High rate of spontaneous inhibitor clearance during the long term observation study of a single cohort of 524 haemophilia A patients not undergoing immunotolerance

Table 1 Distribution of patients with and without inhibitor development, high and low responders, transient, slowly resolving and permanent, dependent on the severity of the disease

	Severe	Moderate	Mild	Total	High responders	Low responders	OR
	n = 434	n = 60	n = 30	n = 524	n = 79	n = 101	(*)
Without inhibitor	266	50	28	344
With inhibitor	168	10	2	180
Transient	64 (38%)	5 (50%)	1 (50%)	70 (39%)	8 (10%)	62 (61%)	-
Slowly resolving	44 (26%)	2 (20%)	1 (50%)	47 (26%)	15 (19%)	32 (32%)	3.6
Permanent	60 (36%)	3 (30%)	0	63 (35%)	56 (71%)	7 (7%)	62

The condition of HR at the onset confers the highest risk of persistent inhibitor (56 out of 79, 71%) while only a minority of the patients become persistent when the onset is as LR (7 out of 101, 7%).
(*)The OR represents the risk of having a permanent or slow resolving inhibitor for those being HR as compared to those being LR.

ISSN: 1756-8722