Recognition and management of platelet-refractory bleeding in patients with Glanzmann’s thrombasthenia and other severe platelet function disorders

Figures & data

Table 1 Adjudicator evaluation of GTR rFVIIa efficacy data

Adjudicator evaluation of efficacy – bleeding episodes^Citation16
Treatment group	Number of patientsa	Number of episodes	Success n (%)	Failure n (%)	Insufficient data n (%)	No consensus n (%)
All rFVIIab	92	266	251 (94.4)	4 (1.5)	6 (2.3)	5 (1.9)
By treatment regimen
rFVIIa only	44	109	101 (92.7)	2 (1.8)	4 (3.7)	2 (1.8)
rFVIIa ± platelets ± other hemostatic agents	69	157	150 (95.5)	2 (1.3)	2 (1.3)	3 (1.9)
By antibody/refractory group
Refractoriness ± platelet-specific antibodiesc,d	31	79	75 (94.9)	2 (2.5)	2 (2.5)	0 (0.0)
Platelet-specific antibodiesc,d	8	10	10 (100.0)	0 (0.0)	0 (0.0)	NA
Neither or unknownd,e	57	177	166 (93.8)	2 (1.1)	4 (2.3)	5 (2.8)

Adjudicator evaluation of efficacy – surgical procedures^Citation16
Treatment group	Number of patientsa	Number of procedures	Success n (%)	Insufficient dataf n (%)
All rFVIIab	77	160	159 (99.4)	1 (0.6)
By treatment regimen
rFVIIa only	35	66	65 (98.5)	1 (1.5)
rFVIIa ± platelets ± other hemostatic agents	57	94	94 (100.0)	0 (0.0)
By antibody/refractory group
Refractoriness ± platelet-specific antibodiesc,g	33	70	69 (98.6)	1 (1.4)
Platelet-specific antibodiesc,g	11	24	24 (100.0)	0 (0.0)
Neither or unknowne,g	36	66	66 (100.0)	0 (0.0)

Notes:

a Patient numbers are not additive. Patients may have episodes with different treatment regimens and have more than one antibody/refractory status.

b All treatment regimens that included treatment with rFVIIa.

c Includes GPIIb/IIIa, HLA, and unspecified platelet-specific antibodies.

d Treatment was rFVIIa only for 26/79 episodes with refractoriness with or without antibodies, 2/10 episodes with platelet-specific antibodies only, and 81/177 episodes with neither or unknown. The remainder received rFVIIa with platelets and/or antifibrinolytic agents.

e Assumes no platelet-specific antibodies or refractoriness reported or antibody and refractory status unknown.

f No reports of failure or lack of consensus were reported.

g Treatment was rFVIIa only for 22/70 episodes with refractoriness with or without antibodies, 13/24 episodes with platelet-specific antibodies only, and 31/66 episodes with neither or unknown. The remainder received rFVIIa with platelets and/or antifibrinolytic agents.

Abbreviations: GP, glycoprotein; GTR, Glanzmann’s Thrombasthenia Registry; HLA, human leukocyte antigen; NA, not available; rFVIIa, recombinant activated coagulation factor VII.

Figure 1 Platelet refractoriness management algorithm in severe or postsurgical bleeding. ^aIn postsurgical bleeding, causes of bleeding unrelated to hemostasis should also be considered.

NovoSeven RT Coagulation Factor VIIa (Recombinant) room temperature stable [prescribing information]Plainsboro, NJNovo Nordisk2015

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