28 years old Vanitha W/o.Vijayakumar with Glanzmann Thrombasthenia in pregnancy Booked our hospital Fathima maternity Home with BMI of 30. She was diagnosed with type 1 Glanzmann Thrombasthenia at the age of 9 years of CMC Vellore, She was a Carrier of a Glycoprotein III misense Mutation which resulted in a life long absence of the IIB – IIIa platelet Glycoprotein on the platelet surface. This deficiency confers abnormal primary haemostasios and a Bleeding phenotype, Her partner was non – Consanguinous and as such paternal Genotyping was not performed. During pregnancy, she developed Haematuriya and Bleeding per vagina on off frequently Hospitalized and she received Huge platelets infusion. Multiple Blood Tranfusion done. The patient was regularly reviewed by our medical team.
The maternal platelet count was maintained Throughout pregnancy. Multi disciplinary meeting between the Haematology- Dr.Ancy Abraham CMC Vellore. Dr.Rath, Trichy, Obstetrician Dr. Rohaiyaah (Fathima Maternity Home) Anaethetist Dr.Srinivasan was arranged for 37 weeks. It was felt that instrumental delivery should he avoided due to the rise of intrcraial haemorrhage and the mother was counselled relarding this risk. The safest mode of delivery for the Baby was deemed to be caesarean section due to the combination of oligohydrominos, Bleeding plv with uneffaced cervix and potential risk of neonatal alloimmune thromhocytopenia A clear plan was made for Haemostasis, totally we given 30 units platlets and Blood we done Caesarean for her on 06.06.2019 delivered Boy Baby. Careful postpartum monitoring don in our Hospital Baby hand over to Dr. Senthil, Thangavelu neronatologist for further management, Mother & baby doing well.
Glanzmann Thrombasthenia is a an autosomal recessive Haemorhagic disorder, Prevalence of disease 1 in 10,00,000 and predominated among certain ethnic groups such as southern Indians as with our patient. It is caused by a deficiency (or) dysfunction of Glycoprotein IIb-IIIa receptors on platets which are required for platlets aggregation.
The absence of platlets Gp IIb-IIIa demonstrated using monoclonal antihodies and flow cytometry. Pregnancy in indiiduals with Glanzmann carrier multiple risks . The risks of antepartum peripartum and post partum Harmorrhage are all increased managing in preganancy individuals with poses multiple challenge for both the mother and fetus. Management must be multi disciplinary between obstetries, Haematology and anaesthetic tams and Preconception Counselling is essential regarding High risk of mother & Baby outcome. Written consent for the publicaton was obrained from the patient.