Case Report
The 23-year-old patient was admitted to the hospital for ”26+5 weeks of
amenorrhea and 5+ hours of epistaxis.” During the whole pregnancy, the
patient repeatedly had nosebleed and gingival bleed, by oneself
alleviate. 10+ years ago, she was diagnosed as ”myelodysplastic
syndrome” by bone marrow puncture, and repeatedly had blood and platelet
transfusions. With long-term oral medication (self-purchased hormones
and traditional Chinese medicine), the condition is basically stable.
Physical Examination: T 39.7 ° C, P 127 times/min, R 25 times/min, BP
131/64 mmHg. The whole body is filled with
scattered petechia and ecchymosis,
with bleeding gums and intranasal blood clots. Gynecological
Examination: she had no uterine contraction, but we found a small amount
of bloody secretion in the vagina. Fetal Color Doppler Ultrasound:
intrauterine single live birth (equivalent to 24 weeks of pregnancy)
with breech position, single peak in S/D(Umbilical Systolic pressure /
Diastolic pressure of the fetus)value. Laboratory Examination: WBC
count 3.0×109 /L, N 53.2%, Hb 2.8 g/dL, Plt count
2×109 /L.
After admission, the patient developed subconjunctival hemorrhage,
impaired vision, and increased petechia and ecchymosis. The blood
culture indicated Klebsiella pneumoniae infection.
We gave her Jisaixin Recombinant
Human Granulocyte Colony-stimulating Factor Injection, transfused 6 U of
erythrocyte suspension without WBC and 4U of apheresis platelet in total
within 8 times, and gave her piperacillin and meropenem against
infection. Blood routine test: WBC count 0.9 × 109 /
L, Hb 4.1g / dL, Plt count 1 × 109 / L. The result of
Blood routine indicated that the blood transfusions were not effective.
The patient’s platelets were extremely reduced, we considering the
presence of platelet and red blood cell antibodies in her blood vessels.
After multidisciplinary discussion in departments of obstetrics,
anesthesiology, hematology, infection department, and department of
critical medicine, we transfused again with 1 U of cross-matched
platelets, 1.5 U of erythrocyte suspension without WBC, and the
reexamination of Blood routine showed Hb was 4.3 g/ dL, and Plt count
was 47×109 /L. Rivanol was then injected into the
amniotic cavity, and 72 hours later, the labor process started after
propess was placed in the vagina. 1U of cross-matched platelets and 1.5
U of erythrocyte suspension without WBC were re-transfused to the
patient half an hour before delivery. In order to shorten the labor
process, a stillbirth was delivered using breech traction. The fetus was
36 cm in length, weighed 1010 g, and the patient had 300 ml of bleeding.
Postpartum review of blood routine test: WBC count
2.5×109 /L, Hb 52 g/L, Plt count
43×109 /L (table). The patient complained of a
relatively stable condition in the telephone follow-up 42 days later.
Discussion
Myelodysplastic syndrome (MDS) is a set of clonal disorders generating
from myeloid-oriented stem cells or pluripotent stem cells,the overall
incidence is reported in literature as 4/100,000, and the incidence
gradually increases with age, with rare cases under 40 years
old.1,2 Its main features include ineffective
hematopoiesis, cytogenetic and molecular biological abnormalities, and
high-risk transformation into acute myeloid leukemia (AML), which has
various clinical manifestations and is difficult to diagnose and
treat.3 Pregnancy complicated with myelodysplastic
syndrome is extremely rare, mostly reported in individual
cases,4-6 and is even more difficult to treat. There
is no unified norm in diagnosis and treatment at home and abroad.
The effect of pregnancy on myelodysplastic syndrome is still
controversial. Siddiqui7 et al. have reported cases of
conversion of myelodysplastic syndrome to AML during pregnancy, while
Ikeda8, Gidiri9 et al. believe that
pregnancy itself does not affect the outcome of myelodysplastic
syndrome, and the hematology situation of the patient can also be
improved after delivery. Our patient in this case takes the medicine on
her own at ordinary times, and her condition is basically stable.
However, nosebleed and gingival bleeding occurred repeatedly during her
pregnancy, suggesting that the condition might get aggravated. After
being discharged, the patient didn’t receive further treatment, and her
condition was relatively stable in the telephone follow-up 42 days
later. Therefore, we speculate on the possibility of pregnancy-induced
exacerbation.
The American Society of Hematology recommends that platelets of patients
with idiopathic thrombocytopenia maintain at least
50×109 /L before and during
delivery10. Steensma11 et al.
suggest that patients with myelodysplastic syndrome should maintain more
than 50×109 /L platelets if taking cesarean section
and 20-30×109 /L platelets if taking vaginal delivery.
In this case, our patient’s platelet count fluctuated at
1-2×109 /L, multiple hemorrhages occurred over the
body and became increasingly severe, and repeated platelet transfusions
were ineffective. If taking vaginal delivery, the induction of labor may
last for a long time , the use of induced labor drugs and abdominal
pressure during the labor process both may cause visceral and
intracranial hemorrhage. What’s more, the hemostasis of birth canal
bleeding is also difficult to treat. On the contrary, hemostatic
difficulty, massive bleeding, pelvic hematoma, subcutaneous hematoma are
all likely to appear during the cesarean operation. The patient has
severe anemia herself and has extremely low tolerance to blood loss. Our
experience is to try to increase her platelet counts to
20×109 /L and hemoglobin concentration to 4 g/dl for
induction of labor and attempt vaginal delivery. However, in this case,
there may exist anti-blood-cell and anti-platelet antibodies in the
patient’s body, and conventional transfusion was difficult to improve
the situation. There has been literature reported that cross-matched
platelets can be used for immune refractory
thrombocytopenia12, and the American Society of
Hematology recommends Prednisolone as
the first-line drug for pregnancy complicated with myelodysplastic
syndrome10. Hence, we had the patient take 30mg of
Prednisolone orally every day and transfused cross-matched platelets at
the same time to rapidly increase her platelets for induction of labor.
At the same time, in order to avoid bleeding, patients should be
prevented the use of abdominal pressure and given effective labor
analgesia during delivery (epidural analgesia can be applied if platelet
count is above 50×109 /L13).
The oncome of myelodysplastic syndrome is insidious, the clinical
manifestations are atypical, and the treatment is intractable.
Therefore, early diagnosis is especially important in this syndrome.
When there exist unexplained anemia, fever, and bleeding tendency during
pregnancy, hematological system diseases should be taken into
consideration. Medical staff should firstly test the patient’s blood
routine and leukocyte differential count, and if necessary, perform a
bone marrow puncture to clarify the diagnosis. This case report suggests
that pregnant patients with myelodysplastic syndrome should enhance the
management during pregnancy to determine whether there is any indication
to continue the pregnancy. At the time of delivery, whether to take
cesarean section or vaginal delivery, doctors should fully evaluate the
possibility of bleeding and prepare adequately.