ALK positive histiocytosis:a clinicopathological analysis of five cases and review of literature
Objective To analysis the clinical and pathological characteristics of ALK positive histiocytosis.Methods The clinical,morphological,and immunohistochemical results of 5 cases of ALK positive histiocytosis were summarized and followed up.Results There were 2 males and 3 females.Age ranged from 2 months to 21 years old.The course of the disease was 2 to 5 months.Symptoms mainly included sensory and activity disorders,skin nodules,decreased platelets and hemoglobin,etc.At low magnification,small lymphocytes and light stained histiocyte were seen.High magnification showed histiocyte had oval shaped nuclei,basophilic nucleoli and eosinophilic cytoplasm.Epidermotropic histocytes were noted.Three cases showed necrosis.Immunohistochemical staining was positive for CD68,CD163,and ALK.Follow-up for 4 to 26 months,with an average follow-up of 15.4 months.All cases showed ALK gene abnormalities,including four cases of KIF5B-ALK fusion and one of CLTC-ALK fusion.Conclusion ALK positive histiocytosis has a unique clinical and histological spectrum,and comprehensive morphology,immunohistochemistry and molecular detection are helpful for accurate diagnosis and post treatment evaluation.
HystiocytosisAraplastic lymphoma kinaseHistopathologyCentral nervous system
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NSTL
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