Steroid-resistant immune thrombocytopenic purpura in pregnancy complicated by fetal growth restriction and oligohydramnios: A case report with anatomical and pathophysiological review

Gayathri A 1*, Gowdham Pannirselvam2

1In charge, NICU, Kauvery Hospital, Marathahalli, Bangalore

2Senior Nurse Educator Kauvery Hospital, Marathahalli, Bangalore

*Correspondence

Abstract

Immune thrombocytopenic purpura (ITP) is an acquired autoimmune haematological disorder characterized by accelerated platelet destruction and impaired platelet production, resulting in thrombocytopenia. The management of ITP during pregnancy is particularly challenging because of the associated maternal and fetal risks, including haemorrhage, fetal growth restriction (FGR), oligohydramnios, preterm birth, and neonatal thrombocytopenia. We report the case of a 24-year-old gravida 3 abortus 2 woman at 31 weeks and 4 days of gestation who presented with severe thrombocytopenia secondary to steroid-resistant ITP complicated by fetal growth restriction and oligohydramnios. Despite initial corticosteroid therapy, platelet counts remained critically low, necessitating treatment with intravenous immunoglobulin (IVIG). Following haematological stabilization, the patient underwent lower segment caesarean section (LSCS) and delivered a live preterm female infant weighing 1.5 kg. However, platelet counts declined significantly during the postpartum period, requiring escalation to Romiplostim and Rituximab therapy. This case highlights the complexity of managing refractory ITP during pregnancy and emphasizes the importance of multidisciplinary care involving haematologists, obstetricians, intensivists, neonatologists, and nursing professionals.

Keywords: Immune thrombocytopenic purpura; Pregnancy, Steroid-resistant ITP; Intravenous immunoglobulin; Fetal growth restriction; Oligohydramnios; Romiplostim; Rituximab.

 

Introduction

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia resulting from increased platelet destruction and impaired platelet production. Autoantibodies directed against platelet membrane glycoproteins lead to accelerated clearance of platelets by the reticuloendothelial system, particularly the spleen. ITP affects approximately 1–2 pregnancies per 1,000 deliveries and represents one of the most common causes of isolated thrombocytopenia during pregnancy.[1]

The management of ITP during pregnancy is often complex because clinicians must simultaneously consider maternal safety and fetal well-being. Severe thrombocytopenia may predispose patients to antepartum haemorrhage, postpartum haemorrhage, and complications associated with surgical interventions. Furthermore, maternal autoantibodies may cross the placenta and affect fetal platelet counts. Corticosteroids and intravenous immunoglobulin (IVIG) remain the first-line treatment modalities; however, a subset of patients fails to respond adequately and develops steroid-resistant ITP requiring advanced therapeutic interventions.[2]

The coexistence of fetal growth restriction and oligohydramnios further increases maternal and fetal risk and often necessitates early delivery. This case illustrates the successful multidisciplinary management of severe steroid-resistant ITP complicated by fetal growth restriction and oligohydramnios.

Disease Overview

Immune thrombocytopenic purpura is caused by the production of immunoglobulin G autoantibodies against platelet surface glycoproteins, particularly glycoprotein IIb/IIIa and glycoprotein Ib/IX. These antibodies bind circulating platelets and mark them for destruction by splenic macrophages. In addition to peripheral platelet destruction, autoantibodies can impair megakaryocyte maturation within the bone marrow, thereby reducing platelet production.[3]

Pregnancy is associated with significant immunological adaptations that can influence autoimmune diseases. Although many women with ITP remain stable throughout gestation, others experience worsening thrombocytopenia requiring medical treatment. Severe thrombocytopenia may result in spontaneous mucosal bleeding, ecchymosis, petechiae, gastrointestinal haemorrhage, intracranial bleeding, and obstetric haemorrhage.[4]

Steroid-resistant ITP refers to failure to achieve an adequate platelet response despite corticosteroid therapy. Such patients frequently require second-line therapies including IVIG, Rituximab, thrombopoietin receptor agonists, or splenectomy.[5]

Anatomy and Physiology of the Structures Involved

Bone Marrow and Platelet Production

Platelets are produced in the red bone marrow by megakaryocytes under the influence of thrombopoietin. Normally, platelet counts range between 150,000 and 450,000 cells/µL. Platelets circulate for approximately 7–10 days and play a critical role in haemostasis and vascular repair.[6]

In this patient, platelet production was unable to compensate for accelerated immune-mediated platelet destruction, resulting in severe thrombocytopenia.

Spleen

The spleen functions as a major component of the reticuloendothelial system and is responsible for removing aged blood cells and pathogens from circulation. In ITP, antibody-coated platelets are destroyed predominantly within the spleen, making it the principal site of platelet clearance.[7]

Placenta

The placenta serves as the interface between maternal and fetal circulation, facilitating oxygen and nutrient exchange. Adequate placental perfusion is essential for normal fetal growth and development.[8]

In this case, placental insufficiency likely contributed to fetal growth restriction and oligohydramnios.

Amniotic Fluid

Amniotic fluid protects the fetus from trauma, promotes musculoskeletal development, facilitates fetal movement, and contributes to lung maturation. Reduced amniotic fluid volume, known as oligohydramnios, is associated with placental dysfunction and adverse perinatal outcomes.[9]

Fetal Brain (Caudothalamic Groove)

The fetal neurosensorial demonstrated cystic changes within the left caudothalamic groove. This region is a common site for germinal matrix haemorrhage in premature infants because of its rich vascular network and structural fragility.[10]

Normal Physiology versus Abnormality in This Case

StructureNormal Anatomy (Textbook Image)Abnormality in Patient (Clinical Image)
Bone MarrowNormal bone marrow contains healthy megakaryocytes producing adequate platelets (150,000–450,000/µL).In ITP, autoantibodies destroy circulating platelets faster than the bone marrow can replace them, resulting in severe thrombocytopenia.
PlateletsNormal platelets circulate for 7–10 days and maintain hemostasis by forming blood clots at sites of vascular injury.Platelet count remained critically low despite steroid therapy, indicating steroid-resistant immune thrombocytopenic purpura.
SpleenNormal spleen removes aged blood cells and participates in immune surveillance.Antibody-coated platelets were excessively destroyed in the spleen, causing persistent thrombocytopenia.
PlacentaNormal placenta provides adequate oxygen and nutrient exchange between mother and fetus, supporting normal fetal growth.Placental insufficiency resulted in fetal growth restriction (FGR), evidenced by poor fetal growth parameters.
Amniotic FluidNormal amniotic fluid protects the fetus, facilitates movement, and promotes lung development.Oligohydramnios was present, indicating reduced amniotic fluid volume and increased fetal risk.
Fetal Brain (Caudothalamic Groove)Normal fetal neurosonogram demonstrates intact ventricular structures without hemorrhage or cystic lesions.Neurosonogram showed cystic changes in the left caudothalamic groove, suggestive of previous germinal matrix hemorrhage.

Case Presentation

A 24-year-old female, gravida 3 abortus 2, at 31 weeks and 4 days of gestation presented with complaints of generalized weakness. She was a known case of immune thrombocytopenic purpura and had previously been treated at another tertiary care centre where corticosteroid therapy failed to improve platelet counts. She was therefore referred for advanced management.

On admission, she was hemodynamically stable. Her pulse rate was 137 beats/minute, blood pressure was 109/80 mmHg, respiratory rate was 18 breaths/minute, temperature was 98.6°F, and oxygen saturation was 98% on room air. Systemic examination was unremarkable.

Routine laboratory investigations demonstrated severe thrombocytopenia. Obstetric evaluation revealed fetal growth restriction with oligohydramnios. The patient was admitted to the intensive care unit for close maternal and fetal monitoring.

Neuro sonography demonstrated cystic changes within the left caudothalamic groove measuring 0.5 × 0.2 cm, likely representing sequelae of previous germinal matrix haemorrhage. Mild periventricular flare was noted without evidence of active intracranial bleeding.

Management

The patient was initially treated with intravenous methylprednisolone 125 mg daily. Despite therapy, serial platelet counts remained critically low, confirming steroid-resistant ITP. Following multidisciplinary discussions and family counselling, intravenous immunoglobulin therapy was initiated. A cumulative IVIG dose of 2 g/kg was administered over five days. Subsequent platelet counts demonstrated significant improvement. Continuous fetal surveillance was performed using serial growth scans and fetal heart monitoring. Because of persistent fetal growth restriction and oligohydramnios, the obstetric team elected to proceed with delivery.

On 15 April 2026, lower segment caesarean section was performed under spinal anaesthesia. A live female infant weighing 1.5 kg was delivered at 12:01 PM. The newborn was admitted to the neonatal intensive care unit because of prematurity and low birth weight.

Postpartum Course

Initially, the postoperative course was uneventful. The patient received intravenous antibiotics, analgesics, corticosteroids, proton pump inhibitors, and supportive therapy.

However, platelet counts subsequently demonstrated a concerning downward trend:

Post-IVIG platelet count: 240,000/µL

  • 17/04/2026: 80,000/µL
  • 18/04/2026: 46,000/µL
  • 19/04/2026: 30,000/µL

Because of the significant decline in platelet counts, Romiplostim therapy was initiated. Despite treatment, thrombocytopenia persisted, necessitating administration of Rituximab 500 mg under close monitoring.

Discussion

This case demonstrates the challenges associated with managing steroid-resistant ITP during pregnancy. The patient’s failure to respond adequately to corticosteroids reflects persistent autoimmune platelet destruction. IVIG therapy successfully increased platelet counts and allowed safe delivery; however, the postpartum decline highlights the chronic and relapsing nature of refractory ITP.[11]. The coexistence of fetal growth restriction and oligohydramnios significantly complicated management. Placental insufficiency likely contributed to impaired fetal growth and reduced amniotic fluid volume. Timely fetal monitoring and multidisciplinary decision-making allowed safe intervention before fetal compromise occurred.[12]

Romiplostim acts by stimulating thrombopoietin receptors on megakaryocytes, thereby enhancing platelet production. Rituximab reduces autoantibody formation through depletion of CD20-positive B lymphocytes and is increasingly used in refractory ITP.[13]

Image Source: AI generated

The favourable maternal and neonatal outcome despite severe thrombocytopenia underscores the importance of coordinated multidisciplinary care.

Nursing Management and Implications

Nurses played a vital role throughout the patient’s hospitalization. Intensive monitoring of platelet trends, bleeding manifestations, vital signs, and fetal well-being was essential. During IVIG administration, nurses closely observed for infusion reactions and ensured adherence to treatment protocols. Perioperative nursing care focused on haemorrhage prevention, pain management, wound care, and early mobilization. Continuous patient and family education regarding disease progression, treatment options, and neonatal care contributed significantly to overall management. The case emphasizes the importance of specialized nursing competencies in high-risk obstetric and haematological care settings.

Conclusion

Steroid-resistant immune thrombocytopenic purpura during pregnancy remains a challenging condition requiring individualized treatment and multidisciplinary management. This case highlights the successful use of IVIG therapy to achieve temporary haematological stabilization and facilitate safe caesarean delivery in a patient with severe thrombocytopenia complicated by fetal growth restriction and oligohydramnios. The postpartum recurrence of thrombocytopenia requiring Romiplostim and Rituximab further illustrates the chronic nature of refractory ITP. Early diagnosis, continuous monitoring, timely intervention, and coordinated multidisciplinary care remain crucial for optimizing maternal and neonatal outcomes.

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