Platelet storage pool disorder: multidisciplinary planning in pregnancy

Area of Science:

• Hematology
• Obstetrics
• Genetics

Background:

• Platelet storage pool disorder (PSPD) encompasses rare conditions affecting platelet granule storage or secretion, leading to impaired platelet aggregation and activation.
• PSPD is a functional platelet disorder, typically presenting with normal platelet counts but abnormal function.
• Both genetic and acquired forms of PSPD exist, with limited documented cases in pregnant individuals.

Purpose of the Study:

• To discuss the basic science, inheritance, symptoms, and management of PSPD.
• To outline specific antenatal, intrapartum, and postnatal management strategies for pregnant women with PSPD.
• To emphasize the necessity of a multidisciplinary approach for optimal patient care.

Main Methods:

• Case report of a primigravida woman diagnosed with PSPD during pregnancy.
• Review of literature concerning PSPD, its inheritance, and clinical manifestations.
• Discussion of management strategies, including pharmacological interventions and delivery considerations.

Main Results:

• The patient was diagnosed with PSPD during pregnancy and underwent a cesarean delivery for breech presentation.
• Antenatal, intrapartum, and postnatal management plans were developed, anticipating potential complications.
• The case underscores the challenges in managing PSPD during pregnancy due to its rarity.

Conclusions:

• Effective management of PSPD in pregnancy requires a coordinated multidisciplinary team, including obstetricians, anesthesiologists, and hematologists.
• Anticipation and preparedness for postpartum hemorrhage are crucial, involving medications like DDAVP and tranexamic acid, oxytocics, and prompt blood product availability.
• Careful intrapartum management planning is essential for ensuring the safety of both mother and child.