Spontaneous Remission Of 2-chlorodeoxyadenosine (2-cda)-related Secondary Myelodysplastic Syndrome In A Patient With Refractory Langerhans Cell Histiocytosis
Suzuki, D., Kobayashi, R., Sano, H., Kishimoto, K., Yasuda, K., Nakanishi, M., Nagashima, T., & Kobayashi, K. (2013). spontaneous remission of 2-chlorodeoxyadenosine (2-CdA)-related secondary myelodysplastic syndrome in a patient with refractory Langerhans cell histiocytosis. International journal of hematology, 97(6), 782–785. https://doi.org/10.1007/s12185-013-1343-7
View Original Source →Case Details
Disease Location
Skull, left orbit
Personal Characteristics
1-year-old boy
Clinical Characteristics
Presented with an occipital tumor that had been noticed when he had a bruise on the same site. Lactate dehydrogenase was 247 iu/l, magnetic resonance imaging showed an occipital tumor with a bone defect around it. He underwent partial excision of the tumor. Immunohistostaining showed that the tumor cells were positive for membranous cd1a, s100, CD4 and vimentin and negative for CD21, and a diagnosis of lch was therefore made. Chemotherapy was started and latest 1 year. 1 month after completing the treatment, the tumor relapsed inside the left orbital cavity. Chemotherapy was restarted and then dropped because of thrombocytopenia. Bone marrow examination lead to the diagnosis of mds. Fish showed chromosomal abnormality of del(7q) [3/20] and 1.0 % of 7q31 deletion. Hematopoietic stem cell transplantation was planned but never started because of remission.
Remission Characteristics
Thrombocytopenia gradually improved during the preparation for hsct and then g-banding became normal and only 4.0 % of 7q31 deletion was detected by fish. Two years after the onset of mds, without any intervention, the patient achieved hematological remission with no chromosomal abnormality
Treatment & Mechanisms
Proposed Remission Mechanisms
Not discussed
Clinical Treatment
Partial excision. Arabinoside (arac), vincristine (vcr) and prednisolone (psl) (chemotherapy) method- trexate (mtreatment), vinblastine (vbl) and 6-mercapatientopurine (6mp) (chemotherapy)