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Childhood acute lymphoblastic leukemia with hyperleukocytosis at presentation.

Seom Gim KONG ; Jung Ho SEO ; So Eun JUN ; Byung Ki LEE ; Young Tak LIM

Blood Research.2014;49(1):29-35. doi:10.5045/br.2014.49.1.29

BACKGROUND: Hyperleukocytosis caused by acute lymphoblastic leukemia (ALL) is associated with early morbidity and mortality due to hyperviscosity arising from the excessive number of leukocytes.This study was designed to assess the incidence of hyperleukocytosis, survival outcomes, and adverse features among pediatric ALL patients with hyperleukocytosis. METHODS: Between January 2001 and December 2010, 104 children with previously untreated ALL were enrolled at the Pusan National University Hospital. All of them were initially stratified based on the National Cancer Institute (NCI) risk; 48 (46.2%) were diagnosed with high-risk ALL. The medical charts of these patients were retrospectively reviewed. RESULTS: Twenty (19.2%) of the 104 children with ALL had initial leukocyte counts of >100x10(9)/L, and 11 patients had a leukocyte count of >200x10(9)/L. Male gender, T-cell phenotype, and massive splenomegaly were positively associated with hyperleukocytosis. Common early complications during induction therapy included renal dysfunction, and central nervous system hemorrhage. The complete remission (CR) rate for the pediatric ALL patients with hyperleukocytosis (94.1%) was similar to the overall CR rate (95.6%). The estimated 3-year event free survival (EFS) and overall survival of ALL children with hyperleukocytosis were 75.0% and 81.2%, respectively. However, patients with initial leukocyte counts >200x10(9)/L had a lower EFS than those with initial leukocyte counts 100-200x109/L (63.6% vs. 100%; P=0.046). CONCLUSION: The outcome of pediatric ALL cases with an initial leukocyte count >200x10(9)/L was very poor, probably due to early toxicity-related death during induction therapy.
Busan ; Central Nervous System ; Child ; Disease-Free Survival ; Hemorrhage ; Humans ; Incidence ; Leukocyte Count ; Male ; Mortality ; National Cancer Institute (U.S.) ; Phenotype ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Retrospective Studies ; Splenomegaly ; T-Lymphocytes

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Characteristics of hematologic malignancies with coexisting t(9;22) and inv(16) chromosomal abnormalities.

Eunhee HAN ; Hyeyoung LEE ; Myungshin KIM ; Yonggoo KIM ; Kyungja HAN ; Sung Eun LEE ; Hee Je KIM ; Dong Wook KIM

Blood Research.2014;49(1):22-28. doi:10.5045/br.2014.49.1.22

BACKGROUND: The coexistence of t(9;22)(q34;q11.2) and inv(16)(p13q22) chromosomal abnormalities is extremely uncommon, and only a small number of such cases have been reported. Here, we characterized 7 cases of hematologic malignancy exhibiting t(9;22) and inv(16) coexistence. METHODS: We reviewed the cytogenetic data for hematologic malignancies treated at the Catholic Blood and Marrow Transplantation Center between January 2004 and June 2013. We identified 7 cases exhibiting t(9;22) and inv(16) coexistence. In addition, we analyzed mutations in the IKZF1, NPM1, FLT3, N-RAS, K-RAS, c-KIT, and TP53 genes. RESULTS: Four cases of chronic myelogenous leukemia (CML; 1 chronic phase, 2 accelerated phase, and 1 blast phase) and 3 cases of acute myeloid leukemia (AML; 1 de novo and 2 therapy-related) were identified. The percentages of circulating blasts and bone marrow eosinophils were higher in AML cases than in CML cases (53% vs. 5% and 30% vs. 5.5%, respectively). The proportions of each chromosomal abnormality were used along with follow-up karyotyping results to identify secondary changes. In BCR/ABL, a p210 fusion transcript was associated with CML, whereas a p190 fusion transcript was associated with AML. One patient with AML harbored 2 mutations: c-KIT D816V and TP53 E11Q. All patients except 1 with CML blast phase sustained clinical remission after treatment, which included an imatinib mesylate regimen. CONCLUSION: This study shows that observations of bone marrow morphology, initial and follow-up cytogenetic studies, and karyotyping of BCR/ABL1 and CBFB/MYH11 provide valuable information for characterizing hematologic malignancies exhibiting t(9;22) and inv(16) coexistence.
Blast Crisis ; Bone Marrow ; Chromosome Aberrations* ; Cytogenetics ; Eosinophils ; Follow-Up Studies ; Genes, p53 ; Hematologic Neoplasms* ; Humans ; Karyotyping ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Mesylates ; Imatinib Mesylate

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Clinical features and treatment outcomes in patients with mantle cell lymphoma in Korea: Study by the Consortium for Improving Survival of Lymphoma.

Byung Woog KANG ; Sang Kyun SOHN ; Joon Ho MOON ; Yee Soo CHAE ; Jong Gwang KIM ; Soo Jung LEE ; Won Seog KIM ; Je Jung LEE ; Se Ryeon LEE ; Keon Uk PARK ; Ho Sup LEE ; Won Sik LEE ; Jong Ho WON ; Moo Rim PARK ; Jae Yong KWAK ; Min Kyoung KIM ; Hyo Jung KIM ; Sung Yong OH ; Hye Jin KANG ; Cheolwon SUH

Blood Research.2014;49(1):15-21. doi:10.5045/br.2014.49.1.15

BACKGROUND: We investigated the clinical features and treatment outcomes of patients with mantle cell lymphoma (MCL) in Korea. METHODS: We retrospectively analyzed the clinical characteristics and prognosis of 131 patients diagnosed with MCL between January 2004 and December 2009 at 15 medical centers in Korea; all patients received at least 1 chemotherapeutic regimen for MCL. RESULTS: The median age for the patients was 63 years (range, 26-78 years), and 77.9% were men. A total of 105 patients (80.1%) had stage III or IV MCL at diagnosis. Fifty-two patients (39.7%) were categorized with high- or high-intermediate risk MCL according to the International Prognostic Index (IPI). Eighteen patients (13.7%) were in the high-risk group according to the simplified MCL-IPI (MIPI). The overall incidence of extranodal involvement was 69.5%. The overall incidence of bone marrow and gastrointestinal involvements at diagnosis was 41.2% and 35.1%, respectively. Cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab were used frequently as the first-line treatment (41.2%). With a median follow-up duration of 20.0 months (range, 0.2-77.0 months), the overall survival (OS) at 2 years was 64.7%, while the event-free survival (EFS) was 39.7%. Multivariate analysis showed that the simplified MIPI was significantly associated with OS. However, the use of a rituximab-containing regimen was not associated with OS and EFS. CONCLUSION: Similar to results from Western countries, the current study found that simplified MIPI was an important prognostic factor in Korean patients with MCL.
Bone Marrow ; Cyclophosphamide ; Diagnosis ; Disease-Free Survival ; Doxorubicin ; Drug Therapy ; Epidemiology ; Follow-Up Studies ; Humans ; Incidence ; Korea* ; Lymphoma* ; Lymphoma, Mantle-Cell* ; Male ; Multivariate Analysis ; Prednisolone ; Prognosis ; Retrospective Studies ; Vincristine ; Rituximab

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Disease modeling and cell based therapy with iPSC: future therapeutic option with fast and safe application.

Changsung KIM

Blood Research.2014;49(1):7-14. doi:10.5045/br.2014.49.1.7

Induced pluripotent stem cell (iPSC) technology has shown us great hope to treat various human diseases which have been known as untreatable and further endows personalized medicine for future therapy without ethical issues and immunological rejection which embryonic stem cell (hES) treatment has faced. It has been agreed that iPSCs knowledge can be harnessed from disease modeling which mimics human pathological development rather than trials utilizing conventional rodent and cell lines. Now, we can routinely generate iPSC from patient specific cell sources, such as skin fibroblast, hair follicle cells, patient blood samples and even urine containing small amount of epithelial cells. iPSC has both similarity and dissimilarity to hES. iPSC is similar enough to regenerate tissue and even full organism as ES does, however what we want for therapeutic advantage is limited to regenerated tissue and lineage specific differentiation. Depending on the lineage and type of cells, both tissue memory containing (DNA rearrangement/epigenetics) and non-containing iPSC can be generated. This makes iPSC even better choice to perform disease modeling as well as cell based therapy. Tissue memory containing iPSC from mature leukocytes would be beneficial for curing cancer and infectious disease. In this review, the benefit of iPSC for translational approaches will be presented.
Cell Line ; Communicable Diseases ; Embryonic Stem Cells ; Epithelial Cells ; Ethics ; Fibroblasts ; Hair Follicle ; Hope ; Humans ; Precision Medicine ; Leukocytes ; Memory ; Pluripotent Stem Cells ; Rodentia ; Skin ; Stem Cells ; Transplants