Spontaneous remission of 2-chlorodeoxyadenosine (2-CdA)-related secondary myelodysplastic syndrome in a patient with ref
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ORIGINAL ARTICLE
Spontaneous remission of 2-chlorodeoxyadenosine (2-CdA)-related secondary myelodysplastic syndrome in a patient with refractory Langerhans cell histiocytosis Daisuke Suzuki • Ryoji Kobayashi • Hirozumi Sano • Kenji Kishimoto • Kazue Yasuda • Masanori Nakanishi Tetsuro Nagashima • Kunihiko Kobayashi
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Received: 26 July 2012 / Revised: 10 April 2013 / Accepted: 15 April 2013 / Published online: 25 April 2013 Ó The Japanese Society of Hematology 2013
Abstract Langerhans cell histiocytosis (LCH) is sometimes resistant to conventional chemotherapies, and treatment with 2-chlorodeoxyadenosine (2-CdA) is gaining importance as a salvage treatment for refractory or recurrent LCH. Secondary malignancies such as acute myelogenous leukemia and myelodysplastic syndrome (MDS) due to 2-CdA have recently been reported. However, there have been no reports to date of cases of 2-CdA-related secondary MDS in which spontaneous remission was achieved. Here, we report the case of a 1-year-old boy with an occipital tumor who was diagnosed with LCH by biopsy and underwent chemotherapy. However, the disease relapsed and became refractory to chemotherapy. He received 2-CdA treatment, which was effective. However 6 months after the start of treatment, he developed MDS with chromosomal abnormality of 7q-. After 1-year observation without any intervention, his hematological findings had returned to normal, and the chromosomal abnormality had also disappeared. To our knowledge, this is the first report of 2-CdA-related MDS with spontaneous hematological remission. Keyword Secondary myelodysplastic syndrome Spontaneous remission 2-Chlorodeoxyadenosine Langerhans cell histiocytosis
D. Suzuki (&) R. Kobayashi H. Sano K. Kishimoto K. Yasuda K. Kobayashi Department of Pediatrics, Sapporo Hokuyu Hospital, Higashi-Sapporo 6-6, Shiroishiku, Sapporo 003-0006, Japan e-mail: [email protected] M. Nakanishi T. Nagashima Department of Pediatrics, Kushiro Red Cross Hospital, Kushiro 085-0032, Japan
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Introduction The efficacy of 2-chlorodeoxyadenosine (2-CdA) for refractory and recurrent Langerhans cell histiocytosis (LCH) has been recognized since the 1990s [1–3], though, secondary malignancies due to 2-CdA, such as myelodysplastic syndrome (MDS) and acute myelogenous leukemia (AML), have been increasingly reported [4–9]. MDS shows a wide range of variation and its prognosis is generally poor. Hematopoietic stem cell transplantation (HSCT) is considered to be the only curative treatment for MDS. Spontaneous remission of MDS has seldom been reported; most of which in children or young people have been characterized by the presence of a 7th chromosomal abnormality [10–13]. A case of spontaneous remission of 2-CdA-related secondary MDS is reported here.
Case report A 1-year-old boy presented with an occipital tumor that had been noticed when he had a bruise on the same site, and he was referred to our hospital. Laboratory studies revealed a total leukocyte count of 8.2 9 109/L with no abnormal cells, red blood cells o
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