Severe chronic active Epstein-Barr virus (EBV) infection is a rare and life-threatening illness. Although the criteria for diagnosis include chronic or recurrent infectious mononucleosis-like symptoms lasting more than 6 months and high titers of anti-EBV antibodies, clinical and laboratory findings may be heterogeneous and flexible application of those criteria is necessary in cases showing typical clinical and pathologic findings. We report a case of severe chronic active EBV infection in a 62-yr-old female patient who showed classical clinical findings with infiltration of EBV-infected T lymphocytes in the bone marrow, spleen, and lymph nodes, and died four months after presentation.
Antigens, CD3/biosynthesis ; Antigens, CD4/biosynthesis ; Antigens, CD8/biosynthesis ; Bone Marrow Cells/virology ; Epstein-Barr Virus Infections/*diagnosis/*mortality ; Female ; Herpesvirus 4, Human/genetics ; Human ; Immunohistochemistry ; Lymph Nodes/virology ; Lymphocytes/metabolism ; Middle Aged ; Organ Weight ; Spleen/pathology/virology ; Support, Non-U.S. Gov't ; T-Lymphocytes/virology

To verify the spectrum of CD99-expressing lymphoid malignancy, an immunohistochemical study for CD99 was carried out in 182 cases of non-Hodgkin's lymphoma, including 21 lymphoblastic lymphomas, 11 small lymphocytic lymphomas, 9 mantle cell lymphomas, 12 follicular lymphomas, 37 diffuse large B cell lymphomas, 18 Burkitt's lymphomas, 28 NK/T-cell lymphomas, 8 angioimmunoblastic T-cell lymphomas, 23 peripheral T-cell lymphomas, unspecified, and 15 systemic anaplastic large cell lymphomas. CD99 was positive in all T-lymphoblastic lymphomas and in 60% of B-lymphoblastic lymphomas. Majority of T and NK cell lymphomas were negative for CD99, except anaplastic large cell lymphomas (ALCLs). Eight of 15 cases (54%) of ALCLs reacted with anti CD99 antibody. Seven of 10 (70%) ALK positive ALCLs expressed CD99, whereas only 1 of 5 (20%) ALK negative ALCLs were positive. Of the mature B-cell lymphomas, 5.4% (2/37) of diffuse large B cell lymphomas and 11.1% (2/18) of Burkitt's lymphomas expressed CD99. In conclusion, CD99 is infrequently expressed in mature B and T cell lymphomas, except ALK-positive ALCL. High expression of CD99 in ALK-positive ALCL is unexpected finding and its biologic and clinical significances have yet to be clarified.
Antigens, CD/*metabolism ; Blotting, Western ; Cell Adhesion Molecules/*metabolism ; Humans ; Immunohistochemistry ; Lymphoma, Large-Cell/enzymology/*immunology/pathology ; Lymphoma, Non-Hodgkin/enzymology/*immunology/pathology ; Protein-Tyrosine Kinase/immunology/*metabolism

Hemimasticatory spasm is a rare disorder that often accompanies facial hemiatrophy and is characterized by spasm in one or more of the jaw-closing muslces supplied by the fifth cranial nerve. We report a clinical and electrophysiological observation of a patient of hemimasticatory spasm, who presented involuntary spasm on masseter muscle and hemiatrophy of the face on the right side for 7 years.
Facial Hemiatrophy ; Humans ; Masseter Muscle ; Spasm* ; Trigeminal Nerve

Blastic natural killer (NK) cell lymphoma is a rare neoplasm characterized by blastoid tumor cells expressing CD4 and CD56, with predominant skin involvement. Although this tumor has been regarded as a neoplasm related to NK cell, recent studies suggested that it is derived from plasmacytoid dendritic cells, but not from NK cell. Herein we report 4 cases of CD4+CD56+ lineage marker- blastic NK cell lymphomas with a review of literatures. The patients were 3 men and one woman. Three of them were young (17, 18, and 22 yr old). Three patients had skin lesions, at initial presentation in two patients and during the course of disease in other patient. Histologically, tumors consisted of monotonous medium to large blastoid cells showing no necrosis, angiocentric growth or epidermotrophism. All four tumors were CD4+ and CD56+. Three expressed CD68 antigen. Lineage specific markers for B- and T cell were negative. All tumors did not express myeloperoxidase. T-cell receptor gene rearrangement, EBV, CD13 and CD33 were negative. In one patient, tumor cells arranged in Homer-Wright type pseudorosette and expressed terminal deoxynucleotidyl transferase(TdT). Despite the standard lymphoma chemotherapy, the tumors, except one lost during follow-up, progressed and relapsed. The patients died 8-60 months after diagnosis.
Adolescent ; Antigens, CD4/*analysis ; Antigens, CD56/*analysis ; Cell Lineage ; Female ; Humans ; Killer Cells, Natural/immunology/*pathology ; Lymphoma, T-Cell/immunology/*pathology ; Male ; Middle Aged

The objective of this study is to identify any differences in disease status between foreign patients staying in Korea and Korean patients. The study results are : First, more foreign stayers than Korean in their 20's and 30's seek medical services but those after age of 60 show the opposite pattern. This difference seems to feflect cultural difference in the concept of medical services and health seeking behavior. Second, foreign pateints were much more frequently(almost threee times) diagnosed as having an acute disease, and the fact that the most common diseases were "diseases of the respiratory system" can suggest the seriousness of air pollution problem in Korea. And, the fact that foreign stayers, especially those from developed countries, tend to take more periodic general physical examina-tion indicated that their health maintenance efforts place emphasis on prevention. Foreign patients seek more medical services during the spring and summer time when compared to Korean patients, and Korean patients visited the clinic more frequently during the autum time. Also, the review of the number of outpatients seeking medical services and the average duration of outpatient follow-up mainten-ance after discharge suggest that foreigners suffer less severe diseases than Koreans do. Finally, in contrast to the usual practice of Koreans of coming to the center without prior calling for reservation, most foreign patients tend to make reservations before coming(usually a day before) in general. This is also reflective of cultural difference related to the way of utilizing medical services.
Acute Disease ; Air Pollution ; Developed Countries ; Emigrants and Immigrants ; Follow-Up Studies ; Humans ; Korea ; Outpatients

Epstein-Barr virus (EBV) infection is associated with various lymphoproliferative diseases, including Hodgkin lymphoma, extranodal NK/T cell lymphoma, aggressive NK cell leukemia, Burkitt lymphoma and post-transplant lymphoproliferative disorder. In the recent studies, the plasma EBV-DNA levels in patients with EBV-associated lymphoproliferative disease appeared to be correlated with the therapeutic response. Aggressive NK cell leukemia (ANKL) is a fatal disease that's characterized by high fever, lymphadenopathy, hepatosplenomegaly and frequent hemophagocytosis. No serological tumor marker for this malignancy has yet been identified for monitoring the disease and predicting the outcome. We experienced a case of aggressive natural killer cell leukemia in a 48-year-old man, and we serially monitored the plasma EBV DNA load by performing real time quantitative PCR assay. Serial measurements of the plasma EBV DNA level during therapy showed a close correlation between the clinical response and the changes in the plasma EBV DNA titers.
Burkitt Lymphoma ; DNA* ; Fever ; Herpesvirus 4, Human* ; Hodgkin Disease ; Humans ; Killer Cells, Natural* ; Leukemia* ; Lymphatic Diseases ; Lymphoma ; Lymphoproliferative Disorders ; Middle Aged ; Plasma ; Polymerase Chain Reaction

PURPOSE: On the hypothesis that the acromion morphology is changed according to the its site, we identified the morphology of the acromion on the oblique slices of MRI and we investigated the association of the acromial shape with the clinical stages of rotator cuff disorder on the magnetic resonance (MR) images. In addition, we compared the acromion morphology on MRI and simple X-rays. MATERIAL AND METHODS: The MR images of seventy one patients with rotator cuff disorder and who underwent arthroscopic surgery were compared with that of a control group of sixteen patients who didn't have rotator cuff disorder on MRI. On three subsequent oblique sagittal slices from the lateral edge of the acromion (S1, S2 and S3), each acromion morphology on the MRI slices was classified according to Epstein et al: flat, curved or hooked. We investigated the changing parttern of the acromion shape and we compared the acromion shape on MRI and that on simple X-rays. We classified the rotator cuff tear by the severity: bursitis, partial thickness tear or full thickness tear. We investigated which acromial type on the MRI oblique slice was associated with the severity of rotator cuff disease. RESULTS: Changes of the acromial shape occurred in 54 patients (76.1%). The most frequent pattern was that the types are same on S1 and S2 and different on S3 (22 cases, 31.0%). The acromial type on S1 and S2 was significantly associated with the severity of rotator cuff disorder (p=0.001 and 0.022), respectively. There was no reliability of the acromial shape on MRI and roentgenography (p>0.05). CONCLUSION: The type of acromion changed from lateral to medial. Among the three positions, the shape of the acromion on S1 and S2 had meaningful correlation with the clinical stage of rotator cuff disorder. There was no statistical correlation of the acromial shape between MRI and simple X-ray.
Acromion ; Arthroscopy ; Bursitis ; Humans ; Magnetic Resonance Spectroscopy ; Rotator Cuff ; Shoulder

A 43-year-old male presented with a painless left testicular mass. The pathologic diagnosis of the radical orchiectomy specimen was peripheral T-cell lymphoma, unspecified (PTCL-u). According to the Ann Arbor staging system, his initial stage was III because of the right nasopharyngeal involvement. After first-line chemotherapy with four courses of the CHOP regimen and this was followed by involved-field radiotherapy, he achieved complete remission. Two months later, disease recurred to the left ciliary body of the left eye without evidence of involvement at other sites. Although the patient received intensive chemotherapy with autologous hema-topoietic stem cell transplantation, he ultimately died of leptomeningeal seeding. Because both the central nervous system (CNS) and the orbit are sanctuary sites for chemotherapy, orbital infiltration of lymphoma should prompt physicians to evaluate involvement of the CNS and to consider performing prophylactic intrathecal chemotherapy as a treatment option.
Adult ; Central Nervous System ; Ciliary Body ; Diagnosis ; Drug Therapy ; Eye Neoplasms ; Humans ; Lymphoma ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Male ; Orbit* ; Orchiectomy ; Radiotherapy ; Recurrence* ; Stem Cell Transplantation ; T-Lymphocytes* ; Testis

Intravascular lymphomatosis(IVL) which was first described by pfleger and Tappeiner in 1959 is rare malignancy characterized by neoplastic proliferation of lymphoid cell lineage within the vascular lumen with little or no adjacent parenchymal involvement. Its usual sites of involvement are central nervous system and skin or infrequently heart, lungs, pancreas, liver, spleen, kidney, adrenal glands, genitourinary tract, and bone marrow. Pulmonary involvement of IVL is not common. Symptoms of pulmonary involvement include dyspnea, cough and fever. Radiologicially, the disease is manifested with diffuse interstitial infiltrates. We report a recently experienced case of pulmonary intravascular lymphomatosis which was manifested with fever and chest pain.
Adrenal Glands ; Bone Marrow ; Central Nervous System ; Chest Pain ; Cough ; Dyspnea ; Fever ; Heart ; Kidney ; Liver ; Lung ; Lymphocytes ; Lymphoma ; Pancreas ; Skin ; Spleen

OBJECTIVE: The aim of this study was to provide normative data on the Literacy Independent Cognitive Assessment (LICA) and to explore the effects of age, education/literacy, and gender on the performance of this test. METHODS: Eight hundred and eighty-eight healthy elderly subjects, including 164 healthy illiterate subjects, participated in this study. None of the participants had serious medical, psychiatric, or neurological disorders including dementia. Bivariate linear regression analyses were performed to examine the effects of age, education/literacy, and sex on the score in each of the LICA cognitive tests. The normative scores for each age and education/literacy groups are presented. RESULTS: Bivariate linear regression analyses revealed that total score and all cognitive tests of the LICA were significantly influenced by both age and education/literacy. Younger and more-educated subjects outperformed older and illiterate or less-educated subjects, respectively, in all of the tests. The normative scores of LICA total score and subset score were presented according to age (60-64, 65-69, 70-74, 75-80, and > or =80 years) and educational levels (illiterate, and 0-3, 4-6, and > or =7 years of education). CONCLUSION: These results on demographic variables suggest that age and education should be taken into account when attempting to accurately interpret the results of the LICA cognitive subtests. These normative data will be useful for clinical interpretations of the LICA neuropsychological battery in illiterate and literate elderly Koreans. Similar normative studies and validations of the LICA involving different ethnic groups will help to enhance the dementia diagnosis of illiterate people of different ethnicities.
Aged* ; Dementia ; Diagnosis ; Education ; Ethnic Groups ; Humans ; Linear Models ; Nervous System Diseases