Table 1.

Scoring system to grade the severity of clinical manifestations in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia

	iXLT	XLT		WAS
Score	<1	1	2	3	4	5
Thrombocytopenia	−/+	+	+	+	+	+
Small platelets	+	+	+	+	+	+
Eczema	−	−	(+)	+	++	(+)/+/++
Immunodeficiency	−	−/(+)	(+)	+	+	(+)/+
Infections	−	−	(+)	+	+/++	(+)/+/++
Autoimmunity and/or malignancy	−	−	−	−	−	+

iXLT, intermittent X-linked thrombocytopenia

Scoring system: −, absent; (+), mild; +, present; ++, present and severe

Patients who develop a score 5 are defined as 5A if they present autoimmunity, and 5M if they develop malignancies.

Patients may change their score during life-time. In particular, patients with XLT may progress to WAS and may occasionally reach a score of 5.

(Modified from: Ochs et al., 2009).