A positive HLA crossmatch in cadevaric liver transplantation is relatively acceptable, but in living donor liver transplantation (LDLT) using relatively small sized grafts, the rejection rates were higher in positive crossmatchcases than in negative cases, as described in several previous reports. We report a case of LDLT performed with therapeutic plasmapheresis, in a recipient with a positive HLA crossmatch to donor before transplantation. The patient was a 56-year-old male patient with liver cirrhosis (UNOS status IIA, MELD score 28) caused by chronic hepatitis B. The HLA crossmatch results were 1:2 and 1:8 positive for NIH-CDC (complement dependent cytotoxicity) and AHG-CDC, respectively. The flow cytometric crossmatch (FCXM) was also positive (T-MFI ratio 9.0 and B-MFI ratio 3.4). With 5 cycles of preoperative therapeutic plasmapheresis, the HLA crossmatch converted to negative and liver transplantation was performed. The liver function of the patient was well maintained for 5 months, without any sign of hyperacute or acute rejection. However, the patient eventually died from suddenly occurred infection-associated hemophagocytic syndrome at 5 months after surgery. Therapeutic plasmapheresis can be considered as one of therapeutic options for LDLT patients with a positive HLA crossmatch to donor.
Hepatitis B, Chronic ; Humans ; Liver Cirrhosis ; Liver Transplantation* ; Liver* ; Living Donors* ; Lymphohistiocytosis, Hemophagocytic ; Male ; Middle Aged ; Plasmapheresis* ; Tissue Donors ; Transplants

Circumscribed palmar or plantar hypokeratosis is a rare disease that is characterized by a well-circumscribed, erythematous depressed macule or patch on the palm or sole. It usually presents as an asymptomatic, solitary lesion in middle-aged or elderly women. The characteristic histopathological finding is a sharp stair-like alteration between involved and uninvolved skin with a markedly thinner horny layer and slightly decreased granular layer compared with adjacent normal skin. Herein we report a case of circumscribed palmar hypokeratosis and review the literature.
Aged ; Female ; Humans ; Rare Diseases ; Skin

Mastocytosis is an uncommon disease characterized by an abnormal accumulation of mast cells in a variety of tissues, most commonly, in the skin. It may mimic other conditions because of numerous signs and symptoms produced by mast cell mediators. We report a case of an unusual manifestation of cutaneous mastocytosis in a 7-year-old girl who had hypertrophy and periodic swelling of the left labia majora with urticaria pigmentosa.
Child ; Humans ; Hydrazines ; Hypertrophy ; Mast Cells ; Mastocytosis ; Mastocytosis, Cutaneous ; Skin ; Urticaria Pigmentosa

A patient treated with venlafaxine for major depression developed an interstitial lung disease (ILD) with the characteristic clinical, radiological and pathological features of chronic hypersensitivity pneumonitis. A high resolution computed tomography scan demonstrated ground glass opacity, mosaic perfusion with air-trapping and traction bronchiectasis in both lungs. The pathological findings were consistent with a nonspecific interstitial pneumonia pattern. Clinical and radiological improvements were noted after the discontinuation of venlafaxine and the administration of a corticosteroid. This report provides further evidence that the anti-depressant venlafaxine can cause ILD.
Alveolitis, Extrinsic Allergic ; Bronchiectasis ; Depression ; Glass ; Humans ; Hypersensitivity ; Lung ; Lung Diseases, Interstitial* ; Perfusion ; Pneumonia ; Traction ; Venlafaxine Hydrochloride

BACKGROUND: Current status of external quality assessment (EQA) of laboratory tests for syphilis in Korea was analyzed to find out the problems that should be improved in the future. METHODS: Based on the data from the external quality assessment program performed twice a year by the Immunoserology Subcommittee of the Korean Association of Quality Assurance for Clinical Laboratory from the year 2004 to 2006, discordance rates were analyzed according to the test method and commercial kit used. RESULTS: Among the laboratories participating in the EQA program for syphilis test, about 90% of them used non-treponemal tests and about 55% treponemal tests. The non-treponemal tests included RPR (rapid plasma reagin) and VDRL tests used in 88% (363/412) and 11% (45/412), respectively, of the laboratories. The discordance rates were 2.2% for RPR test and 3.6% for VDRL. For the treponemal tests, Treponema pallidum hemagglutination assay (TPHA) was used in 60-76% and Immunochromatography assay (ICA) in about 30% of the laboratories in 2006. A high discordance rate of over 10% was reported in both TPHA and in ICA methods, possibly due to a low titer (1:1 in VDRL) of EQA samples in 2005. Analysis of the accumulated data from year 2004 to 2006 showed that the discordance rates of TPHA, ICA, and FTA-ABS were 4.6%, 3.7%, and 2.7%, respectively. CONCLUSIONS: For syphilis tests, RPR test, TPHA, and ICA are mainly used in Korea. A high discordance rate is still reported in TPHA and ICA, especially when testing samples with a low titer. Further analysis of data and education of laboratory personnel are needed for the improvement of the EQA program.
Enzyme-Linked Immunosorbent Assay ; False Positive Reactions ; Fluorescent Treponemal Antibody-Absorption Test ; Humans ; Korea ; Quality Control ; Reagent Kits, Diagnostic ; Syphilis/*diagnosis ; Syphilis Serodiagnosis/methods/*standards ; Treponema Immobilization Test

Community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) has received considerable attention in recent years as the cause of infections in individuals in the community who do not have traditional risk factors for MRSA infection, such as hospitalization or contact with healthcare services. CA-MRSA strains have different molecular and antimicrobial susceptibilities, as compared to hospital-associated MRSA. Although CA-MRSA strains are primarily associated with skin and soft tissue infections, they can cause more invasive infections, such as severe community-acquired pneumonia. Reports on CA-MRSA pneumonia in Korea are sparse. Therefore, we report a case of CA-MRSA pneumonia with molecular typing of the MRSA isolate.
Community-Acquired Infections ; Delivery of Health Care ; Hospitalization ; Korea ; Methicillin Resistance ; Methicillin-Resistant Staphylococcus aureus ; Molecular Typing ; Pneumonia ; Risk Factors ; Skin ; Soft Tissue Infections

Multicentric Castleman's disease (CD) is a rare atypical lymphoproliferative disorder, which is characterized by various systemic manifestations. Some patients with multicentric CD may have concomitant lung parenchymal lesions, for which lymphoid interstitial pneumonia (LIP) is known to be the most common pathologic finding. Follicular bronchiolitis and LIP are considered to be on the same spectrum of the disease. We describe a case of multicentric CD with pulmonary involvement, which was pathologically proven as follicular bronchiolitis.
Bronchiolitis ; Giant Lymph Node Hyperplasia ; Humans ; Lip ; Lung ; Lung Diseases, Interstitial ; Lymphoproliferative Disorders

Humans ; Immunoassay ; Indicators and Reagents ; Mass Spectrometry ; Methods ; Pathology, Molecular ; Spectrum Analysis

Objective: To evaluate the perspective of healthcare professionals towards the 2019 European Alliance of Associations for Rheumatology (EULAR) vaccination guideline in patients with autoimmune inflammatory rheumatic diseases (AIIRD). Methods: Healthcare professionals who care for patients with AIIRD were invited to participate in an online survey regarding their perspective on the 2019 update of the EULAR recommendations for vaccination in adult patients with AIIRD. Level of agreement and implementation of the 6 overarching principles and 9 recommendations were rated on a 5-point Likert scale (1~5). Results: Survey responses of 371 healthcare professionals from Asia (42.2%) and North America (41.6%), Europe (13.8%), and other countries were analyzed. Only 16.3% of participants rated their familiarity with the 2019 EULAR guideline as 5/5 (“very well”). There was a high agreement (≥4/5 rating) with the overarching principles, except for the principles applying to liveattenuated vaccines. There was a high level of agreement with the recommendations regarding influenza and pneumococcal vaccinations; implementation of these recommendations was also high. Participants also reported a high level of agreement with the remaining recommendations but did not routinely implement these recommendations. Conclusion The 2019 update of EULAR recommendations for the vaccination of adult patients with AIIRD is generally thought to be important by healthcare professionals, although implementation of adequate vaccination is often lacking. Better education of healthcare providers may be important to optimize the vaccination coverage for patients with AIIRD.

The use of liquid chromatography-tandem mass spectrometry (LC-MS/MS) in clinical laboratories is increasing and is likely to expand into even more clinical venues in the future. Mass spectrometry is the standard method for analyte identification in the clinical chemistry field; however, differences in mass spectrometry protocols and handling affect the accuracy and reliability of these tests and prevent direct comparisons of results between laboratories. For example, the results of laboratories using LC-MS/MS methods are less likely to be reproducible than those of laboratories using conventional, automated methods. This is due to inadequate handling of the equipment and/or poor quality control after the implementation of the method, which may result in unnecessary medical expenditures or even adverse outcomes for the patients. Unfortunately, guidelines to monitor the accuracy of LC-MS/MS-based clinical tests are still lacking. In general, the quality control methods used in conventional clinical tests could also be applied to LC-MS/MS. However, additional quality control methods specific to LC-MS/MS techniques must be continuously employed to maintain the same quality level achieved during method development and verification. This report is intended to help clinical laboratories that operate LC-MS/MS improve the accuracy and reliability of their testing by providing guidance for quality assurance and improvement, based on a collection of existing guidelines and expert opinions from the literature.