Abstract Number: PB1193
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Rare Bleeding Disorders
Background: Perioperative management of people with rare bleeding disorders (RBD) continues being a therapeutic challenge for the hematologists.
Aims: Report our experience in perioperative management of people with RBD in our Hemophilia Comprehensive Care Centre (HCCC).
Methods: We reviewed charts of patients with RBD who underwent at least 1 procedure/surgery (excluding exodontics) in our HCCC from September-18 to December-19. Patients with incomplete information, multiple coagulation factor deficits, vitamin K deficiency, autoimmune or liver disease were excluded. Clinical characteristics, type of surgery, perioperative hemostatic treatment and surgical outcome were collected.
Results: We analyzed 62 surgery procedures underwent on 55 patients (16 surgeries in 11 patients with moderate-severe deficiency). Most common deficit was FVII (70.9%). Clinical characteristics of the patients are summarized in Table 1.
Twenty-one patients had surgeries before diagnosis of RBD with only one bleeding complication reported.
We included 15 major and 47 minor surgeries. Three cardiovascular surgeries were undergone in 2 patients with moderate-severe factor deficit. Management of surgeries is shown in Table 2.
Two minor surgeries required no treatment. Replacement therapy without antifibrinolytic agents was prescribed in 4 procedures. Most of the surgeries (90.3%) received tranexamic acid as single agent or associated with factor replacement. Four hemorrhagic complications were reported (3 major procedures in patients with severe deficit). A patient died 5 months after the procedure.
Conclusions: Our perioperative strategy in patients with RBDs achieved good hemostatic results. Adapted use of tranexamic acid to the thrombotic risk in the perisurgical management of patients with no severe factor deficit has been effective and safe. On the other hand, management of patients with severe factor deficiency with replacement therapy +/- tranexamic acid achieved good hemostasis. Three patients had bleeding complications after surgeries requiring factor coagulation products and/or fresh frozen plasma eventually to stop bleeding.
| All | Fibrinogen | FV | FVII | FX | FXI | |
| Patients (%) | 55 | 1 (1,8) | 2 (3,6) | 39 (70,9) | 2 (3,6) | 11 (20) |
| Men (%) / Women (%) | 30 (54,5) / 25 (45,5) | 1 | 1 / 1 | 21 / 18 | 1 / 1 | 6 / 5 |
| Age Median (Range) | 33 (3-88) | 71 | 37 (5-68) | 15 (3-88) | 55 (37-74) | 56 (33-79) |
| Mild nº / Moderate nº / Severe nº | 44 / 7 / 4 | 0 / 0 / 1 | 2 / 0 / 0 | 34 / 5 / 0 | 1 / 1 / 0 | 7 / 1 / 3 |
| Above hemostatic level (%) | 49 (89) | 0 (0) | 2 (100) | 37 (94,9) | 2 (100) | 8 (72,7) |
| Affected relatives Known / Unknown | 2 3 | 1 0 | 1 0 | 7 7 | 0 0 | 1 3 |
| Surgical History (patients) At least 1 procedure / ≥ 2 procedures / Bleeding | 21 /12 / 1 | 1 /0 / 0 | 0 /0 / 0 | 13 / 7 / 0 | 1 / 1 / 1 | 6 / 4 / 0 |
[Table 1: General and clinical characteristics]
| Minor procedure | Major procedure | |||
| Mild Deficiency | Moderate-Severe Deficiency | Mild Deficiency | Moderate-Severe Deficiency | |
| Fibrinogen | 1 (FR) | |||
| FV | 1 (TXA) | 1 (TXA) | ||
| FVII | 25 (TXA) / 1 (FR) / 2 (FR + TXA) / 1 (None) | 1 (TXA) / 1(FR) / 4 (FR + TXA) | 4 (TXA) / 1 (FR + TXA) | 1 (TXA + FR) |
| FX | 1 (None) | 1 (FR + TXA) / 1 (FFP + TXA) | ||
| FXI | 1 (FFP) / 5 (TXA) | 1 (FR) / 1 (FFP + TXA) | 3 (FFP + TXA) | 4 (FFP + TXA) |
[Table 2. Surgical characteristics and treatment / **FR (Factor replacement), TXA (Tranexamic Acid), FFP (Fresh frozen plasma) ]
To cite this abstract in AMA style:
Hernandez Mata CF, Fernández Mosteirín N, Gómez Martínez A, Martín-Consuegra Rámos S, Pinzón Mariño SF, García Ortego A, Moreno Carbonell M, Civeira Marín M, González Gómez E, Rodríguez Lefler C, Lopez Peña A, Calvo Villas JM. Perioperative Management of Patients with Rare Bleeding Disorders in a Hemophilia Comprehensive Care Centre [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/perioperative-management-of-patients-with-rare-bleeding-disorders-in-a-hemophilia-comprehensive-care-centre/. Accessed October 1, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/perioperative-management-of-patients-with-rare-bleeding-disorders-in-a-hemophilia-comprehensive-care-centre/