Distinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia

Induction therapy with all-trans retinoic acid (ATRA) is effective for acute promyelocytic leukemia (APL). ATRA induces neutrophil differentiation and its associated side effects. The differentiation syndrome is the most characterized ATRA-induced adverse effect. Sweet’s syndrome, also known as neut...

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Autores principales:	Osamu Imataki, Shunsuke Yoshida, Tomohiro Kaji, Jun-ichiro Kida, Hiroyuki Kubo, Makiko Uemura, Haruyuki Fujita, Norimitsu Kadowaki
Formato:	article
Lenguaje:	EN
Publicado:	Karger Publishers 2021
Materias:	all-trans retinoic acid pml-rarα differentiation syndrome sweet syndrome neutrophil infiltration scrotal ulceration acute promyelocytic leukemia Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/18cacf218ef143fb971bd916cf1a0bba
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spelling	oai:doaj.org-article:18cacf218ef143fb971bd916cf1a0bba2021-11-18T11:08:46ZDistinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia1662-657510.1159/000516798https://doaj.org/article/18cacf218ef143fb971bd916cf1a0bba2021-10-01T00:00:00Zhttps://www.karger.com/Article/FullText/516798https://doaj.org/toc/1662-6575Induction therapy with all-trans retinoic acid (ATRA) is effective for acute promyelocytic leukemia (APL). ATRA induces neutrophil differentiation and its associated side effects. The differentiation syndrome is the most characterized ATRA-induced adverse effect. Sweet’s syndrome, also known as neutrophilic dermatosis, is another form of ATRA-associated disease characterized by neutrophil infiltrating erythema that develops with fever. This is a case of a 34-year-old Japanese man diagnosed with APL. At the onset, the patient did not have skin involvement of APL cells. He was treated with ATRA and induction chemotherapy with idarubicin and cytarabine. Scrotal skin rash occurred at day 14, which developed into scrotal ulceration up to day 28 even after eliminating APL cells in his peripheral blood. Sweet’s syndrome is a pathological diagnosis of scrotal skin ulceration representing neutrophil infiltration. The infiltrating neutrophils showed PML-RARα rearrangement. The patient was diagnosed with ATRA-associated Sweet’s syndrome with skin ulcer. His cutaneous lesion did not respond to intravenous prednisolone therapy; thereby, ATRA was discontinued. After the cessation of ATRA, the skin lesion improved in the next week. We confirmed he achieved a complete response after induction chemotherapy. In our observation, ATRA-associated Sweet’s syndrome is characterized by the following clinical manifestations: preferable occurrence in the scrota, tend to progress into skin ulcer, and pathogenicity associated with PML-RARα-positive matured neutrophils. The etiology, pathogenesis, and risk factors of ATRA-associated scrotal ulceration were discussed in the literature review.Osamu ImatakiShunsuke YoshidaTomohiro KajiJun-ichiro KidaHiroyuki KuboMakiko UemuraHaruyuki FujitaNorimitsu KadowakiKarger Publishersarticleall-trans retinoic acidpml-rarαdifferentiation syndromesweet syndromeneutrophil infiltrationscrotal ulcerationacute promyelocytic leukemiaNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCase Reports in Oncology, Vol 14, Iss 3, Pp 1466-1474 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	all-trans retinoic acid pml-rarα differentiation syndrome sweet syndrome neutrophil infiltration scrotal ulceration acute promyelocytic leukemia Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	all-trans retinoic acid pml-rarα differentiation syndrome sweet syndrome neutrophil infiltration scrotal ulceration acute promyelocytic leukemia Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Osamu Imataki Shunsuke Yoshida Tomohiro Kaji Jun-ichiro Kida Hiroyuki Kubo Makiko Uemura Haruyuki Fujita Norimitsu Kadowaki Distinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia
description	Induction therapy with all-trans retinoic acid (ATRA) is effective for acute promyelocytic leukemia (APL). ATRA induces neutrophil differentiation and its associated side effects. The differentiation syndrome is the most characterized ATRA-induced adverse effect. Sweet’s syndrome, also known as neutrophilic dermatosis, is another form of ATRA-associated disease characterized by neutrophil infiltrating erythema that develops with fever. This is a case of a 34-year-old Japanese man diagnosed with APL. At the onset, the patient did not have skin involvement of APL cells. He was treated with ATRA and induction chemotherapy with idarubicin and cytarabine. Scrotal skin rash occurred at day 14, which developed into scrotal ulceration up to day 28 even after eliminating APL cells in his peripheral blood. Sweet’s syndrome is a pathological diagnosis of scrotal skin ulceration representing neutrophil infiltration. The infiltrating neutrophils showed PML-RARα rearrangement. The patient was diagnosed with ATRA-associated Sweet’s syndrome with skin ulcer. His cutaneous lesion did not respond to intravenous prednisolone therapy; thereby, ATRA was discontinued. After the cessation of ATRA, the skin lesion improved in the next week. We confirmed he achieved a complete response after induction chemotherapy. In our observation, ATRA-associated Sweet’s syndrome is characterized by the following clinical manifestations: preferable occurrence in the scrota, tend to progress into skin ulcer, and pathogenicity associated with PML-RARα-positive matured neutrophils. The etiology, pathogenesis, and risk factors of ATRA-associated scrotal ulceration were discussed in the literature review.
format	article
author	Osamu Imataki Shunsuke Yoshida Tomohiro Kaji Jun-ichiro Kida Hiroyuki Kubo Makiko Uemura Haruyuki Fujita Norimitsu Kadowaki
author_facet	Osamu Imataki Shunsuke Yoshida Tomohiro Kaji Jun-ichiro Kida Hiroyuki Kubo Makiko Uemura Haruyuki Fujita Norimitsu Kadowaki
author_sort	Osamu Imataki
title	Distinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia
title_short	Distinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia
title_full	Distinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia
title_fullStr	Distinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia
title_full_unstemmed	Distinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia
title_sort	distinct characteristics of sweet’s syndrome of the scrotum caused by all-trans retinoic acid in a patient with acute promyelocytic leukemia
publisher	Karger Publishers
publishDate	2021
url	https://doaj.org/article/18cacf218ef143fb971bd916cf1a0bba
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Distinct Characteristics of Sweet’s Syndrome of the Scrotum Caused by All-trans Retinoic Acid in a Patient with Acute Promyelocytic Leukemia

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