Bilateral renal enlargement is a very rare manifestation as the primary presenting feature of acute lymphoblastic leukemia. We are reporting an unusual clinical picture of a 9-month-old male patient diagnosed as precusor B-cell lymphoblastic leukemia, who showed bilateral nephromegaly without any hepatosplenomegaly at the time of initial presentation.
B-Lymphocytes ; Humans ; Infant ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

No abstract available.
B-Lymphocytes ; Humans ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

B-cell precursor acute lymphoblastic leukemia (BCP-ALL) is characterized by genetic alterations with high heterogeneity. Precise subtypes with distinct genomic and/or gene expression patterns have been recently revealed using high-throughput sequencing technology. Most of these profiles are associated with recurrent non-overlapping rearrangements or hotspot point mutations that are analogous to the established subtypes, such as DUX4 rearrangements, MEF2D rearrangements, ZNF384/ZNF362 rearrangements, NUTM1 rearrangements, BCL2/MYC and/or BCL6 rearrangements, ETV6-RUNX1-like gene expression, PAX5alt (diverse PAX5 alterations, including rearrangements, intragenic amplifications, or mutations), and hotspot mutations PAX5 (p.Pro80Arg) with biallelic PAX5 alterations, IKZF1 (p.Asn159Tyr), and ZEB2 (p.His1038Arg). These molecular subtypes could be classified by gene expression patterns with RNA-seq technology. Refined molecular classification greatly improved the treatment strategy. Multiagent therapy regimens, including target inhibitors (e.g., imatinib), immunomodulators, monoclonal antibodies, and chimeric antigen receptor T-cell (CAR-T) therapy, are transforming the clinical practice from chemotherapy drugs to personalized medicine in the field of risk-directed disease management. We provide an update on our knowledge of emerging molecular subtypes and therapeutic targets in BCP-ALL.
B-Lymphocytes ; Humans ; Mutation ; Oncogene Proteins, Fusion/genetics* ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

OBJECTIVE: To discover the relationship between matrix remodeling associated 7 (MXRA7) and acute B lymphoblastic leukemia (B-ALL), and explore the effect of MXRA7 on the biological functions of B-ALL cell line REH. METHODS: The expression of MXRA7 in blood diseases was searched and analyzed through BloodSpot database. Real-time qPCR was used to detect the expression level of MXRA7 in B-ALL cell line 697 and REH cells. Lentivirus-mediated shRNA interference technology was utilized to knock down the expression of MXRA7 in REH cells. The effects of MXRA7 on the biological functions of REH cells were studied by in vitro experiments. Cell proliferation was detected by CCK-8 assay, cell cycle was detected by PI staining, cell apoptosis was detected by Annexin V and 7-AAD staining, and the expression of apoptosis pathway related proteins was detected by Western blot. RESULTS: Database analysis showed that MXRA7 was highly expressed in B-ALL patients, and real-time qPCR results showed that MXRA7 was also highly expressed in cell lines 697 and REH cells. Knockdown of MXRA7 in REH cells inhibited the cell proliferation and increased the percentage of G₀/G₁ phase cells. After treatment with cytarabine, the apoptotic ratio was increased in MXRA7-impaired REH cells, and the activation of caspase-3 and caspase-9 were also increased. CONCLUSION Knockdown of MXRA7 can reduce the malignancy of REH cells by inhibiting the cell proliferation and increasing the sensitivity of REH cells to cytarabine. These results indicate MXRA7 may be as a novel target for the treatment of B-ALL, and the potential usefulness of MXRA7 in B-ALL deserves further investigation.
Humans ; Apoptosis ; Cell Line, Tumor ; Cell Proliferation ; Cytarabine ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/metabolism*

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

BACKGROUND: The cytological examination of cerebrospinal fluid (CSF) using conventional cytology with a cytocentrifuge (cytospin) is an important method for evaluating the involvement of leukemia in the CNS. Liquid-based cytology (LBC) is now a widely used cytological method not only for gynecological and non-gynecological specimens, but its application to CSF for the identification of leukemic cell has not yet been reported. In this study, we tried to compare conventional cytology with using a cytospin with LBC and Papanicolaou (Pap) staining. We also examined the modified LBC with Wright staining to assess whether this modified method can be useful for diagnosing Leukemia. METHODS: We studied 30 cases of CSF that were obtained from 16 patients, including 17 cases of acute myeloid leukemia, 12 cases of acute lymphoblastic leukemia and 1 case of diffuse large B cell lymphoma. We applied conventional cytology with a cytocentrifuge (cytospin), LBC with Pap staining and modified LBC with Wright staining. RESULTS: The morphological features of the LBC with Pap staining showed difficulty for interpretation when compared with conventional cytology with a cytospin, and mainly because of cellular shrinkage. The modified LBC with Wright staining showed good morphological features. CONCLUSIONS: We suggest that modified LBC with Wright staining may be useful for examining CSF.
Humans ; Leukemia ; Leukemia, Myeloid, Acute ; Lymphoma, B-Cell ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

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

<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.

Therapy-related myeloid neoplasms have been well characterized. However, precursor B-cell acute lymphoblastic leukemia in patients with prior malignancies is uncommon, and the effect of prior cytotoxic therapy on development of precursor B-cell acute lymphoblastic leukemia is controversial. Therapy-related precursor B-cell acute lymphoblastic leukemia has been reported occasionally. However, cytotoxic therapy-related precursor B-cell acute lymphoblastic leukemia has been reported in Korea only rarely. We herein describe two cases of therapy-related precursor B-cell acute lymphoblastic leukemia.
Humans ; Korea ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cells, B-Lymphoid

We present a case of Korean pediatric patient with pre-B cell type acute lymphoblastic leukemia (ALL) with trisomy 5 as a sole cytogenetic anomaly. Here, we compare and describe the present case with previous pediatric case reports and provide a review of the literature. This case report may help elucidate the poor prognostic impact of trisomy 5 as a sole cytogenetic anomaly in pediatric patients with ALL. Additional studies are needed to confirm this hypothesis.
Cytogenetics ; Humans ; Pediatrics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cells, B-Lymphoid ; Prognosis ; Trisomy