Int J Hematol (2009) 90:343–346

DOI 10.1007/s12185-009-0394-2

CASE REPORT

Acute lower limb ischemia in a patient with aortic thrombus

and essential thrombocytosis
P. Caridad Morata Barrado Æ E. Blanco Cañibano Æ
B. Garcı́a Fresnillo Æ M. Guerra Requena

Received: 18 February 2009 / Revised: 6 July 2009 / Accepted: 14 July 2009 / Published online: 11 August 2009
Ó The Japanese Society of Hematology 2009

Abstract Aortic thrombus is rare in patients with essen- appropriate medical treatment after intervention must be
tial thrombocytosis (ET), so the optimal treatment remains supported.
undefined. A 45-year-old man with history of ET, under
chronic treatment with aspirin, presented to the emergency Keywords Essential thrombocytosis
Aortic thrombus

department complaining of acute onset in both the legs and Ischemia
abdominal pain. Physical examination revealed that both
dorsalis pedis pulses were not palpable with cold and pale
1 Introduction
feet. His abdomen was soft and nondistended. The platelet
count was 436 9 109/L. The thoraco-abdominal comput-
Essential thombocytosis (ET) is a myeloproliferative dis-
erized tomographic scanning revealed normal aortic
order (MPD) defined by an autonomous platelet production
diameter with supraceliac and infrarenal nonoccluding
with the absence of other MPD or other causes of reactive
thrombus and infarction areas in spleen and left kidney. At
thrombocytosis. Diagnosis of ET requires meeting all four
the emergency department he presented with recurrent
major criteria according to the 2008 World Health Orga-
symptoms, losing bilateral posterior tibial pulses. A deci-
nization (WHO) diagnostic criteria for ET [1], namely
sion was made to perform a thoracoretroperitoneal incision.
(1) platelet count C450 9 109/L (2) megakaryocyte pro-
A longitudinal sequential aortotomy was performed in the
liferation with large and mature morphology; none or
distal thoracic and infrarenal aorta, and the thrombus was
little granulocyte or erythroid proliferation (3) not meeting
easily removed. Following this, he underwent bilateral
WHO criteria for CML, PV, PMF, MDS or any other
crural thrombectomy and local intra-arterial thrombolytic
myeloid neoplasm, and (4) demonstration of JAK2V617F or
therapy. The postoperative course was uneventful. The left
other clonal markers or no evidence of reactive thrombosis.
toes were amputated because of necrosis. He was dis-
Many patients with ET are asymptomatic, but many
charged and put on antiaggregants, anticoagulants and
others have manifest symptoms or signs of bleeding or
hydroxyurea. Aortic thrombus in patients with ET is unu-
microvascular thrombosis [2–6].
sual, but potentially lethal. There is complete relief from
ET-associated thrombosis in large vessels such as aorta
symptoms in recurrent cases following surgery. An
is uncommon but has also been reported [7–9]. This kind of
thrombus is potentially devastating and its optimal treat-
P. C. Morata Barrado (&)
E. Blanco Cañibano

ment remains undefined. We describe a young patient
B. Garcı́a Fresnillo
M. Guerra Requena with ET who presented peripheral embolism from aortic
Service of Vascular and Endovascular Surgery, Hospital thrombus, developing acute limb ischemia.
Universitario de Guadalajara, Guadalajara, Spain
e-mail: [email protected]
2 Case report
P. C. Morata Barrado
Servicio de Angiologı́a, Cirugı́a Vascular y Endovascular,
Hospital Universitario de Guadalajara, Avd. Donantes de Sangre, A 45-year-old man presented to the emergency department
s/n, 19002 Guadalajara, Spain with sudden onset of numbness and pain in both the legs.

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344 P. C. Morata Barrado et al.

He also referred to mild abdominal pain. He had a past

history of tobacco use and essential thrombocytosis, under
chronic treatment with aspirin. No other risk factors for
atherosclerosis, such as diabetes mellitus or hyperlipid-
emia, were present.
The diagnosis of ET had been made several years
before. He was under revisions in the Service of Hema-
tology. Over the past 10 years platelet counts had ranged
from 500 9 109 to 800 9 109/L. Bone marrow biopsy
showed typical megakaryocytic hyperplasia in clusters
with mature cytoplasm and hyperlobulated nuclei. There
was no proliferation or immaturity of erythropoiesis and
granulopoiesis, only borderline increase of reticulin.
Cytogenetic study had revealed no evidence of Philadel-
phia chromosome. Six months before this episode he had Fig. 2 Arrow demonstrates infrarenal aortic thombus. Infarcted areas
been studied for the JAK2V617F mutation, applying allele- in left kidney are evident
specific polymerase chain reaction (PCR) analysis (DNA
was isolated from granulocytes of peripheral blood): he
was heterozygous for the JAK2V617F mutation.
Physical examination revealed both dorsalis pedis pulses
not palpable with cold and pale feet; femoral, popliteal and
posterior tibial pulses were normal. His abdomen was soft
and nondistended. Laboratory test demonstrated normal
serum chemistry; blood count revealed hemoglobin of
12 mg/dL, leucocyte count of 12 9 109/L and a platelet
count of 436 9 109/L. Chest X-rays and electrocardiogram
were normal. Because of the presence of abdominal pain, a
thoraco-abdominal computerized tomographic scanning
(TC) was performed. TC revealed normal aortic diameter
with supraceliac and infrarenal nonoccluding thrombus and
infarction areas in spleen and left kidney (Figs. 1, 2, 3).
While he was being evaluated in the emergency department
he got worse, with severe pain and progressive coldness in
lower limbs, losing bilateral posterior tibial pulses.
A decision was made to remove the clot surgically
because of recurrent ischemic symptoms. At surgery, the

Fig. 3 Arrows demonstrate both aortic thrombus

aorta was approached via a thoracoretroperitoneal incision.

At exploration, the aortic wall and surrounding tissues were
normal. A longitudinal sequential aortotomy was performed
in the distal thoracic and infrarenal aorta and the thrombus
was easily removed. The total clamp time was 10 min.
Following this, he underwent bilateral crural thrombectomy
Fig. 1 Arrow demonstrates distal thoracic aortic thrombus and local intra-arterial thrombolytic therapy.

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Essential thrombocytosis and aortic thrombus 345

The postoperative course was uneventful. He recovered attack [2, 3]. ET-aortic mural thrombus has occasionally
the right posterior tibial pulses. Treatment with intravenous been reported [7–9].
prostaglandins was established because of the necrosis of Our patient had known ET, but his platelet count at this
the 1st, 2nd and 3rd left toes. After that the toes were moment was not greater than 500 9 109/L. However, the
amputated. He was discharged and put on antiaggregants risk of a bleeding or thrombotic complication does not
(clopidogrel), coumadin and hydroxyurea. necessarily correlate with the platelet count. Indeed, in
The histology of the removed thrombus showed a rich recent reports authors found an inverse correlation between
platelet and fibrin network. extreme thrombocytosis and the risk of thrombosis in ET
[11, 12]. Moreover, these studies have suggested that
leukocytosis predicts both inferior survival and a higher
3 Discussion risk of thrombosis in ET. The prognostic relevance of
JAK2V617F presence in ET is less clear. In this case, the
Essential thrombocytosis is one of a group of clonal mye- patient had high leukocyte count (12 9 109/L); this could
loproliferative disorders that results in an increased platelet be related to the aortic thrombus formation.
production because of excessive megakaryocyte prolifera- Other factors such as atherosclerosis, dissection, trauma,
tion, independent of thrombopoietin. The World Health malignancy and coagulopathies have been associated with
Organization (WHO) revised and redefined the diagnostic aortic mural thrombi [13–16].
criteria in 2008 [1]. All four major criteria are required: This condition can be a source of systemic embolism,
(1) platelet count C450 9 109/L (2) megakaryocyte pro- with a high risk of recurrent events, which occurred in this
liferation with large and mature morphology; none or little case. Treatment is then mandatory, but there is no standard
granulocyte or erythroid proliferation (3) not meeting WHO management. The options include medical treatment with
criteria for CML, PV, PMF, MDS or any other myeloid heparin anticoagulation, aspirin and cytoreductive chemo-
neoplasm, and (4) demonstration of JAK2V617F or other therapy [8, 9], and surgical treatment, with aortic throm-
clonal markers or no evidence of reactive thrombosis. bectomy [7] or endovascular surgery [17, 18]. (Table 1).
The JAK2V617F mutation has recently been discovered Fang described the thrombus resolution without the
as the underlying cause of trilinear MPD and is detected development of any severe embolic events after aspirin and
with a high frequency (92–97%) in polycythemia vera and hydroxyurea treatment [9]. Choukroun et al. [14] suggest a
a lower frequency (49–57%) in ET and idiopathic myelo- gradual treatment: heparin anticoagulation as initial treat-
fibrosis (44–55%) [10]. In the revised criteria of 2008 the ment and surgery when this is not effective.
JAK2V617F has been listed as a major criterion, and the However, others authors recommend aggressive treat-
platelet count threshold for ET diagnosis has been lowered ment from the beginning, and some of them believe that
from 600 to 450 9 109/L. long-term anticoagulation carries unacceptable risk of
ET is associated with significant thrombotic and/or partial lysis and distal embolization. The results of aortic
hemorrhagic clinical events that may be life threatening. thromboendarterectomy have been reported to be satisfac-
The most frequent thrombotic complication affects the tory [7, 13, 15, 16].
microcirculation and small-medium sized vessels, usually Finally, therapeutic options will be influenced by the
as erythromelalgia, digital ischemia or transient ischemic location of the thrombus, its potential for embolism, the

Table 1 Some reports describing aortic thrombus in the recent literature and their management
Reference Year Cases (n) Age (years) Associated pathology Recurrence Treatment

Sohn et al. [20] 2008 1 52 E.T. No Delayed surgery

Lorelli et al. [8] 2002 1 83 E.T. No Medical
Fang et al. [9] 2001 1 45 E.T. No Medical
Johnson et al. [7] 1995 1 68 E.T. No Urgent surgery
Luebke et al. [18] 2008 1 47 – Yes Endovascular surgery
Böckler et al. [16] 2007 1 54 Hyper-coagulopathy No Elective surgery
Choi et al. [15] 2004 1 59 Atherosclerosis No Surgery
Choukroun et al. [14] 2002 9 28–68 Atherosclerosis 1 case (postsurgery) 4 Medical
5 Surgery (persistent thrombus)
Lozano et al. [13] 1998 1 46 Rheumatoid arthritis Yes Surgery
n number, y years

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346 P. C. Morata Barrado et al.

individual risk for the patient and perhaps the pathology of thrombosis with splenic infarction as first presentation of essen-
the thrombus [16, 19, 20]. tial thrombocytosis. J Clin Gastroenterol. 2004;38:540–1.
7. Johnson M, Gernsheimer T, Johansen K. Essential thrombocy-
In our case, the patient was a young man without risk tosis: underemphasized cause of large-vessel thrombosis. J Vasc
factors for open surgery. Even under previous treatment Surg. 1995;22:443–7.
with aspirin, he presented recurrent embolization, and so 8. Lorelli DR, Shepard AD. Aortic mural thrombus embolization: an
we considered that surgical treatment was mandatory. On unusual presentation of essential thrombocytosis. Ann Vasc Surg.
2002;16:375–9.
the other hand, endovascular repair could carry a high risk 9. Fang M, Agha S, Lockridge L, Lee R, Cleary JP, Mazur EM. Medical
of dislodging the thrombus to splanchnic circulation. management of a large aortic thrombus in a young woman with
Moreover, the thrombus was located in two different zones, essential thrombocythemia. Mayo Clin Proc. 2001;76:427–31.
distal thoracic and infrarenal aorta; hence it would have 10. Michiels JJ, De Raeve H, Berneman Z, Van Bockstaele D,
Hebeda K, Lam K, et al. The 2001 World Health Organization
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aorta covering, with increased risk of spinal ischemia. criteria for the diagnosis, classification and staging of the
Finally, long-term results with endovascular treatment in Ph1-chromosome negative chronic myeloproliferative disorders
these cases are unknown. (MPD). Sem Thromb Hemostas. 2006;32:307–40.
11. Carobbio A, Finazzi G, Guerini V, Spinelli O, Delaini F,
Marchioli R, et al. Leukocytosis is a risk factor for thrombosis in
Acknowledgments We are grateful to Arantxa Garcı́a, Molecular essential thrombocythemia: Interaction with treatment, standard
Biology Laboratory, Department of Hematology, Fundación Jiménez risk factors, and Jak2 mutation status. Blood. 2007;109:2310–3.
Dı́az, Madrid. 12. Carobbio A, Finazzi G, Antonioli E, Guglielmelli P, Vannucchi
AM, Delaini F, et al. Thrombocytosis and leukocytosis interac-
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