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- W2043284723 abstract "RATIONALE: Squamous cell and verrucous carcinomas have not been reported with Wiskott Aldrich Syndrome (WAS). METHODS/CASE: AM is a 21 year-old male who was diagnosed with WAS at 2 weeks of age after presenting with petechiae and otitis media. A matched bone marrow transplant donor was unavailable; the patient's only sibling was also affected with WAS. The patient underwent splenectomy at age 7 for bleeding complications. Supportive treatment included prophylactic antibiotics, topical anti-inflammatory medications, and IVIG. Beginning at the age of 12, the patient had recurrent episodes of tinea pedis, unguis incarnatus, bacterial superinfection of affected toes, and warts. At 20 years, the patient developed an ulceration on his right foot that developed into a vascular-type polyp, as well as a large polyp between the 3rd and 4th toes on his left foot that caused pain with ambulation. RESULTS: Pathology of shave biopsies revealed squamous cell carcinoma in situ in the right foot and verrucous carcinoma in the left. CONCLUSIONS: Lymphoreticular malignancies occur in 12 - 18% of patients and are the most common cause of death in patients with WAS who survive early childhood. Two cases of cutaneous lymphoma have been reported in association with WAS, but there are no previously reported cases of cutaneous carcinoma. The development of malignancies in patients with WAS is poorly understood, but may be related to defective immune surveillance associated with immunodeficiency. In the case of AM, chronic immunostimulation of previous recurrent infections at the site of the carcinoma may also have played a role." @default.
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- W2043284723 date "2005-02-01" @default.
- W2043284723 modified "2023-09-27" @default.
- W2043284723 title "A case of Wiskott-Aldrich Syndrome with cutaneous carcinoma" @default.
- W2043284723 doi "https://doi.org/10.1016/j.jaci.2004.12.630" @default.
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