Abstract
Glanzmann's thrombasthenia (GT) is a rare inherited platelet disorder, which is characterized by a complete lack of platelet aggregation due to a deficiency or abnormality of the membrane glycoprotein IIb/IIIa complex. Anti-GPIIb/IIIa antibodies have also been identified to cause platelet dysfunction in patients with a normal platelet count, but this has only been rarely encountered. The condition is also known as acquired GT. Herein, we describe a patient with acquired GT and a history of Evans' syndrome, who presented with severe bleeding and platelet dysfunction, but with a normal platelet count and GP IIb/IIIa expression.
REFERENCES
1). Bellucci S, Caen J. Molecular basis of Glanzmann's thrombasthenia and current strategies in treatment. Blood Rev. 2002; 16:193–202.
2). Niessner H, Clemetson KJ, Panzer S, Mueller-Eckhardt C, Santoso S, Bettelheim P. Acquired trombas-thenia due to GPIIb/IIIa-specific platelet autoantibodies. Blood. 1986; 68:571–6.
3). Tholouli E, Hay CR, O'Gormon P, Markis M. Acquired Glanzmann's thrombasthenia without thrombocytopenia: a severe acquired autoimmune bleeding disorder. Br J Haematol. 2004; 127:209–13.
4). Lisman T, Moschatsis S, Adelmeijer J, Nieuwenhuis HK, Philip G. Recombinant factor VIIa enhances deposition of platelets with congenital or acquired α IIbβ3 deficiency to endothelial cell matrix and collagen under conditions of flow via tissue factor-independent thrombin generation. Blood. 2003; 101(5):1864–70.
5). Greaves M, Pickering C, Porter NR, Magee JM, Preston FE. Acquired Glanzmann's thrombasthenia. Blood. 1983; 61:209.
6). DiMinno G, Coraggio F, Cerbone AM, et al. A myeloma paraprotein with specificity for platelet glycoprotein IIIa in a patient with a fatal bleeding disorder. J Clin Invest. 1986; 77:157–64.
7). Balduini CL, Ascari E. Acquired autoimmune thrombasthenia: a new bleeding disorder. Haematologica. 1987; 72:387–90.
8). Kubota T, Tanoue K, Murohashi I, et al. Autoantibody against platelet glycoprotein IIb/IIIa in a patient with non-Hodgkin's lymphoma. Thromb Res. 1989; 53:379–86.
9). Meyer M, Kirchmaier CM, Schirmer A, Spangenberg P, Stroehl C, Breddin K. Acquired disorder of platelet function associated with autoantibodies against membrane glycoprotein IIb-IIIa complex-1 glycoprotein analysis. Thomb Haemost. 1991; 65:491–6.
10). Malik U, Dutcher JP, Oleksowicz L. Acquired Glanzmann's thrombasthenia associated with Hodgkin's lymphoma: a case report and review of the literature. Cancer. 1998; 82:1764–8.
11). Macchi L, Nurden P, Marit G, et al. Autoimmune thrombocytopenic purpura (AITP) and acquired thrombasthenia due to autoantibodies to GP IIb-IIIa in a patient with an unusual platelet membrane glycoprotein composition. Am J Haematol. 1998; 57:164–75.