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Disease severity scoring system for disorders associated with WAS mutations

Disease	XLN	iXLT	XLT		Classic WAS
Score	0	<1	1	2	3	4	5
Thrombocytopenia*	–	–/+	+	+	+	+	+
Small platelets	–	+	+	+	+	+	+
Eczema^¶	–	–	–	(+)	+	++	–/(+)/+/++
Immunodeficiency^Δ	–/(+)	–	–/(+)	(+)	+	+/++	–/(+)/+/++
Infections^◊	–/(+)	–	–	(+)	+	+/++	–/(+)/+/++
Autoimmunity and/or malignancy	–	–	–	–	–	–	+
Congenital neutropenia	+	–	–	–	–	–	–
Myelodysplasia^§	–/+	–	–	–	–	–	–

Scoring system:

–: Absent
–/+: Intermittent (thrombocytopenia) or may/may not be present (myelodysplasia)
(+): Mild (eczema, immunodeficiency, infections)
+: Moderate (eczema, immunodeficiency, infections) or finding present (thrombocytopenia, small platelets, autoimmunity, malignancy, congenital neutropenia)
++: Severe

WAS: Wiskott-Aldrich syndrome; XLN: X-linked neutropenia; iXLT: intermittent X-linked thrombocytopenia; XLT: X-linked thrombocytopenia.
* Thrombocytopenia in iXLT is intermittent.
¶ Eczema can be mild and transient, persistent but therapy responsive, or severe and difficult to control.
Δ Immunodeficiency can be absent, mild, or severe combined.
◊ Infections can be infrequent and not resulting in sequelae, recurrent and requiring treatment with antibiotics and possibly intravenous immune globulin prophylaxis, or severe and life threatening.
§ Myelodysplasia may be present in XLN.

Original table modified for this publication. From: Stiehm ER, Ochs HD, Winkelstein JA, Rich E (Eds). Immunologic disorders in infants and children, 5th ed. Philadelphia: Saunders, 2004. Table used with the permission of Elsevier Inc. All rights reserved.

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