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Disease severity scoring system for disorders associated with WAS mutations

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 – – – (+) + ++ –/(+)/+/++
ImmunodeficiencyΔ –/(+) – –/(+) (+) + +/++ –/(+)/+/++
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.
Graphic 69089 Version 10.0

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