Glanzmann’s thrombasthenia diagnosed following knee arthroscopy
John Zvijac, Sharhabil Ammus, Fernando Aran and Gary Kiebzak describe a case where treatment of an unremarkable knee injury led to diagnosis of a rare bleeding disorder
A 41-year-old man with an unremarkable medical history presented with a painful knee after a sports injury. He was diagnosed with a medial meniscal tear. Symptoms did not abate after six months of physical therapy, and he underwent arthroscopic partial medial meniscectomy. A week after beginning physical therapy he experienced a knee effusion, decreased ROM, and inability to flex his quadriceps. His knee was aspirated. Blood tests were ordered and his complete blood count, liver functions tests and INR/PTT were normal. The patient had recurrent effusions requiring three additional joint aspirations. Ten weeks after the initial surgery, he underwent a second arthroscopy, during which a haematoma was removed and a synovectomy performed. The patient continued bleeding from the incisions after portals were sutured, and he was admitted to the hospital. A haematologist was consulted and comprehensive platelet aggregation testing revealed previously undiagnosed Glanzmann’s thrombasthenia. The patient began treatment with platelet infusions and desmopressin and progressed to a full recovery. Clinical suspicion for surgical patients with unusual repetitive postoperative bleeding should include previously undetected rare bleeding disorders even in adults.
Minor haemarthrosis (bleeding in the joint space) and pain after arthroscopic knee surgery are not uncommon and are usually self-limiting as healing progresses. Occasionally, various events such as failure of the surgical repair, infection, new traumatic injury, unrecognised hypertension, use of anticoagulation medications, or liver disease affecting synthesis of clotting factors may result in more serious and repetitive haemarthrosis. Typically, however, the surgeon does not suspect rare clotting disorders in adults with normal results of blood tests, as such inherited or acquired disorders are usually detected early in life. In this report, we describe a case of postoperative haemarthrosis after arthroscopic meniscectomy in an ostensibly healthy adult man who was later diagnosed with Glanzmann’s thrombasthenia.
Case presentation
A 41-year-old man with an unremarkable medical history presented to the clinic with right knee pain, severity 3/10, piercing and sharp in quality, which occurred after recreational sports activity. He was diagnosed with a medial meniscal tear confirmed by MRI. Six months of physical therapy did not relieve the symptoms. The patient underwent diagnostic arthroscopy, partial medial meniscectomy, chondroplasty of the trochlea, and partial synovectomy without intraoperative complication (bleeding was controlled by tourniquet). He started a home exercise programme and formal strengthening therapy began on postoperative day 10. Then, in the absence of any particular traumatic event, he experienced a knee effusion, decreased range of motion and inability to flex his quadriceps muscle on postoperative day 17.
Upon physical exam in the clinic, mild effusion of the right knee was noted with no ecchymosis, and laxity tests and meniscal exams were negative. Range of motion was 2 degrees of extension and 110 degrees of flexion. Knee aspiration was done with 62 ml of serosanguinous fluid withdrawn. The patient was taking aspirin and told to discontinue its use. He was instructed to stop physical therapy, rest his knee, use ice for 20 minutes 3–4 times per day, and wear a compression wrap until his next office visit in 2 weeks. Subsequent blood test results showed normal complete blood count, liver function tests, and INR/PTT.
Within the next few weeks, the patient had recurrent effusions with related symptoms requiring three additional joint aspirations. Ten weeks after the initial surgery, the patient underwent a second arthroscopy, during which a haematoma was removed and a synovectomy performed. The procedure was routine with no intraoperative complications. However, after the procedure the patient continued bleeding from the sutured incisions and was admitted to the hospital for observation. A haematologist was consulted. The patient denied previous bleeding problems. Platelet aggregation tests revealed decreased platelet aggregation and secretion with arachidonic acid, collagen, adenosine diphosphate (ADP), and thrombin. In contrast, platelet aggregation was normal in the presence of ristocetin. These results were consistent with the diagnosis of Glanzmann’s thrombasthenia.
After the definitive diagnosis was made, the patient began treatment with platelet infusions and desmopressin and progressed to a full recovery.
Discussion
Glanzmann’s thrombasthenia, first described in 1918 as a hereditary haemorrhagic thrombasthenia, is a rare autosomal recessive bleeding disorder with an estimated incidence of 1 in 1,000,000 [1–3]. It is characterised by normal platelet count but lack of normal platelet aggregation [3]. The platelet function disorder is caused by qualitative and/or quantitative defects in the platelet glycoprotein IIb/IIIa complex (an integrin coded by the ITGA2B and ITGB3 genes and which by binding fibrinogen and other adhesive proteins joins platelets together in