ABSTRACT
An eight-year-old girl was referred and admitted to our hospital with the chief complaint of purpura on her lower legs. Blood tests revealed pancytopenia, and bone marrow findings showed marrow hypoplasia. Refractory cytopenia of childhood (RCC) was diagnosed based on the central diagnostic system of the Myelodysplastic Syndrome Committee of the Japanese Society of Pediatric Hematology. Immunosuppressive therapy was performed with the administration of rabbit antithymocyte globulin, methylprednisolone and cyclosporin A,but it was not effective. Eight months after admission to our hospital, Kampo treatment was started based on traditional Kampo diagnosis. After treatment with oral administration of kamikihito and kyukikyogaito, her pancytopenia gradually improved. Erythrocyte transfusion was discontinued after 2 months, and concentrated platelet transfusion also became unnecessary after 3 months. As a result of improvement in pancytopenia, her white blood cell count, hemoglobin value, and platelet count reached almost normal levels after 16 months. The scheduled bone marrow transplantation was canceled. The action mechanisms of kamikihito and kyukikyogaito for RCC are not clear, and their effective rates are also unknown. However, Kampo treatments are less invasive, inexpensive, and have few side effects. We believe that Kampo medicine is a therapeutic method that should be actively attempted in cases of RCC with poor response to standard treatment.
ABSTRACT
Myelodysplastic syndromes are a group of malignant myeloid clonal diseases that originate from hematopoietic stem/progenitor cells.They are characterized by decreased peripheral blood cells,dysplasia of one or multiple lineages of myeloid hematopoietic cells,and prone to transform into acute myeloid leukemia.Refractory cytopenia of childhood is the most common clinical type of MDS in children.Its clinical manifestations include the decrease in one or multiple lineages of peripheral blood cells,abnormal development of the myeloid lineage,and the absence of excess blasts.This type was first introduced in WHO children's MDS in 2008.Considering the low incidence and lack of specific diagnostic methods,difficulty in early diagnosis and poor prognosis of RCC,we summarize the definition,clinical manifestations,laboratory tests,diagnosis and differential diagnosis,and treatment strategies of RCC in this review.