further relapses or report adverse effects. (modified
Rankin Scale = 1, 75 days after discharge). The patient’s
timeline is shown in Table 2.

Table 2 An overview of the patient’s timeline during hospitalization
Day 0

Initiation of stroke-related symptoms

Day 1

Admission to the hospital
The first brain CT showed the right parietotemporal lobe infarct
Detection of thrombocytopenia

Day 2

Worsening of patient’s neurological condition
The second brain CT showed a new infarct in the left frontal lobe

Day 6

Decrease in hemoglobin ≤ 13 g/dL
Evidence of MAHA

Day 7

ADAMTS-13 activity and inhibitors assay

Day 8

Brain MRI showed subacute infarctions with restriction and mild hemorrhagic transformation in the right parietotemporal, left frontal lobe, and
left centrum semiovale
Bone marrow aspiration and biopsy
Initiation of therapeutic plasma exchange

Day 15

Platelet count reached 91*103/ µL

Day 17

Considerable improvement neurologically
Platelet count started to decrease again
Initiation of pulse IV methylprednisolone

Day 20

Initiation of oral prednisolone

Day 30

Rituximab was added

Day 48

Discharged

MAHA Microangiopathic hemolytic anemia, ADAMTS-13 A disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13, IV Intravenous

Jameie et al. BMC Neurology

(2023) 23:47

Discussion
In this study, we reported a 59-year-old male presented
with hemiparesis, dysarthria, and decreased consciousness who developed two ischemic stroke events during
48 h, which is extremely rare. The results of neuroimaging revealed evidence of infarction in the right (at admission) and left (24 h after admission) MCA territories,
respectively. Initial laboratory findings indicated progressive thrombocytopenia. However, MAHA was not found
at presentation. Further evaluations, including measurement of ADAMTS-13 activity level and ADAMTS-13
inhibitor, confirmed the diagnosis of iTTP. The patient
underwent therapeutic plasma exchange and pulse IV
methylprednisolone therapy. Rituximab was also given
since it could reduce relapses, and is increasingly recommended for use in refractory cases [17, 18]. Notably, studies showed that although iTTP considers an autoimmune
disease, ADAMTS-13 inhibitors are not easily detectable in many patients [19], which was not the case in our
patient. Similar to the prolonged course of treatment in
our patient, studies showed that high titers of inhibitors
of ADAMTS-13 are associated with delayed response to
therapy, high risk of complications, and higher rates of
refractory disease and relapses [19, 20]. Of note, while
there are reports of patients with TTP who presented
with IS as an atypical presentation [15, 21], there are very
few reports of new ischemic lesions during hospitalization following the first ischemic stroke event [14, 15].
One was a 70-year-old female who presented with confusion, fever, and gastrointestinal symptoms who deteriorated neurologically during hospitalization, and the brain
MRI revealed multiple acute and subacute infarcts in
both cerebral hemispheres, midbrain, and right cerebellum. The patient had a history of hypertension, diabetes,
rheumatoid arthritis, two miscarriages complicated by
disseminated intravascular coagulation, two transient
ischemic attacks, and a left occipital infarct and she was
diagnosed with congenital TTP [14]. Another one was a
43-year-old female who was admitted with left hemiparesis, homonymous hemianopia, and dysarthria. The first
brain CT showed an acute stroke in the right MCA territory, and follow-up MRIs during the first week of her
hospitalization demonstrated new ischemic lesions in
various territories [15].
TTP is a relapsing and life-threatening condition
characterized by clot formation in small blood vessels
[22]. Clinical manifestations vary from mild nonspecific symptoms to severe organ damages, including kidney and brain, and death [3]. Neurologic involvements,
such as headaches, confusion, focal neurologic deficit,
etc., are common as the initial presentation of TTP usually as a result of diffuse microthrombi formation in the
microvasculature of the central nervous system (CNS),

Page 5 of 7

resulting in transient or permanent ischemic brain damage [3, 7–9, 23]. However, TTP considers a rare cause of
ischemic stroke [15], although there are similar cases in
the literature of patients being diagnosed with TTP after
a cerebrovascular accident [9, 21, 24–26]. A recent study
on patients with TTP who presented with neurological
symptoms suggested that old age, hypertension, smoking,
and high plasma concentrations of anti-ADAMTS-13
IgG may be the risk factors for developing cerebral infarction in these patients [27]. Furthermore, they reported no
association between iTTP stroke and diabetes, however,
our patient was diabetic [27]. Additionally, it is suggested
that decreased level of ADAMTS-13 is associated with
stroke recurrence after remission [28], hence the importance of patients’ follow-up.
A literature review conducted in 2018 reported 17
cases of IS due to iTTP [15]. Interestingly, none of the
patients showed the classical TTP pentad, and wide
non-specific heterogeneity in clinical manifestations
was observed [15]. The combination of thrombocytopenia and hemolysis was found in less than half of the
patients (41%). In 41% of cases, the stroke was multifocal
and included major artery strokes, with proximal occlusion in 3 cases. Considerably, refractory and relapsing
forms were observed in nearly half of the patients (47%).
They reported small and large artery strokes in these
patients, with the majority being multifocal and without
a specific pattern [15]. These findings confirm the claim
that although TTP is classified as a thrombotic microangiopathic condition, which results in small vessels
obstruction, it should also be considered in patients with
proximal intracranial artery occlusion [9, 15, 24, 29].

Conclusion
In alignment with previous studies, this study showed
that although TTP is considered an uncommon cause
of ischemic stroke, it should be considered in every IS
patient with isolated anemia or thrombocytopenia even
in the absence of other classic symptoms or laboratory
findings. This study also suggests that clinicians should
consider the possibility that worsening of the patient’s
clinical condition could be attributed to emerging of new
infarcts, emphasizing the importance of early diagnosis
and treatment of iTTP to prevent multiple infarcts and a
poor prognosis.
Abbreviations
ADAMTS-13
A disintegrin and metalloproteinase with a thrombospondin
type 1 motif, member 13
ANA
Antinuclear antibody
Anti-dsDNA	 Anti-double strands DNA
Bpm
Beats per minute
C-ANCA
Cytoplasmic antineutrophil cytoplasmic autoantibody
CNS
Central nervous system

Jameie et al. BMC Neurology

(2023) 23:47

C3	Complement 3
C4	Complement 4
ECG	Electrocardiogram
EF	Ejection fraction
HCUP	Healthcare cost and utilization project
Hgb	Hemoglobin
IS	Ischemic stroke
iTTP	Immune-mediated thrombotic thrombocytopenic purpura
IVT	Intravenous thrombolysis
LDH	Lactate dehydrogenase
MAHA	Microangiopathic hemolytic anemia
MCA	Middle cerebral artery
MRA	Magnetic resonance angiography
MRV	Magnetic resonance venography
NIHSS	National institute of health stroke score
NIS	Nationwide inpatient sample
P-ANCA	Perinuclear anti-neutrophil cytoplasmic antibodies
PBS	Peripheral blood smear
RF	Rheumatoid factor
SSA	Sjögren’s-syndrome-related antigen A
SSB	Sjögren’s-syndrome-related antigen B
TPE	Therapeutic plasma exchange
TTP	Thrombotic thrombocytopenic purpura

Page 6 of 7

3.
4.
5.
6.

7.
8.
9.
10.

Acknowledgements
We would like to thank the patient and his brother for their participation and
consent for publication.

11.

Authors’ contributions
HA and MG provided the patient with appropriate treatment according to the
guidelines. SH collected the data and had a major role in the patient’s treatment. MJ assisted with the patient’s follow-up and was a major contributor in
writing the manuscript. All authors read and approved the final manuscript.

12.

Funding
The authors received no financial support for the research, authorship, and
publication of this manuscript.

13.
14.

Availability of data and materials
Data sharing is not applicable to this article as no datasets were generated or
analyzed during the current study.

15.

Declarations

16.

Ethics approval and consent to participate
This study was conducted at an academic hospital complex affiliated with
Tehran University of Medical Sciences (TUMS), Tehran, Iran, and approved
by the ethics committee of the university. Written informed consent was
obtained from the patient. This study was conducted according to the Declaration of Helsinki [30].
Consent for publication
The patient provided written informed consent for the submission and
possible publication of this manuscript. During the patient’s hospitalization,
consent for treatment was also obtained in the usual manner.
Competing interest
There are no conflicts of interest.

17.
18.

19.

20.

Received: 30 November 2021 Accepted: 16 January 2023
21.
22.
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