Clinical And Laboratory Characteristics Of 3 Patients With Hairy Cell Leukemia

Objective:To discuss the clinical and laboratory characteristics of 3patients with hairy cell leukemia(HCL).Methods:Clinical data of 3 cases with HCL from October 2015 to June2017 in hematology department,Hebei general hospital were retrospective analyzed.Patients' clinical characteristics,laboratory examination and treatment were concluded.Results:1 The age of onset of HCL tends to be younger.All of the 3 patients are less than 50 years old.One patient is 30 years old,another one is 31 years old,and the third is 48 years old.The median age is 31.2 Two patients had fatigue,palpitation and shortness of breath.1patient had hemorrhagic spots in skin and mucous membrane.Splenomegaly is one of the main signs.1patient had megalosplenia,another patient had moderate splenomegaly,and the third had normal spleen.3 Two of the three patients had severe anemia and thrombocytopenia.1patient's blood routine examination was normal.The white blood cell count was increased in 2 patients(patients with HCL-c and HCL-v).1patient had normal white blood cell count.4 Flow cytometry analysis showed the cells of 3 patients were CD11c+,CD19+,CD20+,D22+,CD25+,FMC-7+,CD103+,CD200+.Two patients were Lambda and HLA-DR positive.The cells of patient with HCL-v were CD25+.5 One patient's bone marrow showed marked hyperplasia,another's showed hyperplasia,and the third one's showed hypoplasia.Two patients had“dry tap”when received bone marrow aspiration.Hairy cell infiltration was observed in all of the 3 patients' bone marrow and blood.The proportion of hair cells in peripheral blood was 36%-70%,as well as that in bone marrow was38%-65%.6 The immunohistochemical examination of bone marrow biopsy specimen of all the 3 patients showed CD11c+,CD20+,CD25+,CD103+.Beside this,2 patients with HCL-c had Annexin A1+,and 1 patient with HCL-v had Annexin A1-.7 Hairy cells were observed by electron microscope scanning.The diameter of the cells was 6-12 um.There were a large number of protrusions,branched and hairy villi on the surface of the cells.The length of the villi was as long as 5um.8 Two patients had BRAF gene mutation positive.One patient had BRAF gene mutation negative.9 The OS of carcrabin in 3 patients was 100%.The effect of carcrabin seemed better than that of fludarabine.10 One patient(HCL-c)had thyroid papilloma which was confirmed by pathologic diagnosis and there was no evidence for secondary to purine nucleoside analogues treatment.The patient was presumed had multiple primary carcinomas.Conclusion:1 Hairy cell leukemia is a rare disease.The age of onset of HCL tends to be younger.2 Most of patients with HCL have abnormal white blood count,anemia,and splenomegaly.Two patients in this study were CD11c+,CD19+,CD20+,CD22+,CD25+,CD103+,CD123+,cyclin D1+,and BRAF gene mutation positive.One patient with HCL-v had BRAF gene negative.Characteristic hairy cells were observed by electron microscope scanning in 2 patients.3 The BRAF gene mutation is specific for the diagnosis of hairy cell leukemia.The electron microscope scanning is helpful for the diagnosis.The differential diagnosis between HCL and splenic marginal zone lymphoma with villi should be paid attention.4 All of the 3 patients were treated with nucleoside analogues.The effect of carcrabin seemed better than that of fludarabine.5 One patient had thyroid papilloma beside HCL,which was considered as multiple primary carcinomas.