Chiai Nagae

T-cell large granular lymphocyte leukemia in a child with anemia and Crohn's disease

T‐LGL leukemia has been rarely reported in children. We report a child with T‐LGL leukemia who presented with anemia and went on to develop Crohns disease. Although prednisolone treatment proved effective in the treatment of anemia, large granular lymphocyte counts increased as the doses were tapered. T‐cell rearrangement studies revealed a clonal rearrangement of the TCR Vβ/jβ2 gene. Concurrently, the patient developed severe diarrhea. Inflammatory changes across the upper and lower intestines led to the diagnosis of Crohns disease. This case highlights that T‐LGL leukemia could be occurred in children. Flow cytometry and/or T‐cell gene rearrangement studies are recommend in patients of anemia and various kind of autoimmune diseases including Crohns disease, even in children.

Systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease (SM-AHNMD) in an infant patient

no tumor lesions were in the mammary gland and that the tumor was not connected with the mammary gland. CT, esophagogastroduodenoscopy and colonoscopy showed no digestive organ cancers. The patient had a history of prostate cancer that was successfully treated with radiation therapy, and no recurrence was observed by follow-up CT and serum PAS examinations for 4 years. Negative cytokeratin 20 and PAS staining supported the final diagnosis of PMCS in the breast with LN metastasis. In 73 patients studied by Snow and Reizner, PMCS was found to be most prevalent in the face (60.3%) and scalp (16.4%), and rare in the chest (2.7%). Breiting et al. studied 15 cases of PMCS and reported none were in the breast. Metastasis of PMCS is very rare. Among 15 cases studied by Breiting et al., LN metastasis was seen only in one case: neck LN metastasis from a primary lesion in the scalp. Amano et al. reported a case with PMCS in the hypogastric region who had shown inguinal LN metastases 2 years after surgery. The present case had the primary tumor in the breast, with axillary LN metastasis, although the onset time of the LN metastasis was unknown. These cases may support the hypothesis of Kazakov et al. that the closeness of the primary lesions to the regional LN is associated with the occurrence of LN metastasis in PMCS. The present case gives the important lesson that PMCS can occur on the breast and metastasize to regional LN.