Methotrexate (MTX) has been established as a standard disease-modifying anti-rheumatic drug. If adequate disease control is achieved for a reasonable period of time, tapering the MTX dosage is recommended because the chronic use of MTX can result in opportunistic infection. We present here a case of a woman with rheumatoid arthritis taking MTX, and the woman developed actively caseating endobronchial Mycobacterium intracellulare disease with pulmonary infiltrations. After discontinuing the MTX, the patient was able to tolerate 18 months of antimycobacterial treatment without flare ups of rheumatoid arthritis, and she completely recovered from nontuberculous mycobacterial respiratory disease.
Nontuberculous Mycobacteria; Tuberculosis, Pulmonary; Methotrexate; Arthiritis, Rheumatoid; Bronchial Diseases
A 31-year-old Korean male presented with altered consciousness and severe headache. Brain MRI delineated focal leptomeningeal enhancement without any intracerebral lesions. Diagnosis was made based on a brain biopsy showing anaplastic large cell lymphoma (ALCL), immunohistochemical stains revealing positivity for anaplastic lymphoma kinase (ALK) and an absence of involvement in any other organs; specifically, the primary central nervous system ALK+ALCL. Complete remission was achieved following 5 cycles of systemic chemotherapy with a high dose of Methotrexate and a simultaneous 7 cycles of intrathecal triple chemotherapy. Diagnosis of primary leptomeningeal ALK+ALCL is challenging given its rarity and non-specific symptoms along with non-pathognomonic radiologic findings. We present the first case of primary leptomeningeal ALK-positive ALCL where the clinical course, pathologic characteristics and treatment modality are described as well as a review of literature.
ALK-positive; primary; CNS; anaplastic large-cell lymphoma; leptomeningeal
A 36-year-old woman presented with erythematous confluent macules on her whole body with fever and chills associated with jaundice after 8 months of dapsone therapy. Her symptoms had developed progressively, and a physical examination revealed bilateral cervical lymphadenopathy and splenomegaly. Excisional biopsy of a cervical lymph node showed effacement of the normal architecture with atypical lymphoid hyperplasia and proliferation of high endothelial venules compatible with angioimmunoblastic T-cell lymphoma. However, it was assumed that the cervical lymphadenopathy was a clinical manifestation of a systemic hypersensitivity reaction because her clinical course was reminiscent of dapsone-induced hypersensitivity syndrome. A liver biopsy revealed drug-induced hepatitis with no evidence of lymphomatous involvement. Intravenous glucocorticoid was immediately initiated and her symptoms and clinical disease dramatically improved. The authors present an unusual case of cervical lymphadenopathy mimicking angioimmunoblastic T-cell lymphoma as an adverse reaction to dapsone.
Pseudolymphoma; Dapsone therapy
Extranodal natural killer/T-cell lymphoma, nasal type (nasal ENKTL) is a distinct clinicopathologic entity of lymphoid tumors with variable size and differentiation of tumor cells. Nasal ENKTL is related to infection of the tumor cells with Epstein-Barr virus (EBV) and virtually all cases contain monoclonal episomal EBV DNA and detectable EBV encoded small nuclear RNAs (EBERs). Several clinical factors are known for their relation to the prognosis, but histopathologic prognostic factors of nasal ENKTL have not yet been well established. We evaluated the prognostic value of the longest nuclear diameter of EBER+ tumor cells (NDTC) along with the result of CD30 expression. Twenty two patients with newly diagnosed nasal ENKTL were evaluated regarding clinicopathologic characteristics. NDTC was measured using a computerized image analysis system. The results were expressed as the mean diameter of ≥ 50 cells in a patient. Median of the mean NDTC of the patients was 7.32 μm (5.15-11.27). Patients with larger mean NDTC (≥ 7.35 μm) had a poorer event-free survival (EFS) than those with smaller mean NDTC (<7.35 μm; p = 0.024) and had a tendency of inferior overall survival (OS) (p = 0.08). Patients with CD30 expression had a inferior EFS (p = 0.018) and OS (p = 0.011) compared those without CD30 expression. The NDTC of EBV infected tumor cell and CD30 expression had relation to survival in the current exploratory analysis.
Extranodal NK/T-cell lymphoma; nasal type; epstein-barr virus; CD30; prognosis; nuclear diameter
A 62-yr-old man presented with a 5-yr history of intermittent abdominal distention and pain. These symptoms persisted for several months and subsided without treatment. A diagnosis of suspected small bowel lymphoma was made based on plain radiograph and computerized tomogram findings, and he was referred to our institution for further evaluation. Segmental resection of the small intestine was performed and the diagnosis of marginal zone B-cell lymphoma associated with amyloidosis was made. This is the first case of marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) in the small intestine associated with amyloidosis in Korea.
Gastrointestinal Tract; Lymphoma; Lymphoma, B-Cell, Marginal Zone; Amyloidosis
Intrabdominal actinomycosis is difficult to diagnose preoperatively. This chronic infection has a propensity to mimic many other diseases and may present with a wide variety of symptoms. The aim of this study was to evaluate the characteristic clinical features with review of the literature.
Materials and Methods
We retrospectively analyzed 22 patients with intrabdominal actinomycosis between January 2000 and January 2006.
There were two men and 20 women with a mean age of 42.8 years (range, 24 - 69). Twelve patients presented with masses or abdominal pain, whereas 3 patients presented with acute appendicitis. The rate of performing an emergency surgery was 50% due to symptoms of peritonitis. The mean size of tumor was 5.5 cm (range, 2.5 - 11.0). Sixty percent (n = 12) of female patients had intrauterine device (IUD). The average time to definite diagnosis was 10.6 days.
Intrabdominal abdominal actinomycosis must first be suspected in any women with a history of current or recent IUD use who presents abdominal pain. If recognized preoperatively, a limited surgical procedure, may spare the patient from an extensive operation.
Actinomycosis; surgery; intrauterine device
Fulminant Epstein–Barr virus (EBV+) T-cell lymphoma in immunocompetent elderly patients is rare and its character has not been well defined. This study analyzed the clinicopathological features of five elderly patients (group A: 50–84 years) and compared them with those of eight children and young adult patients with systemic T-cell lymphomas (group B: 10–34 years). Group A more commonly presented with generalized lymphadenopathy (n = 3) than did group B (n = 1). Chronic active EBV infection (n = 3) and hydroa vacciniforme-like eruptions (n = 1) were seen in group B, while group A showed no evidence of chronic EBV infection, but did show chronic hepatitis B or C virus infections (n = 3). The histological and immunophenotypical findings were similar. All patients died within 1 to 14 months of diagnosis. These findings suggest that EBV+ T-cell lymphoma in elderly patients is a unique disease with an underlying derangement of T-cell immunity and failure to eradicate infected virus. Additional factors related to senility may play a role in the disruption of homeostasis between the virus and the host’s immune system.
Epstein–Barr virus; Lymphoma; T-cell
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.
CD99 protein, human; Lymphoma, Non-Hodgkin; Lymphoma, Large-Cell, Ki-1
Collagenous gastritis, a counterpart of collagenous colitis, is an extremely rare disorder. The first case of collagenous gastritis in a Korean boy in his pre-teens who had been receiving treatment for refractory iron deficiency anemia has been reported. The patient had been suffering from intermittent abdominal pain, recurrent blood-tinged vomiting and poor oral intake. The gastric endoscopy revealed diffuse cobble-stone appearance of the mucosa with easy touch bleeding throughout the stomach but no abnormalities in the esophagus, duodenum, and colon. Pathologic examination of the gastric biopsies from the antrum, body and cardia showed a subepithelial collagen deposition with entrapped dilated capillaries, moderate infiltrates of lymphoplasma cells and eosinophils of the lamina propria, and marked hypertrophy of the muscularis mucosa. The collagen deposition appeared as discontinuous bands with focally irregular extension into the deeper part of the antral mucosa. It measured up to 150 µm. Helicobacter pylori infection was not detected. The biopsies from the duodenum, esophagus and colon revealed no pathologic abnormalities.
Gastritis; Gastritis, Collagenous; Child; Endoscopy