1/19. Reversible monoclonal lymphadenopathy in autoimmune lymphoproliferative syndrome with functional FAS (CD95/APO-1) deficiency.The FAS (CD95/APO-1) receptor and its ligand play an important role in the initiation of apoptosis under many physiologic conditions. Loss of function mutations of the FAS gene have been described in lpr mice and in humans with autoimmune phenomena, recurrent lymphadenopathies, and hepatosplenomegaly. This syndrome is now called autoimmune lymphoproliferative syndrome type I (ALPS I). Recently, patients with similar clinical symptoms due to a functional FAS deficiency without FAS gene mutations have been distinguished. This disease has been termed autoimmune lymphoproliferative syndrome type II (ALPS II) or autoimmune lymphoproliferative disease (ALD). This report is the first description of the lymph node pathology and immunohistochemistry in a patient with ALPS II. After recurrent bacterial infections, a 4-year-old child developed cervical giant lymphadenopathy suggesting lymphoma. Lymph node histology resembled the findings in Epstein Barr virus-associated posttransplant atypical lymphoproliferations. Confluent sheets of immunoblasts, however, showed a monoclonal expression of IgG/lambda and a monoclonal rearrangement of the JH chain. The same clone was also present in the peripheral blood. Although high-grade lymphoma could not be excluded, the patient's parents insisted on the patient's leaving the hospital with only antibiotic treatment. Surprisingly, the giant lymphadenopathy completely resolved within 7 weeks, and the clone was no longer detectable in the peripheral blood. Twelve months later the patient was still free from lymphoma and was doing well. Retrospectively, transient monoclonal B-cell populations could be identified in an archival frozen blood sample taken when the patient was 3 years old. Increased FAS-independent spontaneous apoptosis was a feature of the patient's lymphocytes and could be the molecular basis for self-elimination of B-cell clones. We conclude that the diagnosis of a FAS-FAS-L deficiency should be considered in children with an otherwise unexplained atypical lymphoproliferation and that a diagnosis of lymphoma in patients with functional FAS deficiency should be made with considerable reservation.- - - - - - - - - - ranking = 1keywords = still (Clic here for more details about this article) |
2/19. A case of resectable lung adenocarcinoma associated with sarcoidosis.A 71-year-old woman with uveitis was referred to our hospital for further examination of the possible underlying diseases. In roentgenological examination with plain X-ray and CT scan, hilar and mediastinal lymphadenopathy and a mass shadow in the right upper lung field was observed, whereas fibrotic changes were not obvious in both lung fields. Transbronchial lung biopsy with fiberoptic bronchoscope revealed granulomatous interstitial pneumonia. CD4-positive lymphocytes were increased in bronchoalveolar lavage. The patient was diagnosed as having sarcoidosis. Subsequently, right upper lobectomy was performed, and Stage I lung adenocarcinoma was diagnosed. The patient is under follow up without medication and the disease has been stable for two years. A relationship between epithelioid granulomatosis and malignant diseases is discussed and a review of the literature is given. Since it is still controversial as to the incidence of malignant diseases in sarcoidosis patients, it is important to accumulate data on these associations.- - - - - - - - - - ranking = 1keywords = still (Clic here for more details about this article) |
3/19. Lymph node lesion in adult-onset Still's disease resembling peripheral T-cell lymphoma: a report of three cases.adult-onset Still's disease (AOSD) is known to be a cause of fever of unknown origin. We describe the clinicopathologic, immunohistologic, and genotypic features of 3 patients with lymph node lesions from AOSD, which posed a serious diagnostic difficulty from peripheral T-cell lymphomas. The patients were 22-, 26-, and 63-year-old Japanese women. At the onset of disease, all patients had multicentric lymphadenopathy in association with clinical and laboratory findings suggestive of a malignant lymphoma. None of the patients developed malignant lymphomas during the follow-up period. Histologically, the lesions were characterized by paracortical hyperplasia with prominent vascular proliferation. In the paracortical area, there was a mixed infiltrate including small-to-medium-sized lymphocytes, variable numbers of eosinophils, plasma cells, and B immunoblasts. polymerase chain reaction analysis demonstrated that neither clonal rearrangement of the T-cell receptor gamma-chain gene nor immunoglobulin heavy-chain rearrangement was detected in any patient. Although AOSD appears to be a rare systemic inflammatory disorder, the lymph node lesion should be added to the differential consideration of benign lymph node lesions simulating node-based peripheral T-cell lymphoma.- - - - - - - - - - ranking = 0.83363827447546keywords = adult-onset (Clic here for more details about this article) |
4/19. Treatment of South American blastomycosis (paracoccidioidomycosis) with miconazole by the oral route: an on-going study.Results are presented from 12 patients of both sexes, whose ages range from 34 to 65 years, observed over a period of 16 months and receiving miconazole treatment for South-American blastomycosis. Ten of them were being treated for the first time and the remaining 2 presented relapses after treatment with other chemotherapeutic agents. The blastomycotic lesions were localized in skin, mucosa, lung and lymphatics. The drug was administered orally, at a dosage of 1 g three times daily. One relapsing patient had his first daily oral dose replaced by an intravenous dose of 200 mg of miconazole diluted in 250 ml of glucose solution during the initial four weeks. The criterion for diagnosis was finding of the fungus in sputum and/or in the material collected through scrapings or biopsy of the lesions. All patients were treated in hospital until the cure was apparent, clinical examinations being conducted weekly and bi-weekly. Bi-weekly laboratory examinations were performed to evaluate the patient's tolerance to the drug. All treated patients presented complete healing of cutaneomucous lesions (mean time: 4 weeks) and involution of lymphatic and pulmonary features (mean time: 4 months). Two of them still presented palpable lymphatics after 5 months of treatment, in spite of the cure of mucosal and pulmonary lesions. Monitoring the progress of the cured patients was and is being carried out quarterly. Currently, this observation period ranges from three to 15 months. Nine patients are still returning and none have shown the reappearance of mycotic lesions. Diarrhoea was the only side-effect observed after a period of therapy, but it was easily controlled by specific medication (kaolin, pectin, &c.). In a single case the treatment was interrupted because of this symptom.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 2keywords = still (Clic here for more details about this article) |
5/19. Syphilitic cervical lymphadenopathy.syphilis is well known as a great mimic, however it is not recognized as a cause of cervical lymphadenopathy. We report a case of a 21-year-old man who presented with marked unilateral cervical lymphadenopathy. He had no evidence of oropharyngeal chancres, skin or genital lesions. Computed tomography (CT) and magnetic resonance (MR) images showed multiple cervical lymphadenopathy, and serologic tests for syphilis were positive. syphilis should still be a factor for consideration in the differential diagnosis of cervical lymphadenopathy.- - - - - - - - - - ranking = 1keywords = still (Clic here for more details about this article) |
6/19. Massive abdominal lymphadenopathy in hairy cell leukaemia: a report of 12 cases.Lymphadenopathy is an uncommon finding in hairy cell leukaemia (HCL). We report 12 HCL patients in whom relapse was associated with massive abdominal lymphadenopathy. All but one had long-standing HCL (range 3-25 years; median 10 years); in one it was discovered at presentation. Nine patients had been splenectomized and seven had previously been treated with 2'deoxycoformycin (DCF) and/or alpha-interferon (alpha IFN): three had achieved complete remission and four a partial response. The computerized tomography (CT) scan appearances were similar in all cases with a primary lymph node mass centred around the coeliac axis and involving upper para-aortic and retropancreatic regions. histology and/or cytology confirmed nodal involvement by HCL in six patients. Large immature hairy cells were seen in both lymph nodes and bone marrow, suggesting a degree of transformation. Nine patients were treated with DCF: one had complete resolution, six responded with 50-90% reduction of the lymphadenopathy, one did not respond and one is still on treatment; alpha-IFN was used concomitantly or sequentially in two of the responders. One responding patient died of sepsis after four injections of DCF. Three patients received either alpha- or beta-IFN alone with no response. One elderly patient was not treated. Abdominal lymphadenopathy could be part of the natural history of HCL and/or may represent a transformation analogous to that seen in other low-grade lymphoproliferative disorders. Routine abdominal CT scanning should be part of the work up of all patients with HCL.- - - - - - - - - - ranking = 1keywords = still (Clic here for more details about this article) |
7/19. Sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease). Clinico-pathological analysis of a paediatric case.Histochemical and immunohistochemical studies performed in only a few cases of sinus histiocytosis with massive lymphoadenopathy (SHML) indicated that SHML cells belong to the macrophage--histiocyte system, though their exact origin is still uncertain. We analyzed the morphological, antigenic and enzymatic characteristics of the histiocyte-like cells in one paediatric case of SHML (also named Rosai-Dorfman disease). The SHML cells expressed the S-100 protein, lectins concanavalin a, peanut agglutinin and monocyte-macrophage related antigens CD 11c, CD 14, CD 33, CD 68 and LN 5. Reactivity with other anti-macrophage antibodies (MAC387, lysozyme, alpha-1 anti-chymotrypsin) was variable. The CD1a antigen was present only in scattered cells, whereas HLA-DR and the HLA-DR associated invariant chain were absent. Cytochemistry demonstrated an intense activity of acid phosphatase and non specific esterase of SHML cells. A large amount of medium sized mononuclear cells were located in the sinuses and intersinusoidal tissue. Our findings suggest that SHML cells have intermediate features between phagocytes and langerhans cells/interdigitating reticulum cells. The heterogeneity of marker expression on SHML cells might be related to the local content of factors (e.g., cytokines), capable of modulating the phenotype of monocyted and derived cells.- - - - - - - - - - ranking = 1keywords = still (Clic here for more details about this article) |
8/19. Lymphadenopathy in adult-onset Still's disease mimicking peripheral T-cell lymphoma.Lymphadenopathy (LAP) that is seen in adult onset Still's disease (AOSD) may be confused with lymphoma. Here we present a patient with AOSD and with LAP that histopathologically mimicked T-cell lymphoma.- - - - - - - - - - ranking = 0.83363827447546keywords = adult-onset (Clic here for more details about this article) |
9/19. Nail changes during docetaxel containing combination chemotherapy.Nail toxicity following systemic chemotherapy is common. Onychopathy during the period of neutropenia following chemotherapy may cause subungual abscesses and serious infection. Despite taxoid-related toxicity being increasingly reported since 2000, there are still phase II systemic chemotherapy studies using taxoid that have never mentioned nail changes. Recently, new criteria for the evaluation of nail toxicity have been suggested. The present report is the first of its kind, in korea, to describe a case of docetaxel-associated onychopathy, which improved following a reduction in the docetaxel dose.- - - - - - - - - - ranking = 1keywords = still (Clic here for more details about this article) |
10/19. Sinus histiocytosis with massive lymphadenopathy (SHML) prednisone resistant but dexamethasone sensitive.Sinus histiocytosis with massive lymphadenopathy (SHML) is a histiocytic disorder affecting children and adults. It usually presents as markedly enlarged lymph nodes that require surgical biopsy for confirmation. This lesion is usually self-limited but can present in areas that can cause significant morbidity or disfigurement. We report a case that required therapy due to the severe disfigurement but was resistant till treated with dexamethasone. This case illustrates that SHML may be resistant to prednisone but still be sensitive to dexamethasone.- - - - - - - - - - ranking = 1keywords = still (Clic here for more details about this article) |
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