DOI 10.1515/crpm-2013-0046 Case Rep. Perinat. Med. 2014; 3(1): 27–30
Chana Adler-Lazarovits*, Moshe Mazor and Offer Erez
Autoimmunity, preeclampsia and splenic rupture:
a case report and literature review
Abstract: Antiphospholipid antibody (APLA) syndrome is
an autoimmune disease which is associated with preeclampsia and can cause thromboembolic events in several
organs including the spleen. This report includes a case
of post-partum splenic rupture in a woman with pre­
eclampsia in the presence of APLA syndrome and a literature review of splenic rupture during the third trimester
and puerperium. Unlike the prominent clinical manifestation of liver hematoma and rupture during preeclampsia, rupture of the spleen can be silent and mistakenly
underdiagnosed.
Keywords: Antiphospholipid antibody syndrome; post
thrombotic hemorrhage; preeclampsia; splenic rupture.
*Corresponding author: Chana Adler-Lazarovits, MD, Department of
Obstetrics and Gynecology, Soroka University Medical Center, School
of Medicine, Faculty of Health Sciences, Ben Gurion University of the
Negev, P.O. Box 151, Beer Sheva 8410101, Israel, Tel.: +972 86403551,
Fax: +972 86403294, E-mail: [email protected]
Moshe Mazor and Offer Erez: Department of Obstetrics and
Gynecology, Soroka University Medical Center, School of Medicine,
Faculty of Health Sciences, Ben Gurion University of the Negev, Beer
Sheva, Israel
Introduction
We present an atypical case of a woman with preeclampsia
who had a post-partum splenic rupture that was managed
conservatively. Subsequently, she was diagnosed with
antiphospholipid antibody (APLA) syndrome and autoimmune hemolytic anemia. Up until this report, all cases of
splenic rupture that had been reported resulted in a splenectomy due to their severity. Our report suggests the possibility
that subtle cases of splenic rupture may be underdiagnosed
and proposes that splenic involvement in preeclampsia may
have a similar mechanism to that of hepatic involvement.
Case report
A 24-year-old primigravida, at 35 weeks’ gestation, presented to the obstetrical emergency department with
bilateral leg pain and elevated blood pressure. During
gestation, she was treated with low-molecular-weight
heparin (LMWH) due to a prior deep vein thrombosis
(DVT) at the age of 18 years. Thrombophilia work-up
was not done prior to pregnancy. Upon admission, her
blood pressure was 150/91 mm Hg with bilateral +3 leg
edema without any other significant findings. Laboratory results showed mild proteinuria, lactate dehydrogenase (LDH) of 820 U/L, hemoglobin of 7.1 g/dL, negative
schistocytes and prolonged partial thromboplastin time
of 73 s. Liver enzymes, bilirubin, platelet count, prothrombin time and fibrinogen levels were normal. Fetal
ultrasound exam demonstrated an estimated fetal
weight compatible with the 10th percentile. Due to fetal
growth restriction, hemoglobin decrease and the possibility of hemolysis according to the laboratory findings,
the patient was diagnosed as having severe preeclampsia [1]. Infusion of magnesium sulfate was initiated as
well as treatment with 2 U of packed red blood cells;
eventually, the patient delivered a healthy newborn
weighing 2130 g, by an uneventful cesarean delivery due
to failed induction.
During puerperium, hemoglobin levels decreased
furthermore to 6.1 g/dL, with no obvious vaginal or intraabdominal bleeding, and the patient was transfused with
additional 2 U of packed red blood cells. In addition, due
to her complaints regarding leg pains, she was treated with
a therapeutic dosage of LMWH for 2 days until a repeated
venous duplex scan was performed, demonstrating only
an old left femoropopliteal vein DVT. In spite of repeated
blood transfusions, the patient’s hemoglobin concentration decreased to 5.5 g/dL with no hemodynamic compromise. In addition, she had elevated LDH levels, a repeated
test for schistocytes that turned positive in her blood
smear, and a positive direct as well as an indirect Coombs
test, with no specific alloantibodies. A possible diagnosis of autoimmune hemolytic anemia was suggested. In
addition, in search of the source of the intra-abdominal
bleeding, an abdominal computed tomography was performed, demonstrating an enlarged spleen with surrounding varicose veins (see Figure 1), left spleno-renal shunt,
third-grade splenic multiple ruptures and a large splenic
hematoma without active bleeding (see Figure 2). An additional 2 U of packed red blood cells was transfused, and
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28 Adler-Lazarovits et al., Autoimmunity, preeclampsia and splenic rupture
demonstrating an enlarged spleen with a hematoma in
the inferior pole with no impending rupture; so conservative management was chosen and splenectomy
was not performed. Concomitantly, high concentrations
of antiphospholipid antibodies supported the diagnosis of APLA syndrome (anti-cardiolipin IgM 70 U/mL,
anti-cardiolipin IgG 79 U/mL, anti-β2 glycoprotein I IgM
188 U/mL, anti-β2 glycoprotein I IgG 47 U/mL and β2
microglobulin 3490 μg/L), while anti-nuclear antibodies and double-stranded DNA antibodies were negative;
thus systemic lupus erythematous was ruled out. Treatment with oral glucocorticosteroids and LMWH were
initiated, and the patient was discharged 3 weeks after
admission for ambulatory follow-up.
Discussion
Figure 1 A computed tomography image of an enlarged spleen.
Enlarged spleen (S) marked by an arrow; note the splenic size
compared to the liver (L).
the patient was transferred to the intensive care unit (ICU)
for observation.
At the ICU, the patient continued to be symptomatic;
as a result, an investigating laparoscopy was performed,
Figure 2 A computed tomography image of a splenic rupture. Area
of splenic hemorrhage marked by an arrow.
Splenic rupture during the third trimester or post-partum is a rare event. We searched the literature using
MEDLINE for all cases reported in apparently normal
spleens since 1988 using the terms postpartum/postpartum/pregnancy and splenic rupture (see Table 1).
Cases of rupture occurring in the first trimester, or in
association with a prior known pathology of the spleen,
were excluded. Of note, all cases resulted with a splenectomy, two cases occurred following LMWH treatment, and four others in association with preeclampsia/
eclampsia.
The etiology of splenic rupture is traditionally
divided into traumatic or spontaneous rupture. The latter
is usually associated with a prior splenic disease, which
is then termed pathological rupture. The differential diagnosis of pathological splenic rupture is diverse including infections, malignancies, hematological disorders
influencing the spleen or cardiovascular disorders [12].
In our patient, a possible cause for rupture was hemolytic anemia (which caused enlargement of the spleen)
or possible splenic thrombosis as demonstrated by the
tomographic picture of the surrounding varicose veins.
Similar to other thrombotic events, splenic thrombosis
can be a manifestation of APLA syndrome, as shown in
prior studies [2, 13], making APLA syndrome a possible
cause for splenic rupture.
Preeclampsia, which is known to be associated
with APLA syndrome [8], might be an additional cause
of pathological splenic rupture in our case. Although
the association between preeclampsia and hepatic
subcapsular or ruptured hematoma is well known,
the relationship with splenic rupture is infrequent.
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Adler-Lazarovits et al., Autoimmunity, preeclampsia and splenic rupture 29
Table 1 Case reports of splenic rupture during pregnancy or post-partum.a,b
Case Presenting symptom and outcome
Additional diagnosis
Rupture
timing
1 G1P0, 34 weeks’ gestation, acute left upper abdominal pain.
Emergent cesarean due to suspected placental abruption, delivery of stillbirth [18].
Placenta previa
Antepartum
2 G3P2, term pregnancy, two previous cesarean deliveries, chronic
hypertension.
Repeated cesarean section due to contractions [16].
Postpartum
Superimposed preeclampsia and
HELLP syndrome
3 G5P1, 41 weeks’ gestation, one previous cesarean delivery.
Elective repeated cesarean section [14].
Antithrombin-III deficiency,
anticoagulation
Postpartum
4 G1P0, 28 weeks’ gestation, upper left abdominal pain.
Delivery of stillbirth and patient death [7].
Carrier of double heterozygosis for
hemoglobin C/β-thalassemia
Antepartum
5 G4P2, 34 weeks’ gestation, diffuse abdominal pain.
Emergent cesarean section due to suspected placental abruption [15]. Homozygous MTHFR deficiency,
anticoagulation
Antepartum
6 38 years old with unknown gestational age, hemodynamic collapse.
Emergent cesarean section, newborn with severe perinatal
asphyxiation [6].
Antepartum
7 G6P3, 32 weeks’ gestation, one previous cesarean delivery.
Elevated blood pressure and severe headache.
Delivery of stillbirth and patient death [11].
Preeclampsia, ruptured liver
Antepartum
8 G2P0, 36 weeks’ gestation, triplet pregnancy.
Cesarean hysterectomy due to uterine atony [17].
Eclampsia
Postpartum
9 G6P4, 36 weeks’ gestation, stillbirth delivery at patient’s home.
Hysterectomy due to failed removal of placenta and massive bleeding.
Abdominal distension post surgery [17].
Cholestasis
Postpartum
10 G2P1, 37 weeks’ gestation.
Elevated blood pressure and proteinuria [3].
Severe preeclampsia
Postpartum
11 G1P0, term pregnancy and delivery.
Left hypochondrial pain with radiation to shoulders [5].
Huge splenic cyst
Postpartum
12 G3P2, term pregnancy, abdominal and back pain.
Emergent cesarean section due to fetal bradycardia [9].
Partial splenic torsion, presumably during labor
Antepartum
All cases went through splenectomy and had normal spleens on pathological examination, except cases number 4 and number 11.
G=Gravidity; P=Parity; HELLP=Hemolysis, Elevated Liver enzymes, Low Platelets; MTHFR=Methyl-Tetra-Hydro-Folate-Reductase.
a
b
Indeed, we found only four publications describing splenic rupture in the presence of preeclampsia. Barton and Sibai [4], who reviewed the entity of
acute pancreatitis associated with preeclampsia, suggested that vascular endothelial injury can be the
underlying cause of pancreatic injury as it is in other
manifestations of preeclampsia. We propose that a
similar mechanism of vascular injury and thrombosis, especially in the presence of APLA syndrome,
can be the cause of postpartum splenic rupture complicating preeclampsia. Moreover, it is possible that
some cases remain sub-clinical and underdiagnosed,
provided that the patient is hemodynamically stable.
In such cases with concealed bleeding, the spleen
can be spared and conservative management can
be used.
In summary, we suggest that the unique combination of APLA syndrome and preeclampsia could have
caused splenic rupture following a thrombotic hemorrhage in the splenic vasculature caused by an APLA
syndrome thrombotic event and enhanced by the vasculature damage in a similar pattern associated with
preeclampsia. Since scarce previous reports [10] failed
to demonstrate any correlation between anticoagulation
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30 Adler-Lazarovits et al., Autoimmunity, preeclampsia and splenic rupture
treatment and splenic rupture, it is not clear whether
it had an additional effect. Our case suggests that, in
the absence of hemodynamic compromise, diagnosis
of splenic rupture can be delayed or misdiagnosed. It
is therefore vital for clinicians to consider the possibility of concealed splenic bleeding in patients with APLA
syndrome and/or preeclampsia, since symptoms may be
subtle and implications may be severe.
Received April 30, 2013. Accepted October 8, 2013. Previously
published online November 2, 2013.
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The authors stated that there are no conflicts of interest regarding
the publication of this article.
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