الملخص
PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone. METHODS: We performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared 8 secondary HLH patients who were first diagnosed with incomplete KD with all 247 incomplete KD diagnosed patients during the study period. The complete blood count, erythrocyte sedimentation rate, platelet count, and serum total protein, albumin, triglyceride, C-reactive protein, N-terminal pro-brain natriuretic peptide (NT-proBNP), and ferritin levels were compared. Clinical characteristics and echocardiography findings were also compared between the 2 groups. RESULTS: The total duration of fever was longer in the HLH-KD group than in the KD group. White blood cell and platelet counts were higher in the KD group. Alanine aminotransferase, ferritin, and coronary artery diameter were increased in the HLH-KD group compared with those in the KD group. The median of NT-proBNP was significantly higher in the HLH-KD group than in the KD group at 889.0 (interquartile range [IQR], 384.5–1792.0) pg/mL vs. 233.0 (IQR, 107.0–544.0) pg/mL. CONCLUSION: The NT-proBNP level may be helpful in distinguishing incomplete KD from KD with HLH. The NT-proBNP level should be determined in KD patients with prolonged fever, in addition to the white blood cell count, platelet count, and ferritin level, to evaluate secondary HLH.
الموضوعات
Humans , Alanine Transaminase , Blood Cell Count , Blood Sedimentation , C-Reactive Protein , Coronary Vessels , Echocardiography , Ferritins , Fever , Leukocyte Count , Leukocytes , Lymphohistiocytosis, Hemophagocytic , Mucocutaneous Lymph Node Syndrome , Natriuretic Peptide, Brain , Platelet Count , Retrospective Studies , Triglyceridesالملخص
Lange-Giedion syndrome, or trichorhinophalangeal syndrome type 2 (TRPSII), is a clinical syndrome characterized by mild growth restriction, mental retardation, microcephaly and dysmorphic face. Bulbous nose, large protruding ears and loose redundant skin are distinguishing features, as well as lax joints and phalangeal abnormalities of the hands and multiple exostoses. TRPS1 and EXT1 gene deletion are responsible for this. Diagnosis is mainly based on clinical and radiographic features. In Korea, no cases of this disease have been reported thus far. Along with a review of the literature, we report a case of TRPSII in a neonate who had peculiar face representing TRPSII, polydactyly, Mullerian duct cyst, and ptosis and was found to have an interstitial deletion of 8q23-24.1.
الموضوعات
Humans , Infant, Newborn , Diagnosis , Ear , Exostoses, Multiple Hereditary , Gene Deletion , Hand , Intellectual Disability , Joints , Korea , Langer-Giedion Syndrome , Microcephaly , Nose , Polydactyly , Skinالملخص
Eisenmenger syndrome is a severe form of pulmonary arterial hypertension related to congenital cardiac defects. Many patients die at a young age from such complications. The treatment of primary pulmonary hypertension is being applied to Eisenmenger syndrome such as endothelin receptor antagonists, phosphodiesterase-5 blockers, and prostacyclin. We experienced a case of 29-year female with ventricular septal defect-related Eisenmenger syndrome complicated with Down syndrome and Moyamoya disease, who was admitted to intensive care unit due to enteritis-associated septic shock. After the combination treatment with iloprost and sildenafil within the intensive care unit, the patient was able to wean mechanical ventilation without further applications of invasive rescue therapy such as extracorporeal membrane oxygenator. She was later discharged with bosentan. She maintained bosentan therapy for 34 months continuously without aggravations of symptom but eventually died with intracranial hemorrhage, a complication of Moyamoya disease. To our knowledge, this is the first case report of Eisenmenger syndrome accompanied by mosaic Down syndrome and Moyamoya disease.
الموضوعات
Female , Humans , Cyclic Nucleotide Phosphodiesterases, Type 5 , Down Syndrome , Eisenmenger Complex , Epoprostenol , Hypertension , Hypertension, Pulmonary , Iloprost , Critical Care , Intensive Care Units , Intracranial Hemorrhages , Moyamoya Disease , Oxygenators, Membrane , Piperazines , Purines , Receptors, Endothelin , Respiration, Artificial , Shock, Septic , Sulfonamides , Sulfones , Sildenafil Citrateالملخص
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.
الموضوعات
Adult , Female , Humans , Male , Middle Aged , Bone Marrow Cells/cytology , Cell Lineage , Chromosome Aberrations , Diagnosis, Differential , Megakaryocytes/immunology , Myelodysplastic Syndromes/diagnosis , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Retrospective Studiesالملخص
BACKGROUND: Despite the diagnostic utility of immunophenotyping for myelodysplastic syndromes (MDS), it has not been widely performed, and reports on this are absent in Korea. We aimed to evaluate the immunophenotypic features of non-blastic granulocytes, monocytes, and blasts in patients with MDS and non-clonal disorders using routine flow cytometry (FCM). Moreover, we evaluated the phenotypic abnormalities of mature cells in leukemic patients. METHODS: Marrow aspirates from 60 patients, including 18 with MDS, 18 with leukemia, and 24 with non-clonal disorders (control group), were analyzed using FCM. Blasts, non-blast myeloid cells, and monocytes were gated based on CD45 expression and side scatter (SSC). The phenotypes were then compared among the 3 groups. RESULTS: Compared to non-clonal disorders, the granulocytic lineages of MDS showed decreased SSC (P=0.005), increased CD45 intensity (P=0.020), decreased CD10-positive granulocytes (P= 0.030), and a higher CD56-positive rate (P=0.005). It is noteworthy that similar results were obtained in the leukemia group, and these findings were not related to the phenotypes of the leukemic cells. Using blast and monocytic gating, useful parameters for generating a differential diagnosis were not found. CONCLUSIONS: Gating the granulocytic region is a relatively easy method for MDS immunophenotyping. Among the parameters studied, SSC, CD10, and CD56 were the most useful for differentiating MDS from non-clonal disorders. While immunophenotypic changes in MDS appear to be useful for differentiating MDS from non-clonal disorders, these changes were also noted in the mature cells of leukemic patients.
الموضوعات
Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Leukocyte Common Antigens/metabolism , CD56 Antigen/metabolism , Bone Marrow Cells/cytology , Cell Lineage , Diagnosis, Differential , Flow Cytometry , Granulocytes/classification , Immunophenotyping , Leukemia/diagnosis , Monocytes/classification , Myelodysplastic Syndromes/diagnosis , Neprilysin/metabolism , Phenotypeالملخص
PURPOSE: The treatment outcome of an intestinal obstruction depends on the early recognition and emergent operation of a strangulated intestinal obstruction. Endotoxin is a lipopolysaccharide component of the cell wall of gram negative bacteria that normally exist in the intestinal lumen. When a strangulated obstruction occurs, the endotoxin passes transluminally into the peritoneal cavity and blood stream. A disruption of the mucosal barrier is an important putative cause in this pathogenesis. This study investigated the relationship between the endotoxin level and progress of an intestinal obstruction to a strangulated obstruction. METHODS: 25 adult male Sprague-Dawley rats (200~250 g) were divided into 3 groups, the control group, simple obstruction group, and closed loop obstruction group. An intestinal obstruction was induced using a silk tie. Blood samples and obstructed bowel wall specimens were obtained at 24, 48 and 72 hours after surgery. The endotoxin and IL-6 levels were examined, and all specimens were reviewed by a pathologist for mucosal damage after H-E staining. RESULTS: In the obstruction groups, dilated bowel loops at the proximal end of the obstruction site was identified but there were no ischemic changes. The endotoxin and IL-6 levels were similar regardless of the obstruction types and times. There were no differences between the three groups in the degree of mucosal damage. However, according to the endotoxin level, the groups with an endotoxin level < 0.2 EU/ml showed mild mucosal damage. The severity of mucosal damage increased with increasing endotoxin level. CONCLUSION: There is a positive relationship (r(2)=0.673, P-value=0.002) between the endotoxin level and mucosal damage. This suggests the possibility of using endotoxin as a predictive factor for the detection of mucosal injury in an intestinal obstruction. However, a larger scale will be needed to confirm the statistical significance.
الموضوعات
Adult , Humans , Male , Cell Wall , Gram-Negative Bacteria , Interleukin-6 , Intestinal Obstruction , Peritoneal Cavity , Rats, Sprague-Dawley , Rivers , Silk , Treatment Outcomeالملخص
PURPOSE: To compare the evaluation results of faculties to those of Standardized Patients (SP) participating in a Clinical Performance Examination (CPX) administered at Ewha Womans University College of Medicine. METHODS: The CPX was taken by 77 fourth year medical students. Cases and checklist were developed by the medical school consortium in capital area. Six cases were used and 24 SPs participated and evaluated the students' performances. The whole session was recorded on videotapes so that 6 medical school faculties could analyze and evaluate the students' performances as well. The results were compared and analyzed by SPSS package. RESULTS: The agreement between the faculties and the SPs was relatively good (r=0.79), but not good enough. In every case, SPs gave higher marks than did the faculties. Clear disease entity cases like "hepatitis" and "anemia" showed better agreement than obscure clinical contexts such as "bad news delivery". Better agreement was seen in the items of physical exam category (r=0.91), but the agreement was very poor in the items of doctor-patient (Dr-Pt) relationship category (r=0.54). The construction of checklist and the character of each evaluation item should influence the differences. CONCLUSION: More detailed guidelines and clear/specific evaluating items are necessary to improve the agreement rate. In certain categories like physical exam and brief history taking, the SP' s evaluation can replace the faculties', but for complex contexts like Dr-Pt relationship.
الموضوعات
Female , Humans , Checklist , Schools, Medical , Students, Medical , Videotape Recordingالملخص
BACKGROUND: Abnormal sex differentiation and development may present ambiguous genitalia in the newborn or lack of secondary sexual characteristics in puberty. A prompt and accurate diag-nosis should be established to minimize or avoid medical, psychological and social complications. The purpose of this study was to evaluate the causes and clinical characteristics of patients with abnormal sex differentiation and development. METHODS: We analyzed 35 patients with abnormal sex differentiation and development. Twenty patients had been considered or reared as males and fifteen patients as females. The diagnostic evaluation consisted of physical examination, hormonal analysis, sonogram, genitogram, gonadal biopsy and cytogenetics. RESULTS: Among the thirty-five patients, 11 patients were hypogonadism, 9 male pseudoherma-phroditism (5 hypospadia, 2 androgen insensitivity syndrome), 6 female pseudohermaphroditism (4 congenital adrenal hyperplasia), 4 micropenis, 4 congenital anomaly and 1 mixed gonadal dys-genesis. Gonadectomy was performed in patients with androgen insensitivity syndrome and mixed gonadal dysgenesis. Sex of rearing and gender assignment were all concordant with the known sex except one patient, who was previously reared as female and finally reassigned as male due to 5-alpha reductase deficiency. CONCLUSIONS: The causes of abnormal sex differentiation and development were variable. There-fore, an accurate diagnosis should be made by history, physical examination, radiologic and laboratory tests. Proper management and sex assignment are needed in accordance with the cause.
الموضوعات
Adolescent , Female , Humans , Infant, Newborn , Male , 46, XX Disorders of Sex Development , Amenorrhea , Androgen-Insensitivity Syndrome , Biopsy , Cytogenetics , Diagnosis , Disorders of Sex Development , Gonadal Dysgenesis, Mixed , Gonads , Hypogonadism , Hypospadias , Oxidoreductases , Physical Examination , Puberty , Sex Differentiation , Sexual Developmentالملخص
Isolated extramedullary relapse of acute lymphoblastic leukemia (ALL) with sparing of the marrow after allogeneic stem cell transplantation is not common. We report a 32-year-old female patient with isolated ovarian relapse of T-cell ALL 18 months after allogeneic stem cell transplantation. She had no evidence of concomitant relapse in the bone marrow.
الموضوعات
Adult , Female , Humans , Bone Marrow , Hematopoietic Stem Cell Transplantation , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Recurrence , Stem Cell Transplantation , T-Lymphocytesالملخص
BACKGROUND: The t(11;14)(q13;q32) is known to be one of the most frequent chromosomal abnor-malities found in multiple myeloma (MM). However, studies on t(11;14) in MM have been problemat-ic due to the fact that MM cells proliferate poorly in vitro. The purpose of our study is to evaluate inci-dence, clinical, and hematologic findings of MM with IgH and cyclin D1 gene rearrangement and to investigate the usefulness of interphase FISH (fluorescence in situ hybridization). METHODS: The study group included 36 patients (23 newly diagnosed MM, 8 relapsed MM, 5 per-sistent MM after treatment) admitted to Mokdong and Gil Hospital from November 1998 to July 2002. Interphase FISH was performed with IGH/CCND1 dual color, dual fusion translocation probe (Vysis Inc, Downers Grove, IL USA), using bone marrow mononuclear cells. RESULTS: Incidence of IgH and cyclin D1 gene rearrangement by interphase FISH was 19%. One patient with normal karyotype and another patient without any metaphase cells showed IgH and cyclin D1 gene rearrangement with interphase FISH. The lambda light chain subtype was more frequently found in patients with rearrangement (4/5, 80%) than those without rearrangement (6/23, 26%) (P<0.05). No significant differences were found in other clinical and hematologic findings in the two groups. CONCLUSIONS: We suggest that MM with IgH and cyclin D1 gene rearrangement is associated with the expression of lambda light chain. Interphase FISH may be helpful in samples with normal karyotype or no metaphase cells for detection of gene rearrangement of MM.
الموضوعات
Humans , Bone Marrow , Cyclin D1 , Cyclins , Gene Rearrangement , Genes, bcl-1 , Incidence , Interphase , Karyotype , Metaphase , Multiple Myelomaالملخص
BACKGROUND: Instead of hepatitis C virus (HCV) RNA test using RT-PCR, an enzyme immunoas-say for detection of HCV core protein as a simple, rapid method for the detection of HCV viremia has been developed recently. In this study, we investigated the usefulness of HCV core protein for the detection of HCV viremia by comparing the results of HCV RNA. METHODS: The study group included 71 patients; some of whom showed anti-HCV Ab. The HCV core protein assay was performed by enzyme immunoassay (Ortho Clinical Diagnostics, Raritan, NJ, USA). RESULTS: The concordance rate between HCV RNA and HCV core protein assay was 75%. Compared with the HCV RNA results, HCV core protein assay showed 50% sensitivity and 97% specificity. Among 17 patients whose results for HCV RNA were positive and those of HCV core protein were negative, all of them had anti-HCV Ab. CONCLUSIONS: Although the sensitivity of HCV core protein was not high in cases with anti-HCV Ab, the positive results for HCV core protein suggests the presence of HCV viremia. So, HCV core protein may be used as a simple and rapid method for detection of HCV viremia.
الموضوعات
Humans , Hepacivirus , Immunoenzyme Techniques , RNA , Sensitivity and Specificity , Staphylococcal Protein A , Viremiaالملخص
BACKGROUND: The triple marker test with maternal serum during 15-20 weeks gestation, based on AFP, hCG and uE3, is a useful screening technique for detecting chromosomal abnormalities and neural tube defect (NTD). However, the false positive rate has been reported from 4 to 8%. The purpose of this study was to evaluate whether or not women with a false positive result of the triple marker screening are at an increased risk for adverse outcomes other than chromosomal abnor-malities and NTD. METHODS: The study population was derived from 5,622 women undergoing triple marker screening at Mokdong Hospital between January, 1997 and August, 1999. A false positive was defined as a positive result of the triple marker test without further evidence of NTD or chromoso-mal abnormalities. The study group included 83 women whose results were a false positive and the 129 controls whose results were negative. The adverse outcomes included preterm delivery ( 0.05). However, adverse outcomes such as pla-cental abnormalities (8.4% vs 2.3%) and congenital anomalies (7.2% vs 0.8%) were more frequent in the women with a false positive result than women with a negative result s (P < 0.05). CONCLUSIONS: The results suggest that false positive results of the triple marker screening test do not appear to be associated with an increased risk for an adverse pregnancy outcome, but a careful evaluation for the placental and fetal abnormalities is necessary.
الموضوعات
Female , Humans , Pregnancy , Pregnancy , Chromosome Aberrations , Fetal Death , Mass Screening , Membranes , Neural Tube Defects , Pre-Eclampsia , Pregnancy Outcome , Ruptureالملخص
BACKGROUND: Chronic Myelogenous Leukemia (CML) is the first proven disease in which gene abnormality, t(9;22)(q34;q11) can cause the disease to occur in humans. Recently, targeted therapy with STI571 (GleevecTM), signal transduction inhibitor for BCR-ABL kinase was developed and can induce cytogenetic remission in patients with CML. Hypermetaphase-FISH (HMF)/Interphase-FISH (I-FISH, Fluorescence in situ hybridization) aiming specific chromosomal abnormalities are unambiguous quantitative molecular genetic methods for individual Philadelphia (Ph1) chromosome positive cells. We evaluated the change of Ph1 chromosome in CML patients during STI571 therapy using HMF/I- FISH. METHODS: Twenty one patients with CML were treated with STI571 which was provided from Norvatis pharmaceutical company as Expanded Access Program for Compassionate Use from May 2001 at the doses of 200-600 mg/day orally. Median age of this cohort was 37 years old and median follow up duration was 113 days (48~165 days). HMF or I-FISH using bone marrow or peripheral blood were performed on the sample at baseline, day 14, day 28 and then monthly. RESULTS: Complete cytogenetic responses which were assessed by HMF/I-FISH counting several hundreds cells were found in 8 of 21 patients. Among them, 4 of 10 chronic phase, 2 of 2 accelerate phase and 2 of 8 blastic crisis patients achieved cytogenetic complete response. One patient with blastic crisis was relapsed after achieving cytogenetic complete response. Grade III-IV thrombocytopenia and neutropenia were noticed in 8 and in 7 patients respectively, but there were no major bleeding episodes nor neutropenic fever. CONCLUSION: BCR-ABL tyrosine kinase inhibitor, STI571 was tolerable for patients with CML. The majority of patients achieved hematologic remission and 8 out of 21 patients achieved complete cytogenetic response regardless of their disease stage. Cytogenetic response of Ph1 chromosome can be quantified accurately with HMF/I-FISH.
الموضوعات
Adult , Humans , Bone Marrow , Chromosome Aberrations , Cohort Studies , Compassionate Use Trials , Cytogenetics , Fever , Fluorescence , Follow-Up Studies , Fusion Proteins, bcr-abl , Hemorrhage , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Molecular Biology , Neutropenia , Philadelphia Chromosome , Phosphotransferases , Signal Transduction , Thrombocytopenia , Imatinib Mesylateالملخص
BACKGROUND: The quantitative measurement of HBV DNA is useful in the follow-up of patients with chronic hepatitis B. However, the disappearance of HBV DNA, which is not always followed by HBeAg seroconversion, may not predict the outcome of the treatment. We evaluated the usefulness of HBeAg quantitation in comparison with HBV DNA quantitation. METHODS: A total number of 89 blood samples from 34 patients who were diagnosed with chronic hepatitis B were evaluated for HBeAg quantitation by the Murex HBeAg Standard and the Murex HBeAg/anti-HBe (Murex Biotech, Dartford, England). HBV DNA levels were measured by the Hybrid Capture System (Digene Corp., Beltsville, MD, USA). RESULTS: Among the total of 34 patients, the changes in the HBeAg level in 19 patients were parallel to those of the HBV DNA level in serial monitoring. In 5 patients, whose results showed discrepancy in the levels of HBeAg and DNA, the HBV DNA became undetectable earlier than did the HBeAg. Their HBeAg levels were less than 100 U/mL and were followed by HBeAg seroconversion after 1-4 months. And, in 1 patient, a progressive increase in HBeAg quantitation was not followed by HBeAg seroconversion after 8 months, even though HBV DNA was persistently undetectable. The concor-dance rate between quantitative HBeAg and HBV DNA results was 78.7%. CONCLUSIONS: This study suggests that HBeAg quantitation can be helpful in predicting seroconver-sion, especially when HBeAg is positive and HBV DNA is negative.
الموضوعات
Humans , DNA , Follow-Up Studies , Hepatitis B e Antigens , Hepatitis B, Chronic , Hepatitis, Chronic , Immunoenzyme Techniquesالملخص
BACKGROUND: Differential diagnosis may be difficult between essential thrombocythemia (ET) and chronic myelogenous leukemia (CML) with marked thrombocytosis, mild leukocytosis, and a few immature myeloid cells in peripheral blood at onset. The aim of the present study was to analyze clinical, hematologic, and molecular features of patients with CML, mimicking ET. METHODS: Among patients from Ewha and Gachon Gil Medical Center between January 1990 and June 2001, our study group included 3 patients with Ph-positive CML with marked thrombocy-tosis (>600 X 10(9)/L) and mild leukocytosis (<20 X 10(9)/L) and 12 patients of the typical ET as a con-trol group. RESULTS: Peripheral blood basophilia (4 - 12%) and a few immature granulocytes (1 - 9%) were the characteristic features of CML with thrombocythemic onset, compared with the typical ET. There was no evidence of bone marrow eosinophilia, basophilia, or fibrosis in CML with thrombocythemic onset. CONCLUSIONS: Our study suggests that peripheral basophilia as well as the positivity of Ph chromo-somes or bcr/abl gene rearrangement can be a clue to diagnosis of CML.
الموضوعات
Humans , Bone Marrow , Diagnosis , Diagnosis, Differential , Eosinophilia , Fibrosis , Gene Rearrangement , Granulocytes , Hydrogen-Ion Concentration , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Leukocytosis , Myeloid Cells , Thrombocythemia, Essential , Thrombocytosisالملخص
BACKGROUND: Chimerism analysis used to be one of the most valuable methods for monitoring patients after allogeneic hematopoietic stem cell transplantation (SCT). The relationship between the mixed chimerism status and the risk of relapse has been controversial. We analysed the clinical significance of mixed chimerism for the prediction of relapse after SCT. METHODS: Between October 2000 and January 2002, 16 patients with haematologic malignancies treated with SCT were included in this study. The median follow-up periods were 11.5 months (range 5-32 months) after SCT. For chimerism analysis, STR (D13S317, D5S818, D7S820) and VNTR (D1S80, D17S30) loci were amplified by PCR. Patients who exhibited complete donor hematopoiesis at all times during the follow-up period were defined as CCG (complete chimerism group) and those who showed mixed chimerism at least once at any time were definded as the MCG (mixed chimerism group). Relapse was considered based on clinical, hematologic and cytogenetic findings. RESULTS: MCG was 63% (10/16). Relapse was observed in 80% (8/10) of MCG and none of CCG (P>0.05). Among 8 relapsed patients, two patients showed MC 1 month prior to relapse and 4 patients changed to MC from CC at relapse status. The remaining 1 patient continued to show CC. CONCLUSIONS: Mixed chimerism seems to be associated with a high risk of relapse. For early detection of relapse, chimerism analysis may need to be performed at shorter time intervals than once a month.
الموضوعات
Humans , Chimerism , Cytogenetics , Follow-Up Studies , Hematopoiesis , Hematopoietic Stem Cell Transplantation , Hematopoietic Stem Cells , Polymerase Chain Reaction , Recurrence , Tissue Donorsالملخص
BACKGROUND: Infection can activate the immune system and may trigger the production of autoantibodies. It has been reported that malaria infection triggers the production of various autoantibodies. Therefore, we investigated the pattern and significance of antinuclear antibodies (ANA) found in patients with malaria infection. METHODS: Our study group included 36 patients who were diagnosed with malaria infection at Mokdong Hospital from July 1998 to July 2001. We performed antinuclear antibody test using indirect immunofluorescence method (Quantafluor, Sanofi Diagnostics Pasteur Inc., USA), extractable nuclear antigen test (ENA) using double immunodiffusion method (Nova Gel, Inova Diagnostics Inc., USA), anti-double stranded DNA Ab test (anti-ds DNA Ab) using Farr assay (DPC anti-DNA, Diagnostic products Corporation, USA), and anti-single stranded DNA Ab test (anti-ssDNA Ab) using enzyme immunoassay method (QUANTA, Lite ssDNA, Inova Diagnostics Inc., USA). RESULTS: Among the 36 patients, 32 patients (88.9%) showed ANA positivity and 27 patients (75.0%) showed cytoskeleton or speckled pattern of ANA. Anti-ssDNA Ab was found in 3 of 20 patients; however, anti-dsDNA Ab and ENA were not found in all patients. Patients who had ANA showed higher levels of IgG, IgM and IgA, compared with those patients who did not have ANA. Follow up (11-37 month) of the 13 patients with ANA positivity revealed no symptoms associated with autoimmune disorder. CONCLUSIONS: Malaria infection may develop ANA, especially cytoskeleton or speckled pattern. The follow up of patients with ANA positivity showed no symptoms associated with any autoimmune disorder, but further evaluation would be necessary to reveal the relationship between malaria infection and development of autoimmune disorder.
الموضوعات
Humans , Antibodies, Antinuclear , Autoantibodies , Cytoskeleton , DNA , Fluorescent Antibody Technique, Indirect , Follow-Up Studies , Immune System , Immunodiffusion , Immunoenzyme Techniques , Immunoglobulin A , Immunoglobulin G , Immunoglobulin M , Malaria , Radioimmunoprecipitation Assayالملخص
A CD7 positive acute leukemia, lacking CD4, CD8, CD3, CD13 and CD33 expression may include 4 categories; acute T-cell leukemia, mixed lineage leukemia, acute undifferentiated leukemia and CD7 positive acute myeloid leukemia. Therefore, the expression of cyCD3 or the presence of TCR gene rearrangement can make the diagnosis of acute T-cell leukemia. We report a patient with acute T-cell lymphoblastic leukemia, showing CD7+, CD4-CD8-, and CD3-expression and TCR gamma gene rearrangement.
الموضوعات
Humans , Diagnosis , Genes, T-Cell Receptor , Genes, T-Cell Receptor gamma , Leukemia , Leukemia, Myeloid, Acute , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma , T-Lymphocytesالملخص
BACKGROUND: The test for the anti-rubella IgM Ab (R-IgM) is important in early pregnancies because therapeutic termination may be considered depending on the results. METHODS: The subjects consisted of 52 pregnant women with positive or equivocal R-IgM by Cobas Core Anti-Rubella IgM EIA test (Roche, Basel, Swiss) between January 1997 and July 2000. Three different EIA methods such as the Enzygnost Anti-Rubella-virus/IgM test (DADE Behring, Marburg, Germany), the AxSYM Anti-Rubella IgM test (Abbott, USA), and the IMx Anti-Rubella IgM test (Abbott, USA) were simultaneously performed on 44 specimens as well as the Cobas Core Anti-Rubella IgM EIA test. RESULTS: Among 52 pregnancies, 9 (17%) experienced an artificial abortion due to positive or equivocal R-IgM result. The clinical symptoms associated with rubella infection were observed in 3 cases and the persistent R-IgM positivity was noted for more than 1 year in 4 cases. The concordance rate between 4 different EIAs was 41%. When performed with serial diluted pool and 3 sera, the results of Cobas Core were similar to those of AxSYM, IMx and Enzygnost. The R-IgM were detected one titre higher in diluted sera performed by IMx and Enzygnost than those of Cobas Core and AxSYM. CONCLUSIONS: For pregnancies with positive or equivocal R-IgM, it is recommended that results should be interpreted with caution, considering the possibilities, such as a persistent R-IgM response and discrepant R-IgM depending on the different EIA methods.
الموضوعات
Female , Humans , Pregnancy , Immunoenzyme Techniques , Immunoglobulin M , Pregnant Women , Rubellaالملخص
BACKGROUND: Type and screen is recommended for efficient use of blood and reduction in workload in blood bank. Column agglutiation test is standardized and easy to perform, and provide clear and stable reactions that improve the interpretation of results. In this study, we compared column agglutination test(Ortho Diagnostic Systems Inc., USA) with conventional tube test and investigate its usefulness in irregular antibody screening and identification. METHODS: A total 182 samples were screened for irregular antibodies using column agglutination test and conventional tube test. And 18 patient's sera in which irregular antibodies previously screened by both tube and column agglutination tests were identified for irregular antibodies by tube and column agglutination tests. We compared the results of two tests. RESULTS: In the screening test, there was 96.7%(176/182) agreements between column agglutination test and conventional tube test. The column agglutination test showed stronger reactivity than tube test. In the irregular antibody identification, there was 88.8%(16/18) agreement between two tests and disagreement were seen in the identification of anti-P1 and anti-Leb antibodies. CONCLUSION: The results of column agglutination test are objective and superior to the conventional tube test in irregular antibody screening and identification tests. These results suggest that the column agglutination test will be useful and more convenient test in antibody screening and identification.