Case Report

Deep Vein Thrombosis, Raynaud's Phenomenon, and Prinzmetal Angina in a Patient with Glanzmann Thrombasthenia

Table 1

Patients with DVT described in the literature and this study.

PatientBleedingPlatelet AggregResidual IIb 3 MutationRefComments

N° 1—elderly maleMildAbsent50% 3-Ser752Pro[12]Severe proximal DVT and pulmonary embolism after long airflight—treated with LMWH
N° 2—adult maleGI bleedingAbsentAbsent/lowNot known[9]Recurrent (3X) proximal DVT in same leg. Factor V Leiden. Treated with LMWH
N° 3—adult maleMildAbsentAbsentNot known[10]Recurrent DVT & possible pulmonary embolism. Factor V Leiden. Treated with heparin and warfarin
N° 4—2-yr-old girlRepeated epistaxisAbsentMuch reducedNot known[11]Proximal DVT after platelet transfusions (femoral catheter) and rFVIIa. No anticoagulation
N° 5—adult womanModerateAbsentTrace amounts 3-Cys457Tyr[1] (patient 11)
[22]
Single episode treated with heparin
N° 6—adult womanSevere when childAbsentAbsent IIb-Glu324Lys[1] (patient 9)
and this study
Single episode without treatment
Raynaud’s phenomenon and Prinzmetal angina

Patients were included on the basis of a detailed literature report and of the availability of clinical and biological data. Mutations are given when known. LMWH: low molecular weight heparin. rFVIIa: recombinant factor VIIa. Aggreg: aggregation.