A CD7 positive acute leukemia, lacking CD4, CD8, CD3, CD13 and CD33 expression may include 4 categories; acute T-cell leukemia, mixed lineage leukemia, acute undifferentiated leukemia and CD7 positive acute myeloid leukemia. Therefore, the expression of cyCD3 or the presence of TCR gene rearrangement can make the diagnosis of acute T-cell leukemia. We report a patient with acute T-cell lymphoblastic leukemia, showing CD7+, CD4-CD8-, and CD3-expression and TCR gamma gene rearrangement.
Diagnosis ; Genes, T-Cell Receptor ; Genes, T-Cell Receptor gamma ; Humans ; Leukemia ; Leukemia, Myeloid, Acute ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; T-Lymphocytes

BACKGROUND: Precursor T-cell acute lymphoblastic leukemia (T-ALL) has worse prognosis than B-cell ALL. We aimed to evaluate prognostic variables in pediatric T-ALL. METHODS: Medical records of 36 T-ALL patients (27 males and 9 females; median age at diagnosis, 10.6 years) diagnosed and treated at Asan Medical Center from 2001 to 2017 were reviewed. Six patients (16.7%) had early T-cell precursor ALL (ETP-ALL). Most patients received the Children's Cancer Group-1882 (CCG1882) or Korean multicenter high risk ALL (ALL0601) protocols and prophylactic cranial irradiation. Clinical features at presentation, response to therapy, and treatment outcomes were analyzed. RESULTS: The six patients with ETP-ALL and 17 of 30 with non-ETP-ALL received CCG1882 or ALL0601 chemotherapy. Three patients, including two with ETP-ALL, did not achieve complete remission after induction. Rapid early response during induction was achieved by 26 patients. Five year overall survival (OS) and event free survival (EFS) rates were 71.4% and 70.2%, respectively. ETP-ALL and slow early response during induction were significant adverse prognostic factors, while hyperleukocytosis at diagnosis was not. CCG1882/ALL0601 chemotherapy resulted in superior survival (OS: 78.9%, EFS: 73.3%) compared with CCG1901 chemotherapy (OS: 64.3%, EFS: 64.3%), and patients undergoing prophylactic cranial irradiation had superior EFS to non-radiated patients. CONCLUSION: A high risk ALL protocol with intensified post-remission therapy, including prophylactic cranial irradiation, conferred T-ALL survival outcomes comparable with those of Western studies. Further treatment intensification should be considered for patients with ETP-ALL and slow induction responders. Additionally, CNS-directed treatment intensification, without prophylactic cranial irradiation, is needed.
B-Lymphocytes ; Chungcheongnam-do ; Cranial Irradiation ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Female ; Humans ; Male ; Medical Records ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, T-Lymphoid ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; T-Lymphocytes*

We experienced a 22-year old patient with a documented history of minimal change nephrotic syndrome (MCNS), and a diagnosis of acute lymphoblastic leukemia (ALL) was then made for this patient. The patient received standard daily steroid therapy for the treatment of nephrotic syndrome. Cyclosporin A was administered because there was no clinical improvement with steroid therapy. Six years after the diagnosis of nephrotic syndrome, the patient was diagnosed with ALL. After chemotherapy for ALL, the patient was in complete remission and he showed clinical improvement of nephrotic syndrome. The hematological malignancies associated with nephrotic syndrome are mainly lymphoma and chronic lymphocytic leukemia. ALL has rarely been described in combination with nephrotic syndrome. Although the exact mechanism for development of ALL after nephrotic syndrome is unknown, at least two possibilities exist. First, the incidence of leukemia may be increased after immunosuppressive therapy, which may include cyclosporin A. Second, the underlying defect in T-lymphocyte function could account for both nephrotic syndrome and ALL. The possible mechanisms for such a relationship are discussed here along with a review of the relevant literature.
Cyclosporine ; Diagnosis ; Drug Therapy ; Hematologic Neoplasms ; Humans ; Incidence ; Leukemia ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lymphoma ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; T-Lymphocytes ; Young Adult

BACKGROUND: Immunophenotyping is an important technique for the diagnosis and classification of acute leukemia, as well as French-American-British (FAB) classification on the basis of morphologic characteristics and cytochemistry. We evaluated the expression patterns of immunologic surface markers in acute leukemia. METHODS: Peripheral or bone marrow leukemic cells from 75 leukemic patients (acute lymphoblastic leukemia, ALL 40 cases; children (26 cases), adults (14 cases) and acute myeloid leukemia, AML 35 cases; children (9 cases), adults (26 cases)) were studied. Monoclonal antibodies which were designed for two color direct immunofluorescence (IF) analysis with combination of fluoresceinisothiocynate (FITC) and phycoerythrin (PE) conjugated, CD10/CD19, CD20/CD5, CD3/CD22, CD7/CD33, HLA-DR/CD13 (Acute Leukemia Phenotyping Kit, Becton Dickinson; BD, USA) were analyzed by flow cytometry. RESULTS: Blasts from these patients could be classified as CALLA (+)B-ALL (26 cases, 65.0%), CALLA (-)B-ALL (6 cases, 15.0%), T-ALL (6 cases, 15.0%), biphenotypic ALL (2 cases, 5.0%). The positive expression rates were CD19 (100%), CD10 (78.1%), CD22 (75.0%) and CD20 (50.0%) in B-ALL, CD7 (100%), CD3 (50.0%) and CD5 (50.0%) in T-ALL and CD33 (85.7%), CD13 (74.3%) in AML, respectively. The incidence of acute mixed lineage leukemia (AMLL) was 26.7% and leukocytosis, anemia and thrombocytopenia were frequently seen in AMLL. CONCLUSION: By the study of immunophenotyping we could more exactly diagnosed ALL and AML, as well as AMLL which was not exactly diagnosed by characteristics of morphology and cytochemistry only. Therefore the best method for the diagnosis of acute leukemia will be achieved by using of immunophenotyping and FAB classification on the basis of morphology and cytochemistry.
Adult ; Anemia ; Antibodies, Monoclonal ; Antigens, Surface* ; Bone Marrow ; Child ; Classification ; Diagnosis ; Flow Cytometry ; Fluorescent Antibody Technique, Direct ; Histocytochemistry ; Humans ; Immunophenotyping ; Incidence ; Leukemia* ; Leukemia, Myeloid, Acute ; Leukocytosis ; Phycoerythrin ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Thrombocytopenia

Objective: To evaluate the clinical characteristics of T-cell acute leukemia/lymphoma (T-ALL) and explore the prognosis significance of early T-cell precursor leukemia/lymphoma. Methods: A cohort of 126 patients diagnosed with T-ALL from 2008 to 2014 in West China Hospital, Sichuan University were enrolled in this study. They were further categorized by immunophenotype according to the expression of T-cell lineage markers CD1a, CD8, CD5 and one or more stem cell or myeloid markers. The laboratory indicators and prognosis factors were also statistically analyzed. Results: Of all patients, the ratio of male to female was 2.5∶1, with the median age of 25 years old (range 14 to 77) . The percentage of ETP-ALL was up to 47.6%. T-ALL patients showed higher ratio in first clinical remission rate (CR(1)) than T-LBL ones (64.4% vs 30.8%, P=0.032) . Group with WBC count higher than 50×10(9)/L at presentation showed higher ration of achieving CR(1) than those lower than 50×10(9)/L (78.4% vs 50.9%, P=0.010) . In comparison with the non-ETP-ALL, ETP-ALL patients had older age of onset (P<0.001) , lower WBC count (P<0.001) , lower risk of CNS involvement (10.0% vs 30.2%, P=0.009) and slightly inferior overall survival (P=0.073) . T-cell lineage markers CD1a(-), CD8(-) and CD4(-) positive patients had higher CR(1) than their corresponding negative ones (P=0.002, P=0.000, P=0.001) , while CD33(-) and CD56(-) positive patients had lower ratio of achieving CR(1) than their negative ones, respectively (P=0.035, P=0.035) . Conclusion: Flow cytometry and associated markers for immunophenotyping was of significance in the diagnosis and prognosis monitoring of T-ALL/LBL. The percentage of ETP-ALL/LBL subtype was high in Chinese adolescent and adult T-ALL patients. ETP-ALL/LBL was a high risk subtype, which needs more precise standard for diagnosis and advanced therapies for better outcome.
Adolescent ; Adult ; Aged ; China ; Female ; Humans ; Immunophenotyping ; Male ; Middle Aged ; Precursor Cells, T-Lymphoid/cytology* ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/diagnosis* ; Prognosis ; Young Adult

We herein present a case of a 2-year-old girl with non-Hodgkin's lymphoma(NHL) of the lymphoblastic type involving cutaneous sites at the time of diagnosis. The histological finding was typical of lymphoblastic lymphoma. However, immunophenotypically, this lymphoma was not of the T-cell or B-cell type, although the vast majority of lymphoblastic lymphomas involving the skin are usually of the pre-B cell or T-ce11 type. Until now, there have been few reports of non-T, non-B primary cutaneous lymphoblastic lymphoma expressing surface CD10 and CD56 antigens as in this case.
Antigens, CD56 ; B-Lymphocytes ; Child, Preschool ; Diagnosis ; Female ; Humans ; Lymphoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Skin ; T-Lymphocytes

To analyze the treatment and prognosis of T cell acute lymphoblastic leukemia(T-ALL)in adults. Method The clinicobiogical and survival data of 68 adult patients with newly diagnosis T-ALL were retrospectively analzyed. Results The median age of these 68 patients was 23 years(14-60 years).T-ALL was more common in men(81%).After the first cycle of treatment,complete remission was achieved in 50 patients(73%).The highest complete remission(CR) rate was in patients with cortex T-ALL(100%),followed by other T-ALL(73%)and early T-cell precursor lymphoblastic leukemia(54%),(=5.712,=0.058).The CR rate for adults aged >35 years was significantly lower than that of patients aged ≤ 35 years(40% 79%,=6.364,=0.012).The overall CR rate after the second treatment course was 93%.For patients treated with chemotherapy,autograft hematopoietic stem cell transplantation(auto-SCT),and allogeneic SCT,the median relapse free survival was 10 months,24 months,and not reached,respectively(=0.002).The 5-year overall survival rate was 25% for all patients;for patients treated with chemotherapy,auto-SCT and allogeneic SCT,the median overall survival was 24 months,34 months,and 30 months,respectively(=0.007),and the 5-year overall survival rate was 9%,33%,and 38%(=0.037).Multivariate analysis showed leukocyte count ≥100×10 /L was a risk factor for decreased relapse free survival(risk ratio 2.540,95%=1.058-6.099,=0.037). Conclusion Adult T-ALL patients have poor prognosis,which may be improved by SCT.
Adolescent ; Adult ; Disease-Free Survival ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Male ; Middle Aged ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; therapy ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; therapy ; Prognosis ; Remission Induction ; Retrospective Studies ; Survival Rate ; Treatment Outcome ; Young Adult

Burkitt Lymphoma ; diagnosis ; pathology ; Female ; Hodgkin Disease ; diagnosis ; pathology ; Humans ; Lymphoma ; classification ; diagnosis ; pathology ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; pathology ; Lymphoma, Follicular ; diagnosis ; pathology ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; pathology ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; pathology

JL1 is a novel human thymocyte differentiation antigen, which is exclusively expressed by double positive (CD4+ CD8+) cortical thymocytes. We previously reported that the JL1 antigen was selectively expressed on the surface of acute lymphoblastic leukemia cells. T-Lymphoblastic lymphoma/acute lymphoblastic leukemia (T-LBL/ALL), the 6th prevalent lymphoma in Korea, is composed of immature neoplastic lymphoid cells and shows a rapid response to appropriate treatment. Early and precise diagnosis of LBL/ALL is crucial. Light microscopic distinction of LBL/ALL from other non-Hodgkin's lymphomas can sometimes be difficult and is aided by immunophenotypic studies. This study is designed to investigate the diagnostic utility of anti-JL1 monoclonal antibody (YG5) for LBL/ALL in formalin fixed, paraffin embedded tissue. We collected 25 cases of LBL/ALL (18 T-cell, 5 B-cell and 2 undetermined lineage) from 1993 through 1998. We confirmed the diagnosis using morphologic and immunophenotypic data. Strong JL1 expression along cell membrane was observed in 16 out of 18 T-LBL/ALL cases (89%). In 28 cases of other types of lymphomas of including 7 cases of non-T LBL/ALL and 14 cases of small round cell tumors, no JL1 expression was identified. These results show that the immunostaining for JL1 using YG5 on paraffin embedded sections can be useful for the specific diagnosis of T-LBL/ALL in routine diagnostic service.
B-Lymphocytes ; Cell Membrane ; Diagnosis ; Diagnostic Services ; Formaldehyde ; Humans ; Immunologic Tests ; Korea ; Lymphocytes ; Lymphoma ; Lymphoma, Non-Hodgkin ; Paraffin* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; T-Lymphocytes ; Thymocytes

INTRODUCTIONThe analysis of immunophenotype of the leukaemic cells has been of great importance for the diagnosis, classification and prognosis of acute lymphoblastic leukaemia (ALL).

MATERIALS AND METHODSOne hundred and thirteen Chinese patients with ALL were immunophenotyped by fl ow cytometry and 74 cases were also subjected to karyotype analysis by G-banding technology.

RESULTSOf the 113 Chinese ALL patients, 14.2% were identified as T-ALL and 85.8% as B-ALL. Myeloid antigen (MyAg) expression was documented in 34.9% of the cases analysed and CD13 was most commonly expressed MyAg in ALL patients (23.6%). MyAg positivity was higher in adult with ALL (47.6%) than in children with ALL (26.6%). Abnormal karyotypes were detected in 39 out of 74 (52.7%) cases. The clinical and biological characteristics of ALL patients between MyAg+ and MyAg- groups showed that increased white blood count (WBC) (>50 x 109/L), higher CD34 positivity and higher percentage of adult patients were found to be correlated with MyAg+ ALL.

CONCLUSIONOur results indicate that the immunophenotype did have relevance to the abnormal cytogenetic changes and clinical features in ALL. Flow cytometry immunophenotype has become the most important method for diagnosis and typing of ALL.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; China ; epidemiology ; Cytogenetic Analysis ; Diploidy ; Female ; Humans ; Immunophenotyping ; Infant ; Male ; Middle Aged ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; classification ; diagnosis ; genetics ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; classification ; diagnosis ; genetics ; Translocation, Genetic ; Young Adult