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| CASE REPORT |
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| Year : 2022 | Volume
: 42
| Issue : 2 | Page : 95-97 |
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Vaginal delivery with immune thrombocytopenic purpura-induced severe thrombocytopenia
Yu-Hsuan Chen1, Yeu-Chin Chen2, Cheng-Chang Chang1, Kuo-Min Su1
1 Department of Obstetrics and Gynecology, National Defense Medical Center, Tri-Service General Hospital, Taipei, Taiwan
2 Department of Medicine, Division of Hematology and Oncology, Defense Medical Center, Tri-Service General Hospital, Taipei, Taiwan
| Date of Submission | 15-Dec-2020 |
| Date of Decision | 18-Mar-2021 |
| Date of Acceptance | 18-May-2021 |
| Date of Web Publication | 14-Jun-2021 |
Correspondence Address:
Dr. Kuo-Min Su
Department of Obstetrics and Gynecology, Tri-Service General Hospital, National Defense Medical Center, Taipei
Taiwan
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jmedsci.jmedsci_417_20
Idiopathic thrombocytopenic purpura (ITP) is the most common immune-related thrombocytopenia during pregnancy. Here, we present a case of ITP with acute flare-up during pregnancy. Worsening thrombocytopenia dropped the platelet count to 11,000/dL. Steroid treatment, intravenous immunoglobulin (IVIG) infusion, and anti-CD20 antibody therapy were all unsuccessful. Fortunately, no further critical maternal or fetal complications developed. Transient neonatal thrombocytosis was noted after delivery but was corrected with IVIG.
Keywords: Idiopathic thrombocytopenia purpura, intravenous immunoglobulin, pregnancy, pulse therapy, anti-CD20 antibody
How to cite this article:
Chen YH, Chen YC, Chang CC, Su KM. Vaginal delivery with immune thrombocytopenic purpura-induced severe thrombocytopenia. J Med Sci 2022;42:95-7 |
| Introduction | |  |
Thrombocytopenia is the second most common abnormal hematological disorder, affecting about 7%–11% of pregnancies[1] and characterized by a platelet count of <150,000/dL. Thrombocytopenia during pregnancy may be asymptomatic and self-limited, such as gestational thrombocytopenia. However, it could be life-threatening under several complications, such as preeclampsia and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Therefore, identifying the etiology is important and should always be the very first step of the clinical physician.
The most common cause of thrombocytopenia is gestational thrombocytopenia, accounting for about 76% of the incidence, and is thought to be self-limited without maternal or fetal complication. The platelet count is usually between 100,000 and 150,000/dL, rarely <80,000/dL.[2] Preeclampsia and HELLP syndrome usually accompany with hypertension and occur after 20 weeks gestation, accounting for about 20% of cases of thrombocytopenia during pregnancy. These conditions are the second leading cause of thrombocytopenia preceded only by gestational thrombocytopenia. Termination of pregnancy is the only curative therapy for the patients with all these diseases.
Immune-related thrombocytopenia during pregnancy accounts for 11% of thrombocytopenia during pregnancy. The primary form, idiopathic thrombocytopenic purpura (ITP), is the most common immune-related thrombocytopenia that affects 0.2% of pregnancies.[3] According to the accumulated clinical experience in the past, in addition to usual blood transfusions with concentrate platelets, there are also steroids and intravenous immunoglobulin (IVIG) therapies for the treatment of ITP with real effectiveness. Rituximab was also considered as a safe therapeutic agent used for ITP during pregnancy.[4] Herein, we present a case of chronic ITP presenting with an acute flare-up during pregnancy and poor response to conventional medical therapy.
| Case Report | | |
The patient was a 27-year-old pregnant female, gravida 2 parity 0, with a history of one spontaneous abortion. She had been previously treated for chronic ITP with poor compliance and irregular follow-up for 6 years prior. The patient visited a local hospital for a prenatal checkup. The baseline platelet count of the patient was about 60,000–80,000/dL before pregnancy. However, thrombocytopenia severity progressed after 24 weeks gestation, with the platelet count dropping to 22,000/dL and skin purpura over four limbs. Patient condition failed to respond to corticosteroid treatment and pulse therapy (high-dose steroid treatment over a short period). Side effects of steroid treatment such as moon face, truncal obesity, and weight gain gradually developed. The patient was transferred to the medical center for further evaluation and management.
Owing to the severe thrombocytopenia and poor response to steroid therapy, we placed the patient initially on a low-dose oral corticosteroid course and then weekly anti-CD20 monoclonal antibody therapy (rituximab 100 mg per week). After consulting with a hematologist and discussing with the patient and her family, IVIG infusion was administered until 35 weeks of gestational age due its relatively high cost and short duration. However, thrombocytopenia worsened, even after four doses of anti-CD20 monoclonal antibody, with the platelet count dropping critically to 11,000–14,000/dL. We suggested close observation due to lack of active bleeding or further symptoms.
Unexpectedly, the patient was sent to our emergency room due to preterm labor at 35 1/7 weeks gestation with vaginal bleeding and intermittent lower abdominal pain. On admission, uterine contractions were regular at 5–6 min, and cervical dilation was at 2 cm. Platelet count was 12,000/dL. Empiric antibiotics, prenatal corticosteroids (betamethasone), and a tocolytic agent were administered. Platelet transfusion with single-donor platelets at 2 units and IVIG at a dosage of 2 g/kg were also prescribed for severe thrombocytopenia with active bleeding (bloody show). The treatments are shown in [Figure 1]. Nonetheless, the platelet count gradually dropped to 11,000/dL within 5 days. The cervix dilated to 4 cm and preterm labor was inevitable. A premature infant was eventually delivered via vaginal delivery with the platelet count at 11,000/dL. Fortunately, delivery ensued smoothly, and no further bleeding occurred. Total blood loss was about 300 ml. The Apgar score at 1 and 5 min after delivery were 8 and 9, respectively. Pulse therapy with betamethasone at 15 mg/day was prescribed for 3 consecutive days after delivery. The platelet count final began to rise and stabilized at around 140,000/dL after delivery. In addition, the purpura on the limbs and trunk gradually disappeared after birth. | Figure 1: Platelet count during the entire therapeutic course. No response to medication including steroids, intravenous immunoglobulin, and rituximab. Platelet transfusion increased the platelet count but was not sustained
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The infant was admitted to the intensive care unit after delivery due to prematurity (35 6/7 weeks), low birth weight (2060 g), and severe thrombocytopenia (platelet count 5000/dL). IVIG infusion at a dosage of 2 g/kg was administered. The platelet count of the premature infant gradually increased to 15,000/dL without bleeding tendency. No intracerebral hemorrhage (ICH) was noted.
| Discussion | | |
Thrombocytopenia is a common hematological problem during pregnancy.[1] Immune-related thrombocytopenia accounts for 11% of thrombocytopenia cases during pregnancy.[2] ITP may cause pregnancy complications such as fetal loss, stillbirth, preterm birth,[3] and increased risk of neonatal ICH during labor or delivery.[5]
The treatments of ITP during pregnancy should be tailored according to gestational age, symptoms, and cost-effectiveness. Apart from transfusion therapy, corticosteroid is the first choice of treatment due to its effectiveness, lower cost, and a longer therapeutic window. However, side effects of steroid usage such as moon face, central obesity, peptic ulcer, and hyperglycemia should be noted. IVIG infusion is a more effective option compared with steroid treatment. Its advantages include immediate results (24–72 h for IVIG compared with 3–7 days for steroids) and its relative safety for use throughout all three trimesters of pregnancy (steroids should be avoided in the first trimester). The main disadvantages are the higher cost and relatively shorter therapeutic effect (<2 weeks).
So far, no formal standards have been established on optimal platelet count during pregnancy and during delivery.[6] Postpartum hemorrhage occurs less commonly with ITP, and no relevant correlation has been established between maternal platelet count and amount of blood loss during delivery. Obstetric complications such as uterine atony or residual placenta are still the major causes of postpartum hemorrhage.[5],[6] However, epidural and spinal anesthesia may have risk of procedure-related bleeding (spinal hematoma) due to thrombocytopenia. The ideal target of platelet count was also not established until now[7] and varied from institution to institution. The current consensus target of platelet count for spinal anesthesia and epidural anesthesia was around 80,000–100,000/dL. The patient may need platelet transfusion to correct thrombocytopenia before procedure if need.
A recent study demonstrated that neither vaginal delivery nor cesarean section was correlated with maternal or fetal outcomes.[8] In the 1970s, routine cesarean section was advised to prevent fetal ICH in cases of thrombocytopenia and ITP. However, a recent study revealed that fetal ICH is a rare occurrence (0%–1.5%) and is not prevented by routine cesarean section.[9]
In summary, ITP may induce severe thrombocytopenia during pregnancy; blood transfusion, steroid administration, IVIG infusion, and anti-CD20 antibody therapy may have proper effects under close monitoring. These treatment experiences will guide obstetricians to improve the health of these patients and their fetuses during delivery.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Reese JA, Peck JD, Deschamps DR, McIntosh JJ, Knudtson EJ, Terrell DR, et al. Platelet counts during pregnancy. N Engl J Med 2018;379:32-43.  |
| 2. | Parnas M, Sheiner E, Shoham-Vardi I, Burstein E, Yermiahu T, Levi I, et al. Moderate to severe thrombocytopenia during pregnancy. Eur J Obstet Gynecol Reprod Biol 2006;128:163-8. |
| 3. | Wyszynski DF, Carman WJ, Cantor AB, Graham JM Jr., Kunz LH, Slavotinek AM, et al. Pregnancy and birth outcomes among women with idiopathic thrombocytopenic purpura. J Pregnancy 2016;2016:8297407. |
| 4. | Webert KE, Mittal R, Sigouin C, Heddle NM, Kelton JG. A retrospective 11-year analysis of obstetric patients with idiopathic thrombocytopenic purpura. Blood 2003;102:4306-11. |
| 5. | Chakravarty EF, Murray ER, Kelman A, Farmer P. Pregnancy outcomes after maternal exposure to rituximab. Blood 2011;117:1499-506. |
| 6. | Fujita A, Sakai R, Matsuura S, Yamamoto W, Ohshima R, Kuwabara H, et al. A retrospective analysis of obstetric patients with idiopathic thrombocytopenic purpura: A single center study. Int J Hematol 2010;92:463-7. |
| 7. | Estcourt LJ, Malouf R, Hopewell S, Doree C, Van Veen J. Use of platelet transfusions prior to lumbar punctures or epidural anaesthesia for the prevention of complications in people with thrombocytopenia. Cochrane Database Syst Rev 2018;4:CD011980. |
| 8. | ACOG Practice Bulletin No. 207 Summary: Thrombocytopenia in Pregnancy. Obstet Gynecol 2019;133:589-91. |
| 9. | British Committee for Standards in Haematology General Haematology Task Force. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. Br J Haematol 2003;120:574-96. |
[Figure 1]
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