1.Familial Hemophagocytic Lymphohistiocytosis Type 2 in a Korean Infant With Compound Heterozygous PRF1 Defects Involving a PRF1 Mutation, c.1091T>G.
Min Sun KIM ; Young Uk CHO ; Seongsoo JANG ; Eul Ju SEO ; Ho Joon IM ; Chan Jeoung PARK
Annals of Laboratory Medicine 2017;37(2):162-165
No abstract available.
Asian Continental Ancestry Group/*genetics
;
Base Sequence
;
Bone Marrow Cells/cytology/pathology
;
Cytomegalovirus Infections/diagnosis
;
Epstein-Barr Virus Infections/diagnosis
;
Female
;
Flow Cytometry
;
Heterozygote
;
Humans
;
Infant
;
Killer Cells, Natural/cytology/immunology
;
Lymphohistiocytosis, Hemophagocytic/*diagnosis/genetics
;
Perforin/*genetics
;
Phagocytosis
;
Polymorphism, Single Nucleotide
;
Republic of Korea
;
Sequence Analysis, DNA
2.Bone Marrow Suppression and Hemophagocytic Histiocytes Are Common Findings in Korean Severe Fever with Thrombocytopenia Syndrome Patients.
Sang Yong SHIN ; Oh Hyun CHO ; In Gyu BAE
Yonsei Medical Journal 2016;57(5):1286-1289
The causes of cytopenia in patients with severe fever with thrombocytopenia syndrome (SFTS) are not fully understood until now. We reviewed the bone marrow (BM) findings of patients with SFTS to unravel the cause of the cytopenia. Three Korean SFTS were enrolled in this study. Thrombocytopenia, neutropenia, and anemia were detected in all three patients. Severe hypocellular marrow (overall cellularity <5%) and a decreased number of megakaryocytes were noted in one patient, and hypo-/normocellular marrow and an increased number of hemophagocytic histiocytes were observed in two patients. Megakaryocytes were relatively preserved in two patients. Although a limited number of cases are available, our observations suggest that both BM suppression and peripheral destruction or sequestration are causes of cytopenia of patients with SFTS. To the best of our knowledge, this is the first well documented pathologic evaluation of Korean SFTS.
Aged
;
Aged, 80 and over
;
Bone Marrow/*pathology
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Female
;
Fever/*complications
;
Histiocytes/*pathology
;
Humans
;
Male
;
Middle Aged
;
Neutropenia/complications
;
Pancytopenia/complications
;
Syndrome
;
Thrombocytopenia/*complications/*immunology
3.First Report on Familial Hemophagocytic Lymphohistiocytosis with an Abnormal Immunophenotype and T Cell Monoclonality in Korea.
Sang Yong SHIN ; Kyunghoon LEE ; Mi Ae JANG ; Seung Tae LEE ; Keon Hee YOO ; Hong Hoe KOO ; Dae Shick KIM ; Hee Jin KIM ; Sun Hee KIM
Annals of Laboratory Medicine 2015;35(1):155-158
No abstract available.
Bone Marrow/metabolism/pathology
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DNA Mutational Analysis
;
Gene Rearrangement, T-Lymphocyte
;
Humans
;
Immunophenotyping
;
Infant
;
Lymphohistiocytosis, Hemophagocytic/*diagnosis
;
Male
;
Membrane Proteins/chemistry/genetics
;
Polymorphism, Single-Stranded Conformational
;
Republic of Korea
;
T-Lymphocytes/immunology/*metabolism
4.The First Case Report of Composite Bone Marrow Involvement by Simultaneously Developed Peripheral T-Cell Lymphoma, Not Otherwise Specified, and Diffuse Large B-Cell Lymphoma.
Hyun Ki KIM ; Chan Jeoung PARK ; Seongsoo JANG ; Young Uk CHO ; Sang Hyuk PARK ; Jene CHOI ; Chan Sik PARK ; Jooryung HUH ; Young Hwa CHUNG ; Jung Hee LEE
Annals of Laboratory Medicine 2015;35(1):152-154
No abstract available.
Antibodies, Monoclonal, Murine-Derived/therapeutic use
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Antineoplastic Combined Chemotherapy Protocols/therapeutic use
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B-Cell-Specific Activator Protein/metabolism
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Bone Marrow/metabolism/*pathology
;
Cyclophosphamide/therapeutic use
;
Doxorubicin/therapeutic use
;
Endoscopy, Digestive System
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Female
;
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
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Genetic Loci
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Humans
;
Liver/metabolism/pathology
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Lymphocytes/cytology/immunology
;
Lymphoma, Large B-Cell, Diffuse/complications/*diagnosis/drug therapy
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Lymphoma, T-Cell, Peripheral/complications/*diagnosis/drug therapy
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Middle Aged
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Prednisone/therapeutic use
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Receptors, Antigen, T-Cell, gamma-delta/genetics
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Tomography, X-Ray Computed
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Vincristine/therapeutic use
5.Bone Marrow Flow Cytometry in Staging of Patients With B-cell Non-Hodgkin Lymphoma.
Borahm KIM ; Seung Tae LEE ; Hee Jin KIM ; Sun Hee KIM
Annals of Laboratory Medicine 2015;35(2):187-193
BACKGROUND: Bone marrow biopsies are routinely performed for staging patients with B-cell non-Hodgkin lymphoma (NHL). In addition to histomorphological studies, ancillary tools may be needed for accurate diagnosis. We investigated the clinical utility of multiparameter flow cytometric examination of bone marrow aspirates. METHODS: A total of 248 bone marrow specimens from 232 patients diagnosed with B-cell NHL were examined. Monoclonal antibodies directed against CD19, CD20, CD10 (or CD5), and kappa and lambda immunoglobulins were used. Multi-stage sequential gating was performed to select specific cells of interest, and the results were compared with bone marrow histology. RESULTS: The concordance rate between histomorphology and flow cytometry was 91.5% (n=227). Eight cases (3.2%) were detected by flow cytometry alone and were missed by histomorphology analysis, and 6 of these 8 cases showed minimal bone marrow involvement (0.09-2.2%). The diagnosis in these cases included large cell lymphoma (n=3), mantle cell lymphoma (n=3), and mucosa-associated lymphoid tissue (MALT) lymphoma (n=2). Thirteen cases were histopathologically positive and immunophenotypically negative, and the diagnoses in these cases included diffuse large cell lymphoma (n=7), T-cell/histiocyte-rich large B-cell lymphoma (n=2), anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma (n=1), follicular lymphoma (n=1), MALT lymphoma (n=1), and unclassifiable lymphoma (n=1). CONCLUSIONS: Multi-color flow cytometry can be a useful method for assessing bone marrow in staging NHL and also plays a complementary role, especially in detecting small numbers of lymphoma cells.
Antibodies, Monoclonal/immunology
;
Antigens, CD19/immunology/metabolism
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Antigens, CD20/immunology/metabolism
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Bone Marrow/*pathology
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Female
;
Flow Cytometry
;
Humans
;
Immunophenotyping
;
Lymphoma, B-Cell/*pathology
;
Male
;
Neoplasm Staging
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Neprilysin/immunology/metabolism
6.Effects of dendritic cell-activated and cytokine-induced killer cell therapy on 22 children with acute myeloid leukemia after chemotherapy.
Yan BAI ; Jin-e ZHENG ; Nan WANG ; He-hua CAI ; Li-na ZHAI ; Yao-hui WU ; Fang WANG ; Run-ming JIN ; Dong-feng ZHOU
Journal of Huazhong University of Science and Technology (Medical Sciences) 2015;35(5):689-693
The efficiency of dendritic cell-activated and cytokine-induced killer cell (DC-CIK) therapy on children with acute myeloid leukemia (AML) after chemotherapy was investigated. Mononuclear cells were collected from children achieving complete remission after chemotherapy, cultured in vitro and transfused back into the same patient. Interleukin-2 (IL-2) was injected subcutaneously every other day 10 times at the dose of 1 × 10(6) units. Peripheral blood lymphocyte subsets and minimal residual disease (MRD) were detected by flow cytometry. Function of bone marrow was monitored by methods of morphology, immunology, cytogenetics and molecular biology. The side effects were also observed during the treatment. The average follow-up period for all the 22 patients was 71 months and relapse occurred in two AML patients (9.1%). The percentage of CD3(+)/CD8(+) cells in peripheral blood of 15 patients at the 3rd month after DC-CIK treatment (36.73% ± 12.51%) was dramatically higher than that before treatment (29.20% ± 8.34%, P < 0.05). The MRD rate was >0.1% in 5 patients before the treatment, and became lower than 0.1% 3 months after the treatment. During the transfusion of DC-CIK, side effects including fever, chills and hives appeared in 7 out of 22 (31.82%) cases but disappeared quickly after symptomatic treatments. There were no changes in electrocardiography and liver-renal functions after the treatment. MRD in children with AML can be eliminated by DC-CIK therapy which is safe and has fewer side effects.
Adolescent
;
Antineoplastic Agents
;
therapeutic use
;
Bone Marrow
;
drug effects
;
immunology
;
pathology
;
Child
;
Child, Preschool
;
Cytokine-Induced Killer Cells
;
cytology
;
immunology
;
transplantation
;
Dendritic Cells
;
cytology
;
immunology
;
transplantation
;
Female
;
Humans
;
Immunotherapy, Adoptive
;
methods
;
Injections, Subcutaneous
;
Interleukin-2
;
therapeutic use
;
Leukemia, Myeloid, Acute
;
immunology
;
pathology
;
therapy
;
Male
;
Neoplasm, Residual
;
Primary Cell Culture
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Recurrence
;
Remission Induction
;
Treatment Outcome
7.Differential analysis of BM cell morphology, immunophenotypic, cytogenetic characters and prognosis between myeloblastic and lymphoblastic crisis of CML.
Ya-Lin LIU ; Xiao-Ning WANG ; Hua-Sheng LIU
Journal of Experimental Hematology 2014;22(3):629-633
This study was purposed to investigate the difference of morphology, immunophenotype, cytogenetic features and prognosis between myeloid blast crisis and lymphoid blast crisis of chronic myelogenous leukemia (CML). A total of 31 patients with CML in blastic crisis in Department of Hematology, the First Affiliated Hospital of Xi'an Jiaotong University school of Medicine from 2009 January to 2014 January were enrolled in this study. Out of 31 CML patients, 24 cases were patients with myeloid blast crisis and other 7 cases were patients with lymphoblastic crisis. The clinical data, blast cell percentage in peripheral blood and bone marrow, eosinophil and basophil percentage, immunophenotype, cytogenetic characteristics and prognosis were analyzed. The results indicated that there was no significant difference of blastic cell percentage in peripheral blood and bone marrow of CML with myeloid blast crisis, and the eosinophil and basophil cells could be easily detected. The ratio of blastic cells in BM was higher than that in PB in lymphoid blastic crisis of CML, eosinophil and basophil cells were rare. 7 cases of CML with lymphoid blastic crisis were B ALL with CD10, CD19, CD34, HLA-DR expression, and 2 cases with CD13 and CD33 expression. The lymphoid score was in all CML patients with lymphoid blastic crisis was greater than or equal to 1.5;and 2 patients with CD13 and CD33 expression, and with 1 myeloid score.24 cases of myeloid blastic crisis of CML patients mainly expressed CD33, CD13, CD38, CD34, CD11b and HLA-DR, and their myeloid score greater than or equal to 2, among them the lymphoid scores of 2 patients were 0.5 and 1 score, respectively. All the 31 patients showed 100% Ph(+) chromosome, among them 3 cases also showed other new chromosome aberrations. There was no significant difference of overall survival rate between lymphoid and myeloid blastic crisis of CML, but the overall survival rate of patients treated with tyrosine kinase inhibitor (TKI ) was higher than that in the patients without TKI treatment. It is concluded that eosinophil and basophil cells in peripheral blood of lymphoid blastic crisis were less than that of CML patients with myeloid blastic crisis. Lymphoid blastic crisis of CML patients occurred mostly in B ALL cases with expression of CD10 and CD19. Patients with myeloid blastic crisis of CML mainly expressed CD33, CD13, CD38, CD34, CD11b and HLA-DR, and could be accompanied by other lineage antigen expression, but the score was less than 2. New chromosome aberration is easily observed in myeloid blastic crisis of CML. There is no significant difference of overall survival rate of between CML patients with lymphoid and myeloid blastic crisis, but the overall survival rate of patients treated with TKI is higher than the patients without TKI treatment.
Adolescent
;
Adult
;
Aged
;
Blast Crisis
;
Bone Marrow Cells
;
immunology
;
pathology
;
Cytogenetic Analysis
;
Female
;
Humans
;
Immunophenotyping
;
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
;
drug therapy
;
pathology
;
Male
;
Middle Aged
;
Precursor Cell Lymphoblastic Leukemia-Lymphoma
;
drug therapy
;
pathology
;
Prognosis
;
Protein Kinase Inhibitors
;
therapeutic use
;
Protein-Tyrosine Kinases
;
antagonists & inhibitors
;
Retrospective Studies
;
Survival Rate
;
Young Adult
8.Changes of CD34(+) and CD71(+)CD45(-) cell levels in bone marrow of MDS and AA patients.
Zhen-Yu YAN ; Xu TIAN ; Ying LI ; Mei-Rong YANG ; Song ZHANG ; Xie-Ming WANG ; Hai-Xia ZHANG ; Nai-Yao CHENG
Journal of Experimental Hematology 2014;22(2):382-386
This study was aimed to investigate the changes of CD34(+) and CD71(+)CD45(-) cell levels in MDS and AA patients. A total of 25 cases MDS and 43 cases of AA (18 cases SAA and 25 cases of NSAA) from January 2010 to October 2013 in the Department of Hematology, affiliated hospital of Hebei United University were enrolled in this study. The complete blood count, bone marrow smears, bone marrow biopsy, karyotype analysis and bone marrow blood cell immune genotyping (mainly the proportion of CD34(+) cells, CD71(+)CD45(-) cells in nucleated cells) were carried out for all patients; the changes of CD34(+) and CD71(+)CD45(-) cell levels in patients with MDS and AA (SAA NSAA) were compared; the differences of white blood cell count, platelet count and hemoglobin concentration in patients with count of CD71(+)CD45(-) ≥ 15% or <15% were analyzed. The results showed that the count of CD34(+) in MDS group was higher than that in AA (NSAA and SAA) group (P < 0.05). The count of CD71(+)CD45(-) cells in MDS group was higher than that in SAA (P < 0.05), there was no significant difference between NSAA group and MDS group. In MDS group with CD71(+)CD45(-) ≥ 15%, the platelet count was significantly higher than that in NSAA group (P < 0.05); and there was no statistical difference for leukocyte, platelet count and hemoglobin level between MDS and NSAA group with CD71(+)CD45(-) <15% (P > 0.05). It is concluded that the count of CD34(+) cells in MDS patients is significantly higher than that in AA and SAA patients. The count of CD71(+)CD45(-) cells in MDS group is significantly higher than that of SAA group. The platelet count in MDS patients with CD71(+)CD45(-) cells ≥ 15% is significantly higher than that of the NSAA group.
Adolescent
;
Adult
;
Aged
;
Aged, 80 and over
;
Anemia, Aplastic
;
pathology
;
Antigens, CD
;
immunology
;
Antigens, CD34
;
immunology
;
Blood Cell Count
;
Bone Marrow
;
Bone Marrow Cells
;
cytology
;
immunology
;
Female
;
Flow Cytometry
;
Humans
;
Leukocyte Common Antigens
;
immunology
;
Male
;
Middle Aged
;
Myelodysplastic Syndromes
;
pathology
;
Receptors, Transferrin
;
immunology
;
Young Adult
9.The protective role of myeloid-derived suppressor cells in concanavalin A-induced hepatic injury.
Wenli DIAO ; Fangfang JIN ; Bing WANG ; Chen-Yu ZHANG ; Jiangning CHEN ; Ke ZEN ; Limin LI
Protein & Cell 2014;5(9):714-724
The mechanism underlying T cell-mediated fulminant hepatitis is not fully understood. In this study, we investigated whether myeloid derived suppressor cells (MDSCs) could prevent the concanavalin A (ConA)-induced hepatitis through suppressing T cell proliferation. We observed an increase in the frequencies of MDSCs in mouse spleen and liver at early stage of ConA treatment, implicating that the MDSCs might be involved in the initial resistance of mice against ConA-mediated inflammation. Subpopulation analysis showed that the MDSCs in liver of ConA-induced mice were mainly granulocytic MDSCs. Adoptive transfer of the bone marrow-derived MDSCs into ConA-treated mice showed that the MDSCs migrated into the liver and spleen where they suppressed T cell proliferation through ROS pathway. In addition, the frequencies of MDSCs in mice were also significantly increased by the treatment with immune suppressor glucocorticoids. Transfer of MDSCs into the regulatory T cell (Treg)-depleted mice showed that the protective effect of MDSCs on ConA-induced hepatitis is Treg-independent. In conclusion, our results demonstrate that MDSCs possess a direct protective role in T cell-mediated hepatitis, and increasing the frequency of MDSCs by either adoptive transfer or glucocorticoid treatment represents a potential cell-based therapeutic strategy for the acute inflammatory disease.
Adoptive Transfer
;
Animals
;
Blotting, Western
;
Bone Marrow Cells
;
immunology
;
CD11b Antigen
;
immunology
;
metabolism
;
Cell Movement
;
immunology
;
Cell Proliferation
;
Chemical and Drug Induced Liver Injury
;
etiology
;
immunology
;
prevention & control
;
Concanavalin A
;
toxicity
;
Dexamethasone
;
pharmacology
;
Flow Cytometry
;
Glucocorticoids
;
pharmacology
;
Liver
;
immunology
;
pathology
;
Male
;
Mice, Inbred C57BL
;
Mitogens
;
administration & dosage
;
toxicity
;
Myeloid Cells
;
immunology
;
metabolism
;
transplantation
;
Receptors, Chemokine
;
immunology
;
metabolism
;
Spleen
;
immunology
;
pathology
;
T-Lymphocytes
;
immunology
;
T-Lymphocytes, Regulatory
;
immunology
10.The mechanisms underlying bone marrow damage by iron overload in pancytopenic patients with positive BMMNC-Coombs test.
Lei HUANG ; Rong FU ; Li-juan LI ; Hui LIU ; Yi-hao WANG ; Hong-lei WANG ; Tian ZHANG ; Kai DING ; Shao-xue DING ; Er-bao RUAN ; Wen QU ; Jing GUAN ; Guo-jin WANG ; Jia SONG ; Hua-quan WANG ; Yu-hong WU ; Li-min XING ; Hong LIU ; Xiao-ming WANG ; Zong-hong SHAO
Chinese Journal of Hematology 2013;34(5):430-434
OBJECTIVETo investigate the mechanisms underlying bone marrow damage by iron overload in pancytopenic patients with positive BMMNC-Coombs test (IRP).
METHODSTwenty-one iron overloading, 26 non-iron overloading IRP patients and 10 normal controls were enrolled in this study. The expressions of ROS, Bcl-2, Caspase-3 and apoptosis of BMMNC were analyzed by flow cytometry (FCM). Antioxidants were added to iron overloading IRP BMMNC, and then the changes of indices above were detected by FCM. The number and apoptosis of T lymphocytes of IRP patients were also detected.
RESULTSROS and apoptosis of BMMNC, myelocytes, erythrocytes and stem cells of iron overloading IRP patients were significantly higher than that of non-iron overloading IRP ones and normal controls (P < 0.05). The expressions of Bcl-2 on BMMNC, erythrocytes and stem cells of iron overloading IRP patients were significantly lower than those of non-iron overloading IRP ones (P < 0.05). The levels of Caspase-3 on myelocytes, erythrocytes and stem cells of iron overloading IRP patients were significantly higher than those of non-iron overloading IRP ones and normal controls (P < 0.05). After treatment with antioxidants, the expressions of ROS, Caspase-3 and apoptosis of iron overloading IRP BMMNC significantly decreased, but opposite for Bcl-2. The percentages of CD4(+) lymphocytes [ ( 40.86 ± 8.74)%] and CD4(+)/CD8(+) (1.44 ± 0.36) in PB of iron overloading IRP patients were significantly higher than that of non-iron overloading IRP ones [(35.96 ± 7.03)% and 1.14 ± 0.37] and normal controls [(28.00 ± 6.73)% and 0.79 ± 0.21], respectively (P < 0.05), as opposite for CD8(+) lymphocytes (P < 0.05). The apoptosis of CD8(+) lymphocytes [(27.35 ± 10.76)%] and the ratio of CD8(+) apoptosis/CD4(+) apoptosis (2.51 ± 0.81) in BM of iron overloading IRP patients were significantly higher than those of non-iron overloading IRP ones [(15.47 ± 8.99)%] and normal controls (1.39 ± 0.47), respectively (P < 0.05). The apoptosis of erythrocytes and stem cells coated with auto-antibodies in BM of iron overloading IRP patients were significantly higher than those of non-iron overloading IRP and normal controls.
CONCLUSIONMechanisms underlying bone marrow damage by iron overload might be through the follows: ①The increased ROS induced by excessive iron deposition affected the expressions of Caspase-3 and Bcl-2, which caused more BMMNC apoptosis; ②The abnormal number and ratio of T lymphocytes caused by iron overload aggravated the abnormality of immunity of IRP; ③Iron overload may increase the damage to erythrocytes and stem cells coated with auto-antibodies.
Adolescent ; Adult ; Aged ; Bone Marrow ; pathology ; Case-Control Studies ; Caspase 3 ; metabolism ; Coombs Test ; Female ; Humans ; Iron Overload ; Male ; Middle Aged ; Pancytopenia ; immunology ; pathology ; physiopathology ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Reactive Oxygen Species ; metabolism ; Young Adult

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