Hemophagocytic lymphohistiocytosis (HLH), although uncommon, is illustrated as a dramatic clinical presentation of multi-systemic inflammation due to the impaired activity of cytotoxic T-cell and NK cell. Etoposide, an important component of the HLH treatment, is thought to have a leukemogenic potential. Furthermore, it has been suggested that the underlying immunologic dysfunction in HLH might be involved in the occurrence of secondary leukemia. Only a few cases of secondary ALLs after HLH have been reported. We report herein a case of secondary precursor B-cell ALL which occurred after the treatment of HLH.
Etoposide ; Inflammation ; Killer Cells, Natural ; Leukemia ; Lymphohistiocytosis, Hemophagocytic ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cells, B-Lymphoid ; 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

<b>OBJECTIVEb>To investigate the expression of CD123 and its significance in lymphocytic leukemia.

<b>METHODSb>CD123 expression in 139 lymphocytic leukemia patients and in lymphocytes from 10 normal bone marrows (BM) was analyzed by multi-parameter flow cytometry. Cytogenetic and minimal residual disease (MRD) analysis were performed in acute B-lymphocytic leukemia (B-ALL) patients.

<b>RESULTSb>CD123 expression was absent in B lymphoid lineage stem-progenitor cells, mature B and T lymphocytes from 10 normal BM. Among 139 lymphocytic leukemia patients, CD123 was negative in 5 T-ALL and 23 B-CLL patients. However, among 111 B-ALL patients, CD123 was expressed in 106 (12 pro B-ALL, 57 common B-ALL and 37 Pre B-ALL) (95.49%) but not in 5 mature B-ALL patients. There was a positive correlation between CD123 and p-Akt expression, and CD123 expression was much higher in hyperdiploid than in non-hyperdiploid B-ALL patients. A statistically significant difference in relapse rate within 12 months (MRD positive group: 63.04% vs MRD negative group 21.56%)and in disease free survival (DFS) time was found beween patients with MRD\[(36.06 +/- 2.62)%\] or not \[(48.23 +/- 1.82)%\] (P < 0.01). Moreover, stable CD123 expression could be observed in B-ALL patients in relapse.

<b>CONCLUSIONSb>CD123 was predominantly expressed in B-ALL patients and remained in patients in relapsec, indicating that it may be an useful MRD marker in B-ALL patients.

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 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

Pneumatosis intestinalis (PI) is an imaging phenomenon that represents air in the bowel wall. The cause of PI is variable, although specific etiologic factors remain unknown. It is an infrequent complication in leukemia patients and is associated with several medical and surgical conditions. PI often represents a benign condition, but it can also require surgery. Therefore, the assessment of PI with or without complications can be difficult. Herein, we report on an unusual case of a 63 year-old woman with refractory acute precursor B-cell lymphoblastic leukemia-lymphoma who presented with PI resulting from the leukemic process, and finally expired due to sepsis.
Female ; Humans ; Leukemia ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cells, B-Lymphoid ; Sepsis

No abstract available.
Leukemia* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, T-Lymphoid*

BACKGROUND: Precursor B-cell acute lymphoblastic leukemia (B-cell ALL) is the most common neoplasm in children and is characterized by genetic and epigenetic aberrations in hematopoietic transcription factor (TF) genes. This study evaluated promoter DNA methylation and aberrant expression levels of early- and late-acting hematopoietic TF genes homeobox A4 and A5 (HOXA4 and HOXA5), Meis homeobox 1 (MEIS1), T-cell acute lymphocytic leukemia 1 (TAL1), and interferon regulatory factors 4 and 8 (IRF4 and IRF8) in pediatric B-cell ALL. METHODS: Blood samples of 38 ALL patients and 20 controls were obtained. DNA was treated with sodium bisulfite and DNA methylation level of HOXA4, HOXA5, MEIS1, TAL1, IRF4, and IRF8 was assessed using quantitative methylation-specific polymerase chain reaction (PCR). Relative gene expression was measured using quantitative reverse transcription-PCR. RESULTS: Aberrant methylation of TAL1, IRF8, MEIS1, and IRF4 was observed in 26.3%, 7.9%, 5.3%, and 2.6% patients, respectively, but not in controls. HOXA4 and HOXA5 were methylated in some controls and hypermethylated in 16% and 5% patients, respectively. IRF8, MEIS1, and TAL1 expression was lower in patients than in controls. MEIS1 expression was inversely correlated with white blood cell (WBC) count. HOXA4 expression was down-regulated in patients with high risk according to the National Cancer Institute (NCI) classification. TAL1 methylation was slightly elevated in patients aged >9 years and in patients showing relapse, suggesting its potential prognostic value. CONCLUSION: Aberrant methylation and expression of the selected hematopoietic genes were correlated with demographic/clinical prognostic factors of pediatric ALL, such as age, WBC count, and NCI risk classification.
B-Lymphocytes* ; Child ; Classification ; DNA ; DNA Methylation ; Epigenomics ; Gene Expression ; Genes, Homeobox ; Humans ; Interferon Regulatory Factors ; Leukocytes ; Methylation* ; National Cancer Institute (U.S.) ; Polymerase Chain Reaction ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence ; Sodium ; Transcription Factors

Precursor B-cell acute lymphoblastic leukemia (ALL), which is the most common subtype of pediatric acute leukemia, generally has a good prognosis. However, the prognosis also depends on the genetic abnormalities of the leukemic blast. Concurrent MYC and IGH/BCL2 translocations have recently been reported as a “double hit” in adult patients, but non-immunoglobulin (non-IG)/MYC translocation has rarely been reported. In this paper, we report a case of pediatric precursor B-cell ALL associated with translocations (14;18)(q32;q21) and (8;9)(q24;p13). The patient was a previously healthy 13-year-old boy. Complete remission was not achieved after first-line four-drug induction chemotherapy; thus, intensive salvage regimen, including high-dose cytarabine and L-asparaginase, were administered, which resulted in morphologic remission. However, his disease relapsed during the second cycle of salvage regimen, and he died of sepsis-induced multiorgan failure.
Adolescent ; Adult ; Cytarabine ; Humans ; Induction Chemotherapy ; Leukemia ; Male ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cells, B-Lymphoid ; Prognosis

BACKGROUND: CD34 is expressed by early hematopoietic (myeloid & lymphoid) progenitor cells, endothelial cells, and bone marrow stromal cells. Recent studies have suggested that the expression of the hematopoietic antigen CD34 is predictive of outcome. But the functional importance of CD34 antigen has not yet been fully elucidated, and CD34 antigen has not yet been included in the routine immunophenotyping panel. We designed this experiment to evaluate the significance of including CD34 antigen in the routine immunophenotyping panel. MATERIALS AND METHODS: We have included CD34 antigen in the immunophenotyping panel from 1994 to 1997. Seventy eight patients with acute leukemia and 7 patients with chronic leukemia and CML-blast crisis were analyzed by direct immunofluorescence using monoclonal antibodies. RESULTS: CD34 was positive in 64% (50/78) of cases with acute leukemia and 100% (3/3) of cases with CML-blast crisis, but negative in 100% (4/4) of cases with chronic lymphocytic leukemia. Seventeen (63%) of 27 patients that belonged to AML FAB subtype M0, M1, M2, M4, M5, M7 were CD34-positive, but all of the 9 patients (100%) that belonged to AML FAB M3 were CD34-negative. Thirty-one patients of CD34-positive non-T-ALL belonged to immunologic group II, III, or IV, and all 2 patients of CD34-positive T-ALL belonged to immunologic group I. There are no significant correlations of CD34 with the remission rate and the survival rate of acute leukemia. CONCLUSIONS: CD34 facilitates the rapid diagnosis of acute promyelocytic leukemia (APL) different from the other AML subtypes in clinical course and therapeutic plan. It is also helpful for the immunologic grouping of ALL, and differentiation of chronic leukemia from CML-blast crisis or acute leukemia. We concluded that it is desirable to include CD34 in the routine immunophenotyping panel.
Antibodies, Monoclonal ; Antigens, CD34 ; Diagnosis ; Endothelial Cells ; Fluorescent Antibody Technique, Direct ; Humans ; Immunophenotyping* ; Leukemia* ; Leukemia, Lymphocytic, Chronic, B-Cell ; Leukemia, Promyelocytic, Acute ; Mesenchymal Stromal Cells ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Stem Cells ; Survival Rate