Clinical Analysis And Literature Review Of Two Rare Types Of Leukemia

SECTION ⅠClinical analysis of 20 cases of hairy cell leukemia and literature review[Background]Hairy cell leukemia(HCL)is a clinically rare B-lymphocyte proliferative disease characterized by infiltration of bone marrow and peripheral blood hair cells and often involving the spleen.The clinical manifestations of the disease lack specificity,often manifesting as fatigue,abdominal distension,abdominal pain,bleeding and repeated infections,as well as signs of hepatosplenomegaly,lymphadenopathy,etc.Laboratory tests have shown that patients with HCL often suffer from pancytopenia,typical hairy cells seen in bone marrow and peripheral blood,and immunotyping usually displays characteristic surface markers.The disease progresses slowly,and the first-line treatment drugs are purine analogs(such as cladribine,pentastatin)and interferon,with a high response rate.This article retrospectively analyzes the clinical and laboratory examination data of 20 HCL patients admitted to Qilu Hospital of Shandong University from January 2006 to December 2019,including the clinical manifestations,cell morphology,histopathology,and immunological markers.The summary of the data explores the clinical features,treatment options and survival of HCL.[Objective]1.Analyze the clinical characteristics and laboratory results of 20 patients with hairy cell leukemia to understand the epidemiological and clinical characteristics of HCL.2.Analyze the treatment effect of hairy cell leukemia patients treated with different drugs,and compare the advantages and disadvantages of different treatment options.3.Review and summarize the cases of hairy cell leukemia reported in the domestic and foreign literature in the past 10 years,and compare with the above results.[Methods]This study adopted a systematic review method,through reviewing the medical record system and telephone follow-up,collected clinical data of HCL patients admitted to Shandong University Qilu Hospital from January 2006 to December 2019.There is a total of 20 cases,and their characteristics such as clinical manifestations and cell morphology,biomarkers,histopathology and immunology,as well as the survival time and remission rate of different treatment options were summarized.The statistical software SPSS was used for statistical comparison,and p<0.05 was defined as a statistically significant difference.[Results]1.Clinical characteristics:Among the 20 HCL patients,the age distributed from 33 to 77 years,with a median age of 49 years,of which 15 were male and 5 were female,with a male to female ratio of 3:1.In terms of clinical manifestations,there were 11 cases with fatigue(55%),6 cases with abdominal discomfort(30%),2 cases with abdominal mass(10%),4 cases with anorexia(20%),8 cases with bleeding(40%),and 5 cases with infection(25%),2 cases with fever(10%),2 cases with skin manifestations(10%),17 cases with splenomegaly(85%),5 cases with hepatomegaly(25%),and 11 cases with lymphadenopathy(55%).2.Laboratory examination:① Blood routine examination:white blood cell count ranges from 0.63×109/L to 65.13×109/L,with median of 3.075×109/L,and 13 cases(65%)presented with leucopenia,3 cases(15%)with leucocytosis.The variation range of neutrophil count is from 0.185×109/L to 4.33 ×109/L(median 0.495 ×109/L),with lymphocyte count from 0.37×109/L to 61.41×109/L(median 1.46×109/L).Monocyte count is from 0×109/L to 5.84×109/L(median 0.14 × 109/L),with monocyte decreased in 15 cases(75%).At the time of diagnosis,16(80%)of patients had at least two cell line reductions,and 12(60%)of patients had pancytopenia.②Bone marrow examination:19 patients underwent bone marrow aspiration in our hospital.Bone marrow smears showed atypical lymphocytic proliferation in 18 cases(94.7%),hairy cells in 14 cases(73.7%).14 patients underwent bone marrow biopsy.12(85.7%)patients had bone marrow fibrosis,of which one was grade 3,8 was grade 2,and 3 was grade 1.③ Immunotyping and gene mutation:14 cases were tested for immunotyping,and 9 cases were tested for BRAF gene mutation.The immunophenotyping of 13 cases of showed CD19+,CD20+,CD5-,CD10-,CD11c+,CD25+,CD103+,of which 7 were positive for BRAF-V600E mutations,in line with the classic hairy cell leukemia(cHCL)phenotype.One case showed CD11c+,CD25-,CD 123-,and BRAF-V600E mutation was negative,which was consistent with the Hairy cell leukemia variant(HCL-V)phenotype.3.Treatment and prognosis:15 cases were treated,of which 10 cases were treated with cladribine(7.85-10mg ivdrip qd × 5d),2 cases were treated with interferon,1 case was performed splenectomy,and 1 case was oral treatment with "glucocorticoids,platelet capsules,vitamin C".4 months after recieving treatment,the therapeutic effect was evaluated,and 13 patients achieved hematological remission,including 8 HCR(53.3%)and 5 HPR(33.3%).2 other cases did not reach remission and the disease was stable.All patients were followed up to February 2020,of which 5 were lost to follow-up,14 were alive,and 1 died.4.Drug side effects:The side effects of cladribine are mainly myelosuppression and infection.7 of 10 patients had granulocytopenia,5 had granulocytopenia combined with infection,of which 4 had mixed infections of bacteria and fungi in the lung.Meanwhile,1 had a drug-induced liver damage.[Conclusion]1.75%of HCL patients are middle-aged men.The main clinical symptoms are fatigue,abdominal discomfort,bleeding and infection,and the main sign is splenomegaly.2.The blood routine of patients with HCL often shows pancytopenia with a decrease in monocyte.Bone marrow examination usually shows atypical lymphocyte proliferation and hairy cell infiltration,and often combined with different degrees of bone marrow fibrosis.cHCL and HCL-V are different in immune typing,BRAF-V600E mutation,etc.In this group of 13 cases,the cHCL phenotype is met,and 1 case is consistent with the variant HCL-V phenotype.3.The therapeutic effect of HCL is good,and the purine analog group is better.The main side effect of cladribine drug therapy is bone marrow suppression combined with infection.In clinical work,attention should be paid to strengthening the prevention and treatment of infection.SECTION ⅡClinical analysis of 21 cases of T-cell large granular leukemia and literature review[Background]T-cell large granular lymphocytic leukemia(T-LGLL)is a rare proliferative disease of the lymphatic system,characterized by clonal proliferation of T-cell large granular lymphocyte(T-LGL).sexual proliferation.This kind of disease shows a chronic course,and the blood routine tests often show one or two lines of cell reduction,with granulocytopenia more common.Another significant feature of T-LGLL is that it is often associated with autoimmune diseases,such as rheumatoid arthritis,systemic lupus erythematosus,and Sjogren’s syndrome.The first-line treatment is usually immunosuppressive therapy.This article retrospectively analyzes the clinical and laboratory data of 21 T-LGLL patients admitted to Qilu Hospital of Shandong University from February 2009 to December 2019.The clinical characteristics,cell morphology,and immunology of T-LGLL patients were analyzed.The summary of the data explores the clinical features,treatment options and prognosis of T-LGLL.[Objective]In this study,a systematic review method was used to collect clinical data of 21 patients with T-LGLL admitted to Qilu Hospital of Shandong University from February 2009 to December 2019 by reviewing the medical record system and telephone follow-up.The characteristics of cell morphology,whether to combine autoimmune diseases,immune phenotypes,and the survival time and remission rate of different treatment options were statistically compared using statistical software SPSS,defining p<0.05 as statistically significant.[Results]1.Clinical features:The age of 21 T-LGLL patients distributed from 29 to 77 years,with a median age of 59 years.Among them,16 cases were male and 5 cases were female with the ratio of male to female about 3:1.There were 18 patients with clinical symptoms,including 13 cases(61.9%)of fatigue or fatigue,6 cases(23.8%)of abdominal discomfort or masses,1 case(4.8%)of bleeding,5 cases(28.6%)of infection,and 4 Cases(19%)of weight loss.On physical examination,13 patients(61.9%)had splenomegaly and 5 patients(19%)had lymphadenopathy.Six patients(28.6%)had autoimmune diseases,including 4 patients with pure red blood cell aplastic anemia(PRCA),2 patients with autoimmune hemolytic anemia(AIHA),and 1 patient with rheumatoid arthritis(RA).2.Laboratory examination:① Blood routine examination:In 21 patients with T-LGLL,white blood cell count ranged from 1.14x109/L to 39.81 ×109/L,with median of 4.22×109/L.9 cases(42.9%)presented with leukopenia,5 cases(23.8%)of leukocytosis.Neutrophil count was from 0.7×109/L to 15.7×109/L,with median of 1.74×109/L,9 cases(42.9%)of neutropenia and no granulocytosis(ANC<0.5 × 109/L).Lymphocyte count varied from 0.3×109/L to 37.16×109/L,with median 2.35×109/L,and lymphocyte ratio was from 15.6%to 93.4%(median 58.6%),with lymphocytes increased in 12 patients.The variation range of hemoglobin was from 38g/L to 156g/L(median 70g/L),and 15 patients(71.4%)developed anemia.The maximum and minimum of platelet count was respectively 43 ×109/L and 619×109/L(median 244×109/L),4 patients(19.0%)with thrombocytopenia.②Bone marrow examination:16 cases(76.2%)showed an increase in the number of large granule lymphocytes in bone marrow and/or peripheral blood smears.Bone marrow biopsy hyperplasia was active or low,with lymphocytes increased,granulocyte and erythroid hyperplasia suppressed.2 patients were combined with bone marrow fibrosis.③Immunotyping:20 patients underwent immunophenotyping,16(80%)showed CD3+CD4-CD8+,1 showed CD3+CD4-CD8-,and 2 showed CD3+CD4+CD8+.93.8%of patients expressed CD57+,40%expressed CD1 6+,and 45.4%expressed CD3+CD56+.④Gene rearrangement:18 patients were tested for gene rearrangement,in which 16 cases were positive,and the total detection rate was 88.9%.The positive rate of TCRβ detected by PCR was 55.6%,with 72.2%of TCRγ,and 14.3%of TCRδ.⑤ Others:1 out of 10 patients detected add(3)(q29)clonal abnormality,and the remaining 9 patients had normal karyotype.Three patients were positive for antinuclear antibodies,and one patient had all types of immunoglobulins decreased,diagnosis with T-LGLL secondary to common variant immunodeficiency disease type Ⅰ.3.Treatment and prognosis:One patient had no indication for treatment,and the remaining 20 patients received treatment:14 patients received cyclosporine-based immunosuppressive therapy,and 2 patients received cyclophosphamide-based therapy.2 patients received COP chemotherapy After one cycle,and sequential treatment with cyclosporine was given.2 cases were treated with glucocorticoid alone.Twenty patients receiving treatment have been followed up until now,with a median follow-up time of 28(4-134)months,2 lost to follow-up,4 died,and the remaining 14 have survived to this day.After 6 months of treatment,the treatment effect was evaluated.The total effective rate of 20 patients was 60%,with the complete remission rate of hematology 45%and the partial remission rate 15%.[Conclusion]1.Most patients with T-LGLL are middle-aged and elder men,and the main clinical manifestations are fatigue,infection,and splenomegaly,which are often combined with autoimmune diseases.2.The routine blood test of T-LGLL patients usually shows blood cell reduction,and bone marrow smears show large granulocytes increase,and bone marrow biopsy shows suppression of granulocyte hyperplasia.80%of patients meet the T-LGLL classic immunophenotype CD3+CD4-CD8+,and the gene rearrangement test is usually positive.3.T-LGLL usually progresses slowly.The first-line treatment is immunosuppressive therapy.Others include combination chemotherapy,splenectomy,and targeted therapy.