American Journal of Hematology 76:143–147 (2004)

Thromboembolic Complications After Splenectomy

for Hematologic Diseases
Martin Mohren,1* Ilka Markmann,1 Ulrike Dworschak,1 Astrid Franke,1 Christian Maas,1
Sabine Mewes,1 Günter Weiss,2 and Kathleen Jentsch-Ullrich1

Thromboembolic complications following splenectomy for hematologic diseases occur in

up to 10% of patients and may range from portal vein thrombosis (PVT) to pulmonary
embolism (PE) and deep vein thrombosis (DVT). Up to now there exist no recommendations
for the duration and intensity of prophylactic anticoagulation, which usually follows local
institutional protocols. We report on three consecutive patients with severe portal vein
thrombosis and/or pulmonary embolism—one with fatal outcome—7 to 35 days after splen-
ectomy for autoimmune hemolytic anemia, immunothrombocytopenia, and indolent lym-
phoma, respectively. Incidence and pathophysiology of thromboembolic events (TE) in this
patient group as well as prophylactic anticoagulation will be discussed, including a review
of the current literature on this topic. Am. J. Hematol. 76:143–147, 2004. ª 2004 Wiley-Liss, Inc.
Key words: splenectomy; hematologic diseases; thromboembolism; prophylactic anti-
coagulation

INTRODUCTION heparin until complete mobilization, had no prior

history of thrombophilia, and exhibited no further
Splenectomy is performed for diagnosis or treatment
obvious clinical risk factors for TE, such as prolonged
of a variety of hematologic diseases such as immune
immobilization, infection, or heart failure except for
cytopenia, indolent non-Hodgkin’s lymphoma (NHL),
smoking in patient I.
and, rarely, for myeloproliferative diseases (MPD) [1].
Within the recent years, laparoscopic splenectomy has
been increasingly employed when possible and is asso- PATIENTS
ciated with a decreased rate of intraoperative organ Patient I
damage and quicker postoperative recuperation of the
patient [2]. A 24-year-old female patient with a 2-year history
Postoperative complications most often comprise of autoimmune hemolytic anemia of the warm anti-
infections, particularly with encapsulated organisms body type had been successfully treated with pred-
such as pneumococci or haemophilus influenzae type nisone and relapsed under a maintenance dose of
B following a sometimes fulminant course, thus pre- 5 mg daily. The hemoglobin normalized when steroids
operative vaccination has become standard practice [2]. were increased to 100 mg/day, and the patient was
Additionally, patients with immunethrombocytopenia referred for splenectomy after the dose was tapered to
and low platelet counts may have an increased peri- <40 mg/day. Laparoscopic splenectomy (spleen weight
operative bleeding risk. In contrast to the former, 395 g) was performed, and after quick recovery she was
thromboembolic risk has so far attracted considerably *Correspondence to: Dr. Martin Mohren, Klinik für Hämatologie/
less attention in this patient group, and published Onkologie, Universität Magdeburg, Leipziger Str. 44, 39120
reports of thromboembolism (TE) so far have come Magdeburg, Germany.
from surgeons rather than hematologists. E-mail: Martin.Mohren@medizin.uni-magdeburg.de
Within a period of 6 months we saw severe TE in
Received for publication 26 May 2003; Accepted 17 October 2003
3 patients several days to weeks after splenectomy
for various hematologic disorders. All 3 patients had Published online in Wiley InterScience (www.interscience.wiley.com).
received anticoagulation with low molecular weight DOI: 10.1002/ajh.20018
ª 2004 Wiley-Liss, Inc.
144 Case Report: Mohren et al.

Fig. 2. Inferior cava vein thrombosis.

Fig. 1. Portal vein thrombosis with Perfusion defects in
the liver.

discharged on day 7 after the operation with a platelet

count of 519 G/l. Anticoagulation with low molecular
weight heparin was carried out until discharge. The
next day, the patient collapsed at home and was
found to have extensive pulmonary embolism as well
as portal vein thrombosis (Fig. 1) on CT scan. Platelets
were at 499 G/l. Although anticoagulation with full-
dose heparin was started immediately, the further
course was complicated by mesenteric vein thrombosis
with severe bowel ischemia, subsequently leading to
Fig. 3. Right pulmonary embolism.
death from multiorgan failure.
Patient III
Patient II A 63-year-old male patient presented to our clinic
A 21-year-old male patient with chronic immune- because of left-sided upper abdominal pain secondary
thrombocytopenia first diagnosed 8 years prior to to splenomegaly. Bone marrow histology revealed infil-
admission had received a short course of therapy with tration by indolent lymphoma, and open splenectomy
immunoglobulins and steroids, whereafter the platelet was performed, yielding a massively enlarged organ, of
count had remained stable at 70 G/l with no further size 24.5  14  6.5 cm and weight 1,660 g. Histologic
medication. At relapse, high steroid doses were neces- findings were consistent with marginal zone lymphoma.
sary to maintain platelets above 40 G/l, and splenec- There was also involvement of abdominal lymph nodes.
tomy was recommended. Laparoscopic splenectomy The postoperative course was uneventful, and he
(spleen weight 350 g) was carried out at a platelet received prophylactic anticoagulation with low mole-
count of 60 G/l with no bleeding complications. cular weight heparin for 30 days due to our previous
He received anticoagulation with low molecular experience. However, on day 35 postsplenectomy, this
weight heparin until discharge on day 5 postsplenect- patient experienced chest pain and dyspnoea of
omy. By that time, platelets were at 360 G/l. Two sudden onset; ventilation and perfusion scintigraphy
days later the patient complained of acute-onset revealed pulmonary embolism in segment 2 in both
shortness of breath and left-sided upper abdominal lungs. The platelet count was at 1,158 G/l. Full-dose
pain. The platelet count was at 1,013 G/l. Embolism heparin was started and was later switched to a vita-
of the right pulmonary artery and portal and inferior min K antagonist for a period of 6 months.
cava vein thrombosis (Fig. 2 and 3) were diagnosed,
and full-dose heparin was begun immediately fol-
DISCUSSION
lowed by oral anticoagulation for 1 year. He is cur-
rently well with no further anti-coagulation and/or We saw 3 patients with severe thromboembolic
immunosuppressive medication. events (TE) after splenectomy for hematologic
 Case Report: Thromboembolic Complications After Splenectomy 145

diseases within a short period of time. TE is a rare patients [13]. In one report, 4 of 8 patients with post-
complication of splenectomy that usually does not splenectomy PVT developed TE later in the course
receive as much attention as secondary infections or [5], whereas 2 of our patients had both, PVT and PE
hemorrhage. However, an increased incidence of TE, at the same time.
portal vein thrombosis in particular, following splen- So far the pathogenesis of TE after splenectomy is
ectomy has been reported in patients with hematolo- poorly understood.
gic diseases. Portal vein thrombosis may result from local fac-
A Dutch study of 563 splenectomies published in tors, such as intraoperative manipulation of visceral
2000 found postoperative portal vein thrombosis in vessels, the splenic vein in particular [14], and may
9 patients (2%). The latter was more likely to occur account for the particular risk in patients with
in patients with hematologic diseases, such as auto- massively enlarged organs. Five of 31 patients (16%)
immune hemolytic anemia and myeloproliferative with mesenteric venous thrombosis (MVT)—a rare
syndrome (incidence 10%) [3]. complication of PVT—had previously undergone
In a recently published report investigating portal splenectomy, whereas 13 (42%) of these patients had
vein thrombosis after splenectomy, an incidence of a hypercoagulable state (protein C, protein S, and
8% was found among 101 patients, 74% of whom ATIII deficiency) without local risk factors [15]. Mes-
had hematologic disease [4]. Among 8 patients who enteric thrombosis, as was also seen in patient I of
developed PVT, 4 had a myeloproliferative disorder. our report, is associated with a poor prognosis due
Interestingly PVT occurred as late as 3 years follow- to subsequent bowel ischemia and multiorgan failure
ing splenectomy [4]. [4,15,16].
Another report of 223 splenectomies in patients with Thrombocytosis that accompanies splenectomy in
myeloid metaplasia, however, detected thrombosis most cases may, although poorly defined, play an
(location not further specified) in 7.2% only. Postsplen- etiologic role.
ectomy thrombocytosis in these patients was associated In several reports, large spleen size, thrombocytosis,
with postoperative thrombosis. Unfortunately, platelet and a myeloproliferative disease (MPD) as the under-
numbers are not provided by the authors [5]. lying disease were described as risk factors for TE [4,7].
An additional analysis of 26 splenectomized patients Patients with MPD and a large spleen (>3,000 g) had
with myelofibrosis revealed venous thrombosis in 12% a 75% incidence of PVT [4]. Splenic weight >1,000 g
[6]. In an American investigation carried out with 50 was found to be associated with significant morbidity
splenectomized patients whose underlying disease was after laparoscopic splenectomy [17], yet none of our
not further specified, 5 of 50 patients (10%) were found patients with severe TE had MPD and only one patient
to develop PVT that was then successfully treated with had a massively enlarged spleen.
anticoagulation [7]. In an Italian report on patients The association of postsplenectomy thrombocyto-
undergoing splenectomy for treatment of thalassemia, sis and PVT is unclear, particularly in patients with-
an incidence of TE as high as 29% was seen [8]. out MPD, since not all patients with thrombocytosis
One of 12 patients with immunethrombocytopenia develop PVT and PVT also occurs in patients with
developed PVT without any further complications in normal platelets [4]. Patient I in our report, for exam-
another Italian report [9]. A French group performed ple, had slightly elevated platelets only (499 G/l), and
repeat Doppler ultrasound studies in 60 consecutive not all of the reported patients with MVT following
splenectomized patients on days 7 and 30 postopera- splenectomy had thrombocytosis [15].
tively and found 1 symptomatic and 3 asymptomatic In a study of 129 patients with extreme thrombo-
PVT (8%) in this patient cohort [10]. Thus asympto- cytosis (>1,000 G/l), 72 in MPD and 57 with reactive
matic PVT may occur more frequent than clinically thrombocytosis, thrombosis was found in 3–4% [18],
apparent TE. A review of imaging findings in postsplen- but a prospective observational study of patients with
ectomy patients revealed PVT in 12 of 123 patients essential thrombocythemia (ET) and a platelet count
(9,8%), all of whom had hematologic disease [11]. A <1,500 G/l as compared to an age- and sex-matched
small number of TE (1%) was reported by a French control group failed to show an increased risk for
group that performed laparoscopic splenectomy in 275 thrombosis in ET patients [19]. A recent study investi-
patients with hematologic disease [12], and a lower gating etiology and clinical significance of thrombocy-
rate of TE was also found in another report on laparo- tosis found a higher incidence of venous and arterial
scopic splenectomy [5]. In contrast to these findings, thrombosis in patients with primary thrombocytosis
2 of our patients with severe TE had undergone laparo- as compared to patients with secondary thrombocyto-
scopic splenectomy. sis (12.4% vs. 1.6%). The authors conclude that post-
There is little data on the incidence of TE other splenectomy thrombocytosis is not associated with an
than PVT, such as DVT and/or PE in splenectomized increased risk for hemostatic complications [20]. Thus
146 Case Report: Mohren et al.

thrombocytosis by itself does not seem an indication approach may not be sufficient in preventing TE in
to initiate anti-coagulation or antiplatelet or platelet- splenectomized patients with hematologic disease.
lowering therapy unless it is being used as secondary In concordance with our findings, the majority of
prophylaxis. patients (approximately 70%) developed PVT while
There exists little data on activation of plasmatic receiving prophylactic anticoagulation with low-dose
coagulation and/or lack of coagulation inhibitors, heparin [4,8]. Although a combination of heparin and
such as protein C or S deficiency after splenectomy or antiplatelet agents or even vitamin K antagonists has
the presence of the factor V Leiden mutation or anti- been suggested in high-risk splenectomized patients
phospholipid antibodies. Few reports demonstrated a [4], its use is problematic due to an increased risk of
systemic hypercoaguable state in patients with PVT bleeding in the postoperative period and the abnor-
[14,15], but these reports focused on the etiology of mal platelet function seen in some patients with
PVT and MVT without special attention to the post- hematologic disease [5]. However, routine postopera-
splenectomy state. tive surveillance ultrasound imaging may be of bene-
Disseminated intravascular coagulation was not fit in patients with myeloproliferative disease and a
found in splenectomized patients in a systemic evalua- large spleen [11] for prompt diagnosis of PVT.
tion [5].

How can TE be prevented in splenectomized CONCLUSIONS

patients?
Although it does not receive as much attention as
Preventive measures still rely preferentially on per- infectious complications, TE occurs in approximately
sonal experience, because accumulated data is incon- 10% of splenectomized patients, and prophylactic
clusive and general recommendations for duration anticoagulation is warranted in patients undergoing
and intensity of anticoagulation after splenectomy splenectomy for hematologic diseases. Duration and
do not exist [2,21]. intensity of mandatory anticoagulation has yet to
So far there is little evidence indicating that the be established. At our institution, patients currently
method of splenectomy has an impact on subsequent receive low molecular weight heparin for a period of
thromboembolic complications. General prophylactic 30 days after splenectomy. However, as has been
measures such as early mobilization and refraining shown in one of our patients and in further previous
from smoking may be of some help. Until now, pro- reports, TE may occur ever after a prolonged time
phylactic low-dose anticoagulation has failed to show interval after surgery, thus physicians as well as
sufficient effect [4] to prevent PVT but may reduce the patients should be alert to symptoms of TE in splen-
risk for TE in other locations, such as deep venous ectomized patients, even years after the operation to
thrombosis and pulmonary embolism. However, 3 of ensure rapid diagnosis and proper treatment.
our patients had pulmonary embolism—2 of whom
also had portal vein and inferior cava vein and/or
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