T-large granular lymphocytic leukemia

T-cell large granular lymphocyte (T-LGL) leukemia is characterized by clonal expansion of cytotoxic T cells resulting in cytopenia. The proliferation of clonal LGLs is caused by prolonged antigenic stimulation, which leads to apoptotic dysregulation owing mainly to the constitutive activation of survival pathways, notably the JAK/STAT pathway.Understanding how leukemic T-LGL persists can aid in the development of future immunosuppressive therapies. In this review, we summarize the diagnosis and current standard of therapy for T-LGL leukemia, as well as recent advances in clinical trials.

High-grade nodal marginal zone lymphoma with diffuse bone marrow involvement and IgM-type monoclonal paraproteinemia: a case report and review of the literature

No abstract available.

Bone marrow metastasis of small cell lung carcinoma with spontaneous tumor lysis syndrome without hepatic metastasis at diagnosis: first case report in Korea and review of literature

No abstract available.

Low-grade follicular lymphoma involvement of the bone marrow with a mixed paratrabecular, diffuse, and massive pattern expressing typical mantle cell lymphoma immunophenotype CD23−/FMC7+: a case report

No abstract available.

Severe Scrub Typhus with Hemophagocytic Lymphohistiocytosis Occurring in Summer / 대한내과학회지

In Korea, scrub typhus usually occurs in October and November. Hemophagocytic lymphohistiocytosis (HLH) is a distinct clinical entity characterized by a high fever, pancytopenia, hepatosplenomegaly, histiocyte proliferation, and hemophagocytosis. We encountered a summertime case of severe scrub typhus presenting as HLH. A 49-year-old female complained of abdominal pain and fever 3 days in duration. On hospital day 3 she was transferred to the intensive care unit because of clinical deterioration accompanied by severe sepsis. As an eschar was evident on the right shoulder, we commenced doxycycline. Her condition improved dramatically and she was discharged on day 14. Although the indirect immunofluorescence antibody test (IFA) for Orientia tsutsugamushi was negative on admission, a repeat IFA test was positive; the antibody titer was 1:5,120 on hospital day 10. Scrub typhus should be considered during differential diagnosis in a patient with severe sepsis in any season except the fall.

Severe Fever with Thrombocytopenia Syndrome Presenting with Hemophagocytic Lymphohistiocytosis

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease caused by the newly discovered SFTS Bunyavirus, and there have been no case reports of SFTS patients presenting with hemophagocytic lymphohistiocytosis (HLH) in the English literature. We report a case of SFTS presenting with HLH in a 73-year-old immunocompetent male farmer. Although the patient had poor prognostic factors for SFTS, such as old age and central nervous system symptoms, he recovered fully with supportive care.

A de novo Microdeletion of ANKRD11 Gene in a Korean Patient with KBG Syndrome

KBG syndrome is a very rare genetic disorder characterized by macrodontia of upper central incisors, global developmental delay, distinctive craniofacial features, short stature, and skeletal anomalies. Ankyrin repeat domain 11 gene (ANKRD11) has recently been identified as a causal factor of this syndrome. We describe a 6-yr-old Korean boy with features of KBG syndrome. The patient had a short stature, macrodontia, dysmorphic facial features, speech and motor delay with intellectual disability, and partial seizures as indicated by the electroencephalogram, but he was neither autistic nor had autism spectrum disorders. Using high-resolution oligonucleotide array comparative genomic hybridization, we identified a heterozygous 240-kb deletion at 16q24.3 corresponding to ANKRD11. This patient provided additional evidence on the influence of ANKRD11 in KBG syndrome and suggested that deletion limited to ANKRD11 is unlikely to cause autism.

The Combination of Real-Time PCR and HPLC for the Identification of Non-Tuberculous Mycobacteria

We used HPLC and AdvanSure real-time PCR (LG Life Sciences, Korea) to retrospectively analyze non-tuberculous mycobacteria (NTM) in 133 clinical specimens. The specimens were culture-positive for NTM and the HPLC method identified 130 strains of mycobacteria from the cultures (97.7%) at the species level. Among the isolates, 48 Mycobacterium. kansasii (36.1%), 39 M. intracellulare (29.3%), 17 M. avium (12.8%), 16 M. abscessus (12.0%), 6 M. fortuitum (4.5%), 2 M. szulgai (1.5%), 2 M. gordonae (1.5%), and 3 unclassified NTM strains (2.3%) were identified. The real-time PCR assay identified 60 NTM-positive specimens (45.1%), 65 negative specimens (48.9%), and 8 M. tuberculosis (TB)-positive specimens (6.0%). The real-time PCR assay is advantageous because of its rapid identification of NTM. However, in our study, the real-time PCR assay showed relatively low sensitivity (45.1%) when using direct specimens including sputum and bronchoalveolar lavage (BAL) fluid. HPLC is useful as it discriminates NTM at the species level, although it is time-consuming and requires specific equipment and technical expertise. A combination of both methods will be helpful for the rapid and accurate identification of mycobacteria in clinical laboratories.

Performance Evaluation of 3 Kinds of HBsAg Qualitative Assays and 2 Kinds of Quantitative Assays

BACKGROUND: Currently used techniques for quantitation of HBsAg often yield discordant results; therefore, development of quantitation techniques that can detect HBsAg with high accuracy has become very important. Recent advances have led to the development of several HBsAg detection systems. Here, we evaluated the performance of 3 newly developed detection systems, which can detect HBsAg both qualitatively and quantitavely, and determined the concordance among their results. METHODS: Four hundred and thirty two samples assigned to 4 groups-patient group, dilution group, weakly reactive group, and linearity group- were subjected to qualitative and quantitative detection of HBsAg by using the 3 systems developed by 3 major manufacturers; Abbott Architect, Roche E170 and Siemens Centaur XP. RESULTS: The results for the qualitative analyses were closely concordant among the three systems (98.3%) for all 432 samples. In 123 samples that were determined as HBsAg-negative, E170 (76%) distributed frequently at the upper half level (0.5-1.0) of negative reference range, compared with Architect (11%) and Centaur XP (22%). In particular, in 65 samples that were diluted from the strongly positive samples to obtain weakly positive samples, the average index values obtained using Architect (3.6 S/CO), E170 (4.2 COI) and Centaur XP (11.4 index value) differed significantly (P<0.0001). In the antiviral treatment group and the post-liver transplantation group, no inconsistency was observed among the results of the qualitative and quantitative assays. In the 18-fold serially diluted samples, no linearity was observed. CONCLUSIONS: Because of the possibility of false-positive detection in the HBsAg-negative samples, regular management of equipment and appropriate selection of reagents are very important. In weakly positive samples, quantitative assay has not to be replaced for qualitative assay. Therefore, the qualitative assays should be used for screening the samples, whereas the quantitative assays should be used for monitoring the Hepatitis B virus (HBV) load in the samples determined as HBsAg-positive. The qualitative index value should not be interpreted as a quantitative measure of HBV load.

Performance Evaluation of Immunoassay Detection of HBsAg Mutants and Their Clinical Significance in the High Risk Groups

BACKGROUND: False negative results have been reported in the immunodetection of hepatitis B virus (HBV) because of the existence of the various mutants of the virus, causing most suppliers to try to develop superior reagents by using highly sensitive and specific monoclonal or polyclonal antibodies. In this study, we evaluated the effectiveness of 3 newly developed reagents by major manufacturers by adopting automated methods with increased sensitivity and specificity in the detection and discrimination of native and recombinant mutant antigens. METHODS: We analyzed samples confirmed positive for hepatitis B surface antigen (HBsAg), high-risk samples from chronic hepatitis patients treated with antiviral agents, and samples from patients who had undergone liver transplantation and were treated with high-dose hepatitis B immunoglobulin (HBIG) by using reagents and systems newly developed by Abbott Laboratories (USA), Roche Diagnostics (Germany), and Siemens Healthcare Diagnostics (USA). Recombinant sample panels from these manufacturers with low and high concentrations were also analyzed for comparing the 3 reagents. RESULTS: There were no discrepant results among the various selected patient groups; however, for the recombinant mutant panels, all of the 3 reagents showed highly positive detection rates for their corresponding mutant panels, but showed relatively discrepant mutant detection rates when cross-tested with the other mutant panels. Detection rates of the HBsAg mutant panels were higher at a higher concentration of the mutant samples, but were lower for the same mutant receptor sites at a lower concentration. CONCLUSIONS: The 3 major detection methods seem to recognize the major native mutants commonly encountered in clinical practice. However, in the case of recombinant mutants, we believe that our data are not to be interpreted as a reference standard for any reagent, because the results can only be validated for the reagents' corresponding mutant panels; such results tend to be mutually exclusive, and the enough concentration of mutants was required to be adjusted for a comparative analysis.

Lung Infection Caused by Mycobacterium riyadhense Confused with Mycobacterium tuberculosis: The First Case in Korea

A slowly growing, non-chromogenic mycobacterial strain was isolated from sputum and bronchial lavage fluid samples of a patient presenting with productive cough, blood-tinged sputum, low-grade fever, and weakness. A positive acid-fast bacilli sputum smear result prompted the initiation of an anti-tuberculosis regimen. Multiplex real-time PCR showed a negative result for Mycobacterium tuberculosis complex and a positive result for nontuberculous mycobacteria. The DNA chip test confirmed this organism as a member of the genus Mycobacterium, but could not specify the species. Interestingly, the mycolic acid patterns obtained by HPLC nearly overlapped with those of M. simulans. The sequences of the Mycobacterium 16S rRNA gene and 16S-23S internal transcribed spacer region were unique and were found to have 100% similarity with those of M. riyadhense. After a review of the literature, we report this case as the first Korean case of M. riyadhense lung infection.

The incidence of cough induced by remifentanil during anesthetic induction was decreased by graded escalation of the remifentanil concentration / 대한마취과학회지

BACKGROUND: It is well known that opioids induce coughing. Many drugs such as lidocaine and ketamine are used to effectively prevent the coughing induced by opioids and this has been revealed to be effective. In this study, we evaluated the preventive effect of a graded escalation of the remifentanil concentration using a target controlled infusion pump and we compared this with the effect of lidocaine. METHODS: One hundred fifty ASA I and II patients who were scheduled for elective surgery were randomly divided into 3 groups. The patients were pretreated with 2% lidocaine 1 mg/kg (Group L) or saline (Group S) and remifentanil infusion (an effect site concentration of 4.0 ng/ml) was followed 1 minute later by using a target controlled infusion pump. Group R was pretreated with saline and this was followed by remifentanil infusion (effect site concentration of 2.0 ng/ml at first and then it was reset to 4.0 ng/ml). We evaluated the incidence, severity and onset time of cough after remifentanil infusion. RESULTS: The incidence of coughing was significantly decreased in Group R (6 cases, 12%) and Group L (7 cases, 14%), as compared to that of Group S (17 cases, 34%) (P < 0.05), but there was no significant difference between Group R and Group L. The groups showed no significant difference in the severity and the onset time of coughing. CONCLUSIONS: This study demonstrated that administering graded escalation of the remifentanil concentration suppresses remifentanil-induced coughing as effectively as lidocaine 1 mg/kg pretreatment.

Clarithromycin and Amoxicillin Susceptibility Testing of Helicobacter pylori by Disk Diffusion Method / 대한임상미생물학회지

BACKGROUND: CLSI provides a guideline only for a agar dilution method of testing clarithromycin susceptibility for Helicobacter pylori. This study was to evaluate a disk diffusion method for clarithromycin and amoxicillin. METHODS: One hundred and forty clinical isolates of H. pylori isolated from May 2005 to May 2007 were tested by the CLSI agar dilution method and a disk diffusion method using 2microgram (2CLR) and 15microgram (15CLR) clarithromycin disks and 2microgram (2AMX) and 10microgram (10AMX) amoxicillin disks. The interpretation criteria used for the disk diffusion method were established by linear regression and error rate-bounded method for disk diffusion zone of inhibition (DDZ) compared to MIC. RESULTS: Resistance and intermediate rates to clarithromycin were 21.4% and 1.4%, respectively. A number of isolates with MIC 0.5, 1, and 2 (microgram/mL) to amoxicillin were 7, 2, and 1, respectively. For 2CLR and 15CLR, the coefficients of determination (R2) between MIC and DDZ were 0.931 and 0.923 (P< 0.001), respectively, and the criteria for resistance/ susceptibility were 12/28 mm for 2CLR and 23/39 mm for 15CLR. For 2AMX and 10AMX, the R2 between MIC and DDZ were 0.478 and 0.421 (P< 0.001), respectively, and the criteria for resistance with breakpoint of 2microgram/mL were 21 mm for 2AMX and 32 mm for 10AMX. All isolates had DDZ<60 mm with 2CLR and 2AMX, but 61.4% and 75.7% of the isolates had DDZ<60 mm with 15CLR and 10AMX, respectively. CONCLUSION: Excellent correlation and agreement between MIC and DDZ were found for clarithromycin and amoxicillin. With 2microgram disks, the susceptibility breakpoints were 28 mm or less; thus, two disks could be tested in one plate.

Transfusion of Jra-positive Red Blood Cells to a Jra-negative Patient with Anti-Jra / 대한수혈학회지

Jra is a high-frequency red cell antigen, and antibodies to Jra (anti-Jra) are rarely found. The clinical significance of anti-Jra has been under debate. We report a case of a Jra negative patient having anti-Jra who was urgently transfused with Jra-positive red blood cells (RBCs) during an aortic valvuloplasty. As Jra-negative RBCs were not available, this 57 year-old female patient was transfused with six units of Jra-positive RBCs during the surgery. The hemoglobin concentration decreased from 13.0 g/dL to 7.7 g/dL due to excessive bleeding, but increased up to 13.2 g/dL after the transfusion of an additional four units of Jra-positive RBCs. A direct antiglobulin test (DAT) was positive on day 3 and day 10 after surgery. Twenty days following surgery at the outpatient clinic, the hemoglobin concentration was 10.4 g/dL and the patient had no clinical symptoms related to anemia. The DAT was negative 6 months after the transfusion. It seems to be relatively safe to transfuse Jra-positive RBCs to the patient with anti-Jra in an emergency situation.

Frequency of Serum Ionized Hypomagnesemia in Patient with Ionized Hypocalcemia / 임상검사와정도관리

INTRODUCTION: Hypomagnesemia, major cause of hypocalcemia, is developed due to insufficient oral intake in hospitalized patient and high prevalence in intensive care unit (ICU) patient. It is important to monitoring ionized magnesium in ICU patient because hypomagnesemia has been implicated in the development of cardiovascular dysfunction. So, in this study we determine the relation between ionized calcium and ionized magnesium and validate the measurement of ionized magnesium. MATERIAL AND METHOD: From March 2005 to may 2005, total 2876 samples were enrolled, which was requested to measure ionized calcium. We measured ionized calcium and ionized magnesium using NOVA CCX (Nova Biomedical, Waltman, MA, USA). Reference range of ionized calcium and ionized magnesium was 3.9~4.5 and 1.1~1.5 mg/dL, respectively. The patients were grouped as adult above 18 years old and pediatric below 18 years old. The ward was intensive care unit (ICU), general ward, outpatient and emergency room. We investigate the frequency of hypocalcemia and hypomagnesemia. RESULTS: The prevalence of ionized hypocalcemia (Ca2+ < 3.9mg/dL) was 22.2% and the prevalence of ionized hypomagnesemia (Mg2+ < 1.1mg/dL) was 41.9%. Of 2876 samples, 377 samples had ionized hypocalcemia and ionized hypomagnesemia simultaneously. Fifty-nine percent samples showing ionized hypocalcemia had ionized hypomagnesemia. In pediatric patients 13.3% of all patients had ionized hypocalcemia, 20.0% showing ionized hypercalcemia and 37.6% showing ionized hypomagnesemia. In adult patients 24.3% of all patients had ionized hypocalcemia, 14.3% showing ionized hypercalcemia, 48.2% showing ionized hypomagnesemia and one patient had ionized hypermagnesemia. When considering total cases of ionized calcium, the average level of ionized calcium was lowest in emergency room. When considering in case of patients, ICU showed the lowest level of ionized calcium. CONCLUSION: Ionized hypomagnesemia had known to be one of the major causes of ionized hypocalcemia and is common in ionized hypocalcemia. It is easy to found by measuring simultaneously. We found high coincidence rate of ionized hypocalcemia and ionized hypomagnesemia. We recommend that all samples ordered to be measuring ionized calcium must be checked ionized magnesium simultaneously.

A questionnaire Survey of HLA laboratories in Korea (2005) / 대한진단검사의학회지

BACKGROUND: As unrelated bone marrow transplantation is rapidly increasing and organ sharing network is becoming solidly instituted in Korea, it is getting more important to make standardization between laboratories performing human leukocyte antigen (HLA) typing or crossmatch. By a questionnaire survey for the laboratories, we intended to make a basis for laboratory standardization in Korea. METHODS: The questionnaires were distributed to 59 HLA laboratories enrolled in the HLA quality assessment (QA) program in Korea and replies were obtained from 51 laboratories. The questionnaire items were slightly modified from those of the previous survey. RESULTS: Most of the HLA laboratories (49/51, 96.1%) belonged to the specialties of laboratory medicine and were small scale in the number of HLA technicians and the annual number of HLA tests. There were 49 laboratories that used DNA typing methods for HLA DR. Nine of 44 laboratories which reported to perform HLA crossmatch did not separate T and B lymphocytes. Three (6.8%) of 44 laboratories did not use more sensitive methods such as T-AHG, T-long or flowcytometric methods. There were 7 laboratories performing flowcytometric crossmatch. Some laboratories (19.6%, 9/46) stored complement at inappropriate temperature. Six of 11 laboratories which were requested to test PRA were performing PRA test by themselves. CONCLUSIONS: It is still problematic for some laboratories performing crossmatches not using more sensitive methods or separation of T and B lymphocyte and storing complement at an inappropriate temperature. A continuous effort for laboratory standardization should be needed.

HLA Typing Proficiency Survey in Korea, 2003-2004 / 대한진단검사의학회지

BACKGROUND: HLA proficiency survey in Korea started in 1996 and the results of the survey were last reported in 1999. In this report, we summarized the results of the survey performed in recent 2 years. METHODS: A total of four proficiency surveys were performed, in which 54-59 laboratories participated. Each survey included 3 tests for HLA class I (serology and DNA) and class II (DNA) typing and 6 for HLA crossmatch test (3 cells x 2 sera). RESULTS: Overall concordance of serologic typing was 99.5% (436/438) for HLA-A, 95.7% (419/438) for HLA-B, and 94.8% (199/210) for HLA-C. The antigens assigned incorrectly by more than 5% of the participating laboratories were B54 (10.3%), B55 (10.3%), B27 (5.4%), Cw6 (22.9%), and C-blank (5.7%). Overall concordance rates of DNA typing were 99.7% (393/394) for HLA-A, 99.8% (415/416) for HLA-B, 100% (156/156) for HLA-C. Correct assignment of HLA-DRB1 and -DQB1 was reportred by 99.7% (98.1-100%) and 99.2% (88.9-100%) for generic and 99.2% and 98.1% (80-100%) for allelic level, respectively. Most laboratories (93.5-97.9%) were using sensitive methods of crossmatch such as T-long, T-AHG, and flowcytometry. The proportion of laboratories evaluated as unacceptable was on the average 3.1% of total laboratories. CONCLUSIONS: The rate of correct identification of HLA antigens was higher this time than in the previous survey in 1999. The rate of unacceptable crossmatch was also low enough to be satisfactory. It is thought that the proficiency survey has contributed to the high quality of HLA tests in the participating laboratories and should be continued to maintain the proficiency in Korea.