1/317. Clonal expansion of gammadelta-T lymphocytes in an HTLV-I carrier, associated with chronic neutropenia and rheumatoid arthritis. We report on an HTLV-I carrier showing clonal proliferation of gammadelta-T lymphocytes associated with chronic neutropenia and rheumatoid arthritis (RA). A 75-year-old Japanese woman had a 20-year history of RA and was found to have neutropenia and lymphocytosis by routine examinations. Her white cell count was 5,800/microl with 89% lymphocytes. The proliferating gammadelta-lymphocytes did not show the typical morphology of large granular lymphocytes (LGL) and were positive for CD3, TCRdelta1, and HLA-DR but negative for CD4, CD8, and deltaTCS1. Clonally rearranged TCRgamma-chain (Jgamma) and TCRbeta-chain (Cbeta1) genes were detected by Southern blot analysis. Clonality of these proliferating gammadelta-T cells was confirmed by CDR3 size analysis for the TCRdelta-chain. Anti-HTLV-I antibody was positive and the pX region of HTLV-I proviral dna was detected by PCR analysis, but clonal integration of HTLV-I proviral dna was not detected by Southern blotting analysis. The patient's clinical course has been stable, except for infrequent infectious episodes. The association of HTLV-I/II infection with T-LGL leukemia has been reported by several groups, although most cases exhibit TCRalphabeta type T cells. Analysis of the junctional sequence of TCR on T-LGL leukemia cells may clarify the role of HTLV-I/II infection in clonal T-cell proliferation. ( info) |
2/317. HIV-associated parotid lymphoepithelial cysts. BACKGROUND: An outstanding feature of the diffuse infiltrative lymphocytosis syndrome, or DILS, a subset of hiv-1 disease, is asymptomatic bilateral parotid swelling. Recognition of the entity is important because people with this disease will seek routine dental care. CASE DESCRIPTION: The authors present a classic case of DILS. The patient exhibited bilateral parotid swellings caused by lymphoepithelial cysts, cervical lymphadenopathy, a CD8 circulating lymphocytosis and a CD8 lymphocytic infiltration into the labial salivary glands. A right superficial parotidectomy had been performed several years previously. However, no intervention was advised for the remaining left parotid because of its benign course. CLINICAL IMPLICATIONS: Since patients with DILS can develop lymphomas, periodic observation is mandatory. Any change in the growth pattern requires that a fine-needle aspiration biopsy be performed. ( info) |
3/317. Linear IgA disease associated with lymphocytic colitis. A 66-year-old woman presented with a bullous skin eruption and chronic diarrhoea. Lesional skin showed subepidermal blistering, and direct immunofluorescence of perilesional skin revealed linear deposits of IgA at the dermoepidermal junction, establishing a diagnosis of linear IgA disease (LAD). Chronic watery diarrhoea complicated by substantial loss of body weight preceded the skin eruption for several months. On endoscopy, the colon appeared macroscopically normal. On histology, the colon mucosa showed increased numbers of intraepithelial lymphocytes and infiltrates of mononuclear cells in the lamina propria, indicative of lymphocytic colitis. Treatment with methylprednisolone and dapsone led to complete clearing of the bullous skin eruption and marked improvement of the patient's diarrhoea. Gastrointestinal disorders such as lymphocytic colitis have rarely been reported in patients with LAD. Whether the simultaneous occurrence of these two diseases is coincidental or due to related pathogenetic mechanisms remains to be seen. ( info) |
4/317. paraproteins and monoclonal expansion of CD3 CD8 CD56-CD57 T lymphocytes in a patient with HIV infection. An expansion of CD8 lymphocytes associated with a monoclonal rearrangement of the T-cell receptor gamma locus was found in a woman with hiv-1 infection. A subpopulation of HIV-positive patients display an unusual response to HIV infection characterized by a persistent marked CD8 lymphocytosis, the presence of which appears to be associated with an improved long-term prognosis. This condition is thought to represent a florid immune response to an ongoing viral infection which may be HIV itself, and suggests that monoclonal proliferation of CD8 lymphocytes does not imply the presence of an underlying malignant process. ( info) |
5/317. Plasma cell mucositis: a review and case report. The simultaneous involvement of the mucous membranes of the oral cavity and upper aerodigestive tract by lesions characterised clinically by an intensely erythematous, lobulated surface and histologically by a dense connective tissue infiltrate composed of non-neoplastic plasma cells may be called plasma cell mucositis. We present a review of the literature, consisting of 14 cases, outlining the multifocal site distribution, chronicity and systemic background that distinguish this entity and report a single case with confirmation of the polyclonal nature of the plasma cell infiltrate using gene rearrangement studies. ( info) |
6/317. Persistent Sweet's syndrome occurring in a child with a primary immunodeficiency. Sweet's syndrome (SS) occurs most commonly in association with inflammatory or neoplastic disorders. Only rarely has it been associated with immunodeficiency disorders. We describe a child with a T-cell immunodeficiency who had a persistent neutrophilic dermatosis that was histologically and clinically consistent with SS. SS associated with immunodeficiencies may occur as a reaction to an underlying infection or a defect in immunoregulation. Such patients, however, may not be able to produce the classic fever and neutrophilia associated with SS. They may fail to respond to standard treatment for SS and may suffer a prolonged and persistent course. ( info) |
7/317. CD5 persistent polyclonal B-cell lymphocytosis in a male. Persistent polyclonal B-cell lymphocytosis with binucleate lymphocytes is a rare lymphoproliferative syndrome of uncertain cause that is strongly associated with HLA-DR7 positivity, cigarette smoking, and female sex. As yet, there is no explanation for the strong sex predilection. We report the third case of persistent polyclonal B-cell lymphocytosis in a male. Other notable findings in this case are lack of HLA-DR7 and strong positive CD5 markers in the polyclonal B-cell population. To our knowledge, CD5 expression has not been mentioned or reported in association with this syndrome. ( info) |
8/317. Lymphocytic hypophysitis associated with dacryoadenitis: an autoimmunologically mediated syndrome. We report a rare case of lymphocytic hypophysitis followed by dacryoadenitis. Lymphocytic hypophysitis is a rare disease that can easily be mistaken for neoplastic proliferation. Because combination with rheumatoid arthritis, thyroiditis, or pernicious anemia is frequent, an immunological pathogenesis is likely. ( info) |
9/317. Aicardi-Goutieres syndrome: a genetic microangiopathy? Aicardi-Goutieres syndrome (AGS) (McKusick 225750) is an autosomal recessive disease with onset in the 1st year of life, resulting in progressive microcephaly, calcification of cerebral white matter, thalamus and basal ganglia, generalized cerebral demyelination and a chronic low-grade CSF lymphocytosis, without evidence of infection. We report the autopsy of a patient who died with this disorder at the age of 17 years. Findings were severe microencephaly, diffuse but inhomogeneous cerebral white matter loss with associated astrocytosis, calcific deposits in the white matter, thalami and basal ganglia. neocortex and cerebellar cortex were affected by wedge-shaped microinfarctions. Small vessels showed calcification in the media, adventitia and perivascular spaces. These findings are similar to some previous publications that in retrospect may have been AGS, but this is the first reported cerebral microangiopathy in which the diagnosis AGS was made during lifetime. This report provides evidence that microangiopathy plays a significant role in the pathogenesis of AGS. ( info) |
10/317. Lymphocytic hypophysitis presenting with diabetes insipidus: MR findings. It has been thought that neurohypophysial involvement manifesting as central diabetes insipidus in lymphocytic hypophysitis is rare. The radiological and clinicopathological features of two cases represent a variant of lymphocytic adenohypophysitis and/or lymphocytic infundibulo-neurohypophysitis are discussed. ( info) |