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Review
. 2023 Jan 1;45(1):e1-e3.
doi: 10.1097/MPH.0000000000002523. Epub 2022 Aug 11.

Autoimmune Myelofibrosis in a 12-Year-old Male With Monosomy 7, Systemic Lupus Erythematous and Lupus Nephritis: A Case Report and Review of the Literature

María G Español  1 , Abdulla M Ehlayel  2 , Minelys M Alicea Marrero  1 , Renee V Gardner  1 , María C Velez  1
Affiliations
Review

Autoimmune Myelofibrosis in a 12-Year-old Male With Monosomy 7, Systemic Lupus Erythematous and Lupus Nephritis: A Case Report and Review of the Literature

María G Español et al. J Pediatr Hematol Oncol. .

Abstract

Complete or partial loss of chromosome 7 is a common and well-known cytogenetic abnormality associated with preleukemic myelodysplasia and myeloid leukemia but not with autoimmune myelofibrosis. Detection of this molecular change represents poor prognosis. When malignant transformation occurs, the condition tends to be chemotherapy-resistant requiring haematopoietic stem cell transplantation (HSCT) to obtain a cure. Disappearance after immunosuppressive therapy has been documented in children with hematological disorders but not in association with cyclophosphamide and systemic lupus erythematous.We present the interesting case of a 12-year-old male with monosomy 7, systemic lupus erythematous, and lupus nephritis with the resolution of the monosomy 7 and autoimmune myelofibrosis after treatment with cyclophosphamide, along with a review of the literature.

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Conflict of interest statement

M.C.V. is a principal investigator for clinical trials sponsored by NovoNordisk and Pfizer, with funds directed to educational funds in the division. The remaining authors declare no conflict of interest.

References

    1. Heerema N, Nachman J, Sather H, et al. Deletion of 7p or monosomy 7 in pediatric acute lymphoblastic leukemia is an adverse prognostic factor: a report from the Children’s Cancer Group. Leukemia. 2004;18:939–947.
    1. Luna-Fineman S, Shannon KM, Lange BJ. Childhood monosomy 7: epidemiology, biology, and mechanistic implications. Blood. 1995;85:1985–1999.
    1. Henrik H, Todd A A, Auvrignon A, et al. Monosomy 7 and deletion 7q in children and adolescents with acute myeloid leukemia: an international retrospective study. Blood. 2007;109:4641–4647.
    1. Masayuki N, Daisuke T, Youichirou T, et al. Pancytopenia presenting with monosomy 7 which disappeared after immunosuppressive therapy. Leukemia Res. 2004;28:315–319.
    1. Mantadakis E, Shannon KM, Singer DA, et al. Transient monosomy 7: a case series in children and review of the literature. Cancer. 1999;85:2655–2661.

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