There have been a few reports of hemophagocytic lymphohistiocytosis (HLH) with chromosomal abnormalities. Clonal chromosomal abnormalities in HLH patients are usually found in association with hematologic malignancies and rarely with epstein-barr virus (EBV) infection. Here, we report a fatal case of HLH with clonal karyotype abnormalities. A 75-yr-old man was admitted with persistent anorexia and high fever. Laboratory data revealed pancytopenia, hypofibrinogenemia, hyperferritinemia, prolonged prothrombin time and activated partial thromboplastin time, and marked elevated level of serum transaminases. In real time-PCR using whole blood, EBV DNA was not detected but cytomegalovirus (CMV) DNA was detected. The bone marrow aspiration smear showed hyperplasia of mature histiocytes with prominent hemophagocytosis. In chromosomal analysis of bone marrow aspirates, complex chromosomal abnormalities were found. In spite of steroid pulse therapy and antibiotic treatment, he died of disseminated intravascular coagulopathy.
Anorexia ; Bone Marrow ; Chromosome Aberrations ; Cytomegalovirus ; DNA ; Fever ; Hematologic Neoplasms ; Herpesvirus 4, Human ; Histiocytes ; Humans ; Hyperplasia ; Karyotype ; Lymphohistiocytosis, Hemophagocytic ; Pancytopenia ; Partial Thromboplastin Time ; Prothrombin Time ; Transaminases

BACKGROUND: The use of automated techniques reduces the impact of human errors in blood banking and it improves the standardization and the quality of the achieved results. Erythrocyte Magnetized Technology (EMT) is now being widely used due to its simplicity and efficiency for detecting alloantibody. We evaluated the antibody screening test of the QWALYS-3 (DIAGAST, Loos Cedex, France). METHODS: The evaluation focused on antibody screening using the QWALYS-3 as compared to the standard manual tube method and the Ortho BioVue system in clinical samples (n=100) and frozen stored samples (n=64), which had RBC alloantibody. RESULTS: Using the manual tube method, the sensitivity of antibody screening was 100% by the QWALYS-3 and 42.8% by the Ortho BioVue in the clinical samples (n=7) and 2 results were discrepant by the QWALYS-3 for negative samples. For the known antibodies from the frozen stored samples (n=64) this correspondence rate amounted to 93.7% (n=60). CONCLUSION: The QWALYS-3 system displayed a good match rate with the Ortho BioVue system (92%). It also showed reliable results for the general accuracy when compared to the manual method (concordance rate: 98%). The QWALYS-3 system will facilitate the automation of routine antibody screening with high reliability, sensitivity and specificity compared to the standard manual methods.
Antibodies ; Automation ; Blood Banks ; Cephalosporins ; Erythrocytes ; Humans ; Magnets ; Mass Screening ; Sensitivity and Specificity

BACKGROUND: Unexpected antibody screening and identification tests are very important for safe blood transfusion. The micro-column agglutination test (MCAT) is widely used due to its simplicity and efficiency for detecting alloantibodies. We analyzed the frequency of unexpected antibodies at three university hospital blood banks, which use two different MCAT systems. METHODS: From February 2002 to December 2009, a total of 295,876 unexpected antibody screening tests were performed at three university hospital blood banks. Two hospital blood banks (Anam and Ansan Hospitals) used the DiaMed-ID system (DiaMed Ag, Switzerland) and the other (Guro Hospital) used the Ortho BioVue system (Ortho-Clinical Diagnostics, USA) for antibody screening and identification tests. RESULTS: The rates of detecting unexpected antibodies on screening test based on the 'tests performed' and the 'persons tested' were 1.16% per test and 0.96% per person in Korea University Guro Hospital, 0.65% and 0.41% in Korea University Anam Hospital and 0.76% and 0.57% in Korea University Ansan hospital, respectively. There were significant differences in the frequencies based on the two different systems (P<0.001). Among the warm antibodies, Rh antibodies were more frequently detected by the DiaMed-ID system, and Lewis antibodies were most frequently detected by the Ortho BioVue System. CONCLUSION: We should carefully interpretate the frequency of unexpected antibodies in the Korean population because the frequencies of unexpected antibodies are different according to different employed micro-column agglutination systems.
Agglutination ; Agglutination Tests ; Antibodies ; Blood Banks ; Blood Transfusion ; Humans ; Isoantibodies ; Korea ; Mass Screening ; Phenytoin