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

This study was purposed to detect the balance and the activity change of cytotoxic T cell subsets in aplastic anemia (AA) patients, myelodysplastic syndrome (MDS) patients and acute myeloid leukemia (AML) patients, and to explore the cellular immune mechanism for abnormal hematopoiesis of the three diseases, so as to provide experimental basis for the choice of clinical treatment. The proportion of the cytotoxic T cells and part of the T-cells subsets in peripheral blood were detected by flow cytometry in 35 cases of MDS, including 19 refractory anemia (MDS-RA), 16 refractory anemia with excess blasts (MDS-RAEB), 17 AA, 15 AML patients and 10 normal donors respectively. The results showed that compared with the control group, the percentage of Tc1, Tc1/Tc2, CD8(+)HLA-DR(+), CD3(+)CD8(+)CD28(+), CD8(+)CD45RO(+) cells was significantly higher and the percentage of CD8(+)CD45RA(+) was significantly lower in AA and MDS-RA group. There was no difference in the percentage of Tc2 cells between AA/MDS-RA and normal controls; the percentage of CD8(+)CD45RO(+) cells was significantly higher and the percentage of Tc1, CD3(+)CD8(+)CD28(+), CD8(+)HLA-DR(+) was significantly lower in MDS-RAEB group, the percentage of CD8(+)CD45RA(+) was lower but the difference was not significant, and there was no difference in the percentage of Tc, Tc1/Tc2 cells between MDS-RAEB group and the control group. The percentage of Tc2 cells was significantly higher and the percentage of other parameters was significantly lower in AML group than those of normal controls. It is concluded that the cellular immune statuses in AA, the different stages of MDS and AML are different. In AA and the early stage of MDS, the balance of Tc1/Tc2 shifts to Tc1, and the activation of T-cell subsets increases. In the late stage of MDS and AML, the balance of Tc1/Tc2 shifts to Tc2, the activation of T-cell subsets decreases. The former may be closely related to bone marrow failure while the latter may be one of the important mechanisms in which the malignant clones escape from immune effect.
Adolescent ; Adult ; Aged ; Anemia, Aplastic ; immunology ; pathology ; CD8-Positive T-Lymphocytes ; cytology ; Case-Control Studies ; Female ; Flow Cytometry ; Humans ; Leukemia, Myeloid, Acute ; immunology ; pathology ; Lymphocyte Count ; Male ; Middle Aged ; Myelodysplastic Syndromes ; immunology ; pathology ; Young Adult

Alteration in the balance between cell apoptosis and proliferation is one of the pathophysiological mechanisms of the myelodysplastic syndromes (MDS). The question of whether the excessive apoptosis and/or proliferation predominantly involve the subset of progenitor cells (CD34(+) cells) or mature cells (CD34(-) cells) remains a controversial issue. This study was purpose to analyze the apoptosis and proliferation status of CD34(+) and CD34(-) cells in bone marrow (BM) of patients with MDS, to investigate the pathogenesis of MDS and to determine the relation of apoptosis and proliferation status of CD34(+) and CD34(-) cells with prognosis of MDS. The proportion of CD34(+) cells, the apoptosis and proliferation ratio (A/P) of CD34(+) and CD34(-) cells in BM of 40 patients with MDS, including 20 cases of high-risk MDS and 20 cases of low-risk MDS, and 10 normal persons as control were detected by flow cytometry; the influence of CD34(+) and CD34(-) cell apoptosis and proliferation levels on prognosis of MDS was evaluated by univariate and multivariate analysis of survival. The results showed that the proportion of CD34(+) cells in BM of high-risk MDS patients was significantly higher than that in BM of low-risk MDS patients and in normal BM [(1.92 ± 0.10)%, (1.09 ± 0.04)%, (1.03 ± 0.05)% respectively]. The apoptotic rates (AR) of both CD34(+) and CD34(-) cells were significantly higher in low-risk MDS [(54.75 ± 2.18)%, (80.36 ± 1.68)%] than in high-risk MDS [(24.87 ± 2.69)%, (23.12 ± 1.23)%] and in normal BM [(18.51 ± 2.74)%, (20.98 ± 2.21)%]. When compared between CD34(+) cells and CD34(-) cells in low-risk MDS, a greater AR of CD34(-) cells was found. However, the higher proliferative rate of CD34(+) cells was observed in high-risk MDS. In low-risk MDS, a higher A/P ratio was found in CD34(-) cells than in CD34(+) cells; whereas this ratio was equalized or inverted in high-risk MDS. In addition, the survival and prognosis correlated significantly with AR of CD34(+) cells. It is concluded that the early MDS is predominantly associated with excessive apoptosis of the mature CD34(-) cells. The proliferation rate of cells increases with the disease progression in MDS subsets, especially, in the subset of CD34(+) cells. Surprisingly, the apoptosis of CD34(+) cells may be a useful prognostic factor, and the inhibition of apoptotic mechanisms may induce the transformation of MDS to leukemia.
Adolescent ; Adult ; Aged ; Antigens, CD34 ; Apoptosis ; Bone Marrow Cells ; immunology ; pathology ; Case-Control Studies ; Cell Proliferation ; Child ; Female ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes ; mortality ; pathology ; Prognosis ; Survival Rate ; Young Adult

We report here a case of inguinal sparganosis, initially regarded as myeloid sarcoma, diagnosed in a patient undergone allogeneic hematopoietic transplantation (HSCT). A 56-year-old male patient having myelodysplastic syndrome was treated with allogeneic HSCT after myeloablative conditioning regimen. At day 5 post-HSCT, the patient complained of a painless palpable mass on the left scrotum and inguinal area. Pelvic magnetic resonance imaging and computed tomography revealed suspected myeloid sarcoma. Gun-biopsy was performed, and the result revealed eosinophilic infiltrations without malignancy. Subsequent serologic IgG antibody test was positive for sparganum. Excisional biopsy as a therapeutic diagnosis was done, and the diagnosis of sparganosis was confirmed eventually. This is the first report of sparganosis after allogeneic HSCT mimicking myeloid sarcoma, giving a lesson that the physicians have to consider the possibility of sparganosis in this clinical situation and perform adequate diagnostic and therapeutic approaches.
Animals ; Diagnosis, Differential ; Hematopoietic Stem Cell Transplantation ; Humans ; Larva ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Myelodysplastic Syndromes/complications/surgery ; Republic of Korea ; Sarcoma, Myeloid/diagnosis ; Scrotum/parasitology/*pathology ; Sparganosis/parasitology/*pathology/radiography ; Sparganum/*immunology/isolation & purification ; Tomography, X-Ray Computed ; Transplantation, Homologous

This study was purposed to analyse the characteristics of blast immunophenotype in patients with refractory anemia with excess blasts, type 1 (RAEB-1) and refractory anemia with excess blasts, type 2 (RAEB-2) by flow cytometry and investigate its diagnostic significance, as well as to compare the blast rate detected by FCM and bone marrow smear (BMS). FCM was used to count the blast rate and detect the expression of its antigens in 29 cases of MDS. The result indicated that: (1) The rate of the blasts detected by FCM and BMS was not statistically significant between patients with RAEB-1 and with RAEB-2 (P > 0.05); (2) Out of 13 patients with RAEB-1, the blasts of 10 cases, 12 cases, 8 cases, 11 cases, 11 cases, 3 cases, 7 cases, 0 case, 0 case, 3 cases and 2 cases patients had positive expressions of CD34, HLA-DR, CD117, CD33, CD13, CD15, CD11b, CD3, CD19, CD7 and CD56, separately. The blasts of 12 cases, 13 cases, 3 cases, 12 cases, 15 cases, 7 cases, 5 cases, 0 case, 1 case, 4 cases and 2 cases among 16 patients with RAEB-2 positively expressed CD34, HLA-DR, CD117, CD33, CD13, CD15, CD11b, CD3, CD19, CD7 and CD56, respectively. However, there was no significant difference in the expression of antigens in blasts of the bone marrow between the patients with RAEB-1 and with RAEB-2 (P > 0.05); (3) The positive expression for CD15 and CD11b in blasts was found in 10 cases and 12 cases, respectively; (4) The positive expression for CD19, CD7 and CD56 was observed in 1, 7 and 4 cases, respectively. None of the 29 cases of MDS was positive for CD3. It is concluded that FCM can reveal the characteristics of immunophenotypic abnormalities of the blasts in patients with MDS and provide the important information for diagnosis and differential diagnosis of MDS.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Bone Marrow ; pathology ; Female ; Flow Cytometry ; Humans ; Immunophenotyping ; Male ; Middle Aged ; Myelodysplastic Syndromes ; diagnosis ; immunology ; pathology ; Prognosis ; Young Adult

Mycobacteruim kansasii occasionally causes disseminated infection with poor outcome in immunocompromised patients. We report the first case of disseminated M. kansasii infection associated with multiple skin lesions in a 48-yr-old male with myelodysplastic syndrome. The patient continuously had taken glucocorticoid during 21 months and had multiple skin lesions developed before 9 months without complete resolution until admission. Skin and mediastinoscopic paratracheal lymph node (LN) biopsies showed necrotizing granuloma with many acid-fast bacilli. M. kansasii was cultured from skin, sputum, and paratracheal LNs. The patient had been treated successfully with isoniazid, rifampin, ethmabutol, and clarithromycin, but died due to small bowel obstruction. Our case emphasizes that chronic skin lesions can lead to severe, disseminated M. kansasii infection in an immunocompromised patient. All available cases of disseminated M. kansasii infection in non HIV-infected patients reported since 1953 are comprehensively reviewed.
Antitubercular Agents/therapeutic use ; Clarithromycin/therapeutic use ; Glucocorticoids/therapeutic use ; Humans ; Immunocompromised Host ; Isoniazid/therapeutic use ; Male ; Middle Aged ; Mycobacterium Infections, Nontuberculous/*diagnosis/drug therapy/immunology ; *Mycobacterium kansasii/isolation & purification ; Myelodysplastic Syndromes/drug therapy ; Rifampin/therapeutic use ; Skin Diseases, Bacterial/*diagnosis/immunology/pathology ; Sputum/microbiology ; Sweet Syndrome/diagnosis

BACKGROUND: In patients with isolated thrombocytopenia, but without significant dysplasia, diagnosis of idiopathic thrombocytopenic purpura (ITP) rather than myelodysplastic syndrome (MDS) may be taken into account. It is important to make an accurate diagnosis because different treatments are used for ITP and MDS. The purpose of this study was to investigate the clinical and hematologic features of patients who were initially diagnosed as ITP but had cytogenetic abnormalities. METHODS: We retrospectively reviewed cytogenetic studies of 100 patients who were diagnosed as ITP from 2004 to 2009 at Mokdong Hospital of Ewha Womans University based on clinical features and hematologic studies. Bone marrow pathology was re-evaluated based on 2008 WHO classification. Cytogenetic analysis was performed by 24-48 hr culture of bone marrow aspirates without using mitogens and 20 metaphases were analyzed. RESULTS: Of the 100 patients diagnosed as ITP initially, three patients (3%) had cytogenetic abnormalities. They had no thrombocytopenia-related symptoms and thrombocytopenia was found accidentally. The numbers of megakaryocytes in bone marrow were increased and dysplasia was not found in megakaryocyte, erythroid, and myeloid cell lineages. The proportion of blasts was within normal limits. Clonal chromosomal abnormalities found were der(1;7)(q10;p10), add(9)(q12), or t(7;11)(p22;q12). Presumptive diagnosis of MDS or diagnosis of idiopathic cytopenia of undetermined significance (ICUS) was made according to 2008 WHO classification. During the follow up, disease progression was not found. CONCLUSIONS: In patients with suspected ITP, cytogenetic analysis should be done. If specific clonal chromosomal abnormality is found, presumptive diagnosis of MDS has to be considered and close follow up is needed.
Adult ; Bone Marrow Cells/cytology ; Cell Lineage ; Chromosome Aberrations ; Diagnosis, Differential ; Female ; Humans ; Male ; Megakaryocytes/immunology/pathology ; Middle Aged ; Myelodysplastic Syndromes/*diagnosis/genetics/pathology ; Purpura, Thrombocytopenic, Idiopathic/*diagnosis/genetics/pathology ; Retrospective Studies

The study was aimed to investigate the abnormality of immunophenotypes in patients with myelodysplastic syndrome (MDS) and its role in the identification of MDS. The cell immunophenotypes of 136 patients with hypocytosis accompanied by abnormal hematopoiesis of bone marrow were detected by flow cytometry, the detected results were evaluated by flow cytometric scoring system (FCSS), and the sensitivity and specificity of positive results were determined by FCSS also. The correlation of results detected by FCSS to traditional diagnosis method was analysed. The results indicated that 111 out of 136 cases were diagnosed as MDS, and 25 were diagnosed as non-MDS. Among 111 MDS cases, 85 cases were FCSS positive, 18 cases were FCSS intermediate and 8 cases were FCSS negative, whereas in 25 non-MDS cases 24 cases were FCSS negative, 1 case was FCSS intermediate and no case was FCSS positive. The sensitivity of FCSS in identification of MDS was 76.6%, and the specificity of FCSS was 100%. There was a good correlation of FCSS to traditional method (R = 0.613, p = 0.000). It is concluded that the various abnormalities of immunophenotyping are found in patients with MDS, in which the main immunophenotype abnormality and the abnormality involving two cell lineages are key points to distinguish MDS from non-MDS.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Bone Marrow ; pathology ; Female ; Flow Cytometry ; methods ; Humans ; Immunophenotyping ; Male ; Middle Aged ; Myelodysplastic Syndromes ; immunology ; pathology ; Sensitivity and Specificity ; Young Adult

Aged ; CD59 Antigens ; immunology ; Female ; Granulocytes ; immunology ; Humans ; Myelodysplastic Syndromes ; immunology ; pathology

The myelodysplastic syndromes (MDS) are a group of heterogeneous hematological disorders and characterized by abnormalities of bone marrow myeloblasts in morphology and count, ineffective hematopoiesis, decrease of peripheral blood cells and high risk of transforming into acute myeloid leukemia. In this review, the morphological examination of bone marrow, bone marrow biopsy, karyotyping analysis of chromosome, and the significance of flow cytometric immunophenotyping for diagnosis and prognosis of MDS patients are discussed.
Bone Marrow ; pathology ; Humans ; Immunophenotyping ; Karyotyping ; Myelodysplastic Syndromes ; genetics ; immunology ; pathology