Author:
Bencharef H.,Lamchahab M.,Dassouli D.,Sraidi S.,Guennoun B.,Hda N.,Oukkache B.,Quessar A.
Abstract
Abstract
Background
Xeroderma pigmentosum is a rare inherited disease characterized by extreme hypersensitivity to ultraviolet rays and predisposing to cutaneous malignancies that can appear in childhood. These manifestations are often associated with ocular lesions and sometimes with neurological disorders. The association of xeroderma pigmentosum with internal neoplasms such as acute myeloblastic leukemia is not reported with great frequency, which confirms the rarity of this occurrence.
Case report
A 26-year-old Moroccan women, xeroderma pigmentosum patient, was diagnosed with acute myeloblastic leukemia with a complex karyotype. Due to the adverse risk of the xeroderma pigmentosum association with acute myeloblastic leukemia and the profile of acute myeloblastic leukemia with complex karyotype and monosomy 7, which constitute factors of poor prognosis, as well as the absence of studies conceding the tolerance of the chemotherapy by patients suffering from xeroderma pigmentosum, our patient was put under low-dose cytarabine protocol with granulocyte colony-stimulating factor. Unfortunately, she died on the tenth day of chemotherapy by acute pulmonary edema of cardiogenic pace complicated by tamponade.
Conclusion
According to reports, it is the second case showing association of xeroderma pigmentosum with acute myeloblastic leukemia. The management of these patients remains a challenge. Studies focusing on xeroderma pigmentosum patients developing hematological malignancies are necessary to better understand the most appropriate strategies and precautions for this specific case.
Publisher
Springer Science and Business Media LLC
Reference23 articles.
1. Zghala M, Fazaaa B, Abdelhakb S, Mokni M. Xeroderma pigmentosum. Ann Dermatol Venereol. 2018;145(11):706–22.
2. Bilodi AK, Gangadhar MR. Xeroderma pigmentosa. A rare clinical entity. Indian J Med Case Rep. 2013;2:21–4.
3. Kraemer KH, Lee MM, Scotto J. Xeroderma pigmentosum. Cutaneous, ocular, and neurologic abnormalities in 830 published cases. Arch Dermatol. 1987;123:241–50.
4. Feller L, Wood NH, Motswaledi MH, Khammissa RA, Meyer M, Lemmer J. Xeroderma pigmentosum: a case report and review of the literature. J Prev Med Hyg. 2010;51:87–91.
5. Tamhankar PM, Iyer SV, Ravindran S, Gupta N, Kabra M, Nayak C, et al. Clinical profile and mutation analysis of xeroderma pigmentosum in Indian patients. Indian J Dermatol Venereol Leprol. 2015;81:16–22.
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