Abstract

Glanzmann Thrombasthenia is a rare autosomal recessive disorder characterized by recurrent episodes of spontaneous or traumatic bleeding starting from the first decade of life. Affection of the gene located in long arm of chromosome-17 prevents platelet agglutination but the morphology and count remains normal. It is common in female, certain races and offspring in consanguineous marriage. As it may be life threatening in certain cases, should be considered in bleeding disorder including menorrhagia with normal platelet and coagulation profile but high bleeding time and defective clot retraction. Anti fibrinolytics(Tranexamic acid), blood and platelet transfusion are the treatment in bleeding episodes. Combined oral contraceptive pills help in menorrhagia. We report a case of Glanzmann Thrombasthenia variant type with history of recurrent bleeding from the age of six, reported to the gynecology outpatient department of Mamata Medical College, Khammam, Telangana state, India at the age of 18 years with menorrhagia and severe anemia. She was diagnosed at the age of nine years by platelet Aggregometry and flow cytometry in addition to tests for other causes of abnormal bleeding. As reported with severe anemia she was treated with blood transfusion, iron and folic acid, Vitamin C and combined oral contraceptive pills. Though a rare and life threatening bleeding disorder, prognosis is good with early diagnosis and adequate management.