Surgical procedures in patients with Glanzmann's thrombasthenia: case series and literature review.
Glanzmann's thrombasthenia is a rare platelet function disorder with an autosomal recessive pattern of inheritance. Achieving haemostasis in such patients who undergo surgical procedures always poses a significant challenge. Herein we report six cases of Glanzmann's thrombasthenia, who underwent nine surgeries under the cover of platelet-rich concentrates with or without recombinant activated factor VII . Of these, five were major surgeries such as thyroidectomy, laparotomy, Hartmann's procedure, reversal of Hartmann's procedure and a complete dental extraction. All five procedures were successfully done without any major bleeding. The major cost incurred in these procedures is due to the large number of blood products used and recombinant activated factor VII if used.
aDepartment of Haematology bDepartment of Immunohaematology and Transfusion Medicine, Christian Medical College, Vellore, Tamil Nadu, India.
Abhijeet
A
Ganapule
Punit
P
Jain
Fouzia N
FN
Abubacker
Anu
A
Korula
Aby
A
Abraham
Joy
J
Mammen
Biju
B
George
Vikram
V
Mathews
Alok
A
Srivastava
Auro
A
Viswabandya
D000293:Adolescent; D000328:Adult; D002648:Child; D015942:Factor VIIa / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D013915:Thrombasthenia / Q000188:drug therapy / Q000601:surgery*; D055815:Young Adult
D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa