Cases reported "Lymphocytosis"

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1/20. 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.
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2/20. Differentiating hiv-1 parotid cysts from papillary cystadenoma lymphomatosum.

    BACKGROUND: patients with parotid cystic lesions may first be seen in the dental office. These conditions most often represent either papillary cystadenoma lymphomatosum, or PCL, or lymphoepithelial cysts associated with human immunodeficiency virus, or HIV, disease. The authors present a case report to illustrate the differential diagnosis. CASE DESCRIPTION: PCL represents a benign, usually unilateral, circumscribed parotid tumor with cystic elements. HIV-associated lymphoepithelial cysts of the parotid gland usually are seen bilaterally, create cosmetic concerns and are hallmarked by an associated cervical lymphadenopathy. Therapy for PCL demands surgical excision, while patients with HIV-associated lymphoepithelial cysts may be treated with antiviral therapy and undergo periodic monitoring by a physician. CLINICAL IMPLICATIONS: As a member of the health care team, the dentist must be familiar with head and neck swellings. Early clinical recognition of parotid swellings leads to successful treatment.
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3/20. Natural course of lymphocytic infundibuloneurohypophysitis.

    Natural course of lymphocytic infundibuloneurohypophysitis is poorly understood. A 49-year-old male had noticed being unnaturally thirsty since about two years previously. An enlargement of the pituitary stalk and pituitary gland was thus observed by MR at that time. However, no medical care had been given. Two years later, he was admitted to our hospital due to headache in addition to panhypopituitarism. The histologic features included T cell dominant lymphocytes infiltrating prominently the entire pituitary gland with a small amount of multinucleated giant cells, focal and small necrosis, cholesterin crystals and granuloma. Neither tuberculosis nor Langerhans histiocytosis were observed. In addition, the patient was found to have a unique massive well-encapusulated lesion in the sphenoid sinus, just below the pituitary fossa, consisting of serous fluid, normal columnar epithelium and submucosal fibrosis. This patient had a fairly typical clinical manifestation of lymphocytic infundibuloneurohypophysitis with invason of the posterior lobe and the stalk. The lesion became chronic and leaked to the sphenoid sinus. As a result, chronic hypophysitis with granuloma formation thus occurred. This case may show the course of this disease if not treated.
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4/20. CD4 T-lymphocyte-induced Epstein-Barr virus reactivation in a patient with severe hypersensitivity to mosquito bites and Epstein-Barr virus-infected NK cell lymphocytosis.

    BACKGROUND: Natural killer (NK) cell lymphocytosis associated with Epstein-Barr virus (EBV) infection often shows severe hypersensitivity to mosquito bites (HMB) characterized by intense local skin reactions and systemic symptoms such as high fever, lymphadenopathy, and hepatosplenomegaly. However, the induction mechanism of HMB is still unclear. OBSERVATIONS: We investigated a typical case of HMB with EBV-positive NK cell lymphocytosis. CD4 T cells dominantly infiltrated the site of the mosquito bite, while EBV-positive cells were few in comparison. CD4 T cells, but not CD8 T cells or NK cells, responded to the mosquito salivary gland extracts. Interestingly, coculturing of the NK cells and CD4 T cells activated by mosquito extracts induced expression of EBV lytic-cycle proteins in the NK cells. Furthermore, the expression of BZLF1, a viral lytic-cycle transactivator, was detectable at the skin lesion induced by scratch patch testing with mosquito extract. The EBV dna copy number levels in the plasma were elevated in systemic HMB symptoms compared with the normal condition. CONCLUSIONS: CD4 T cells are important for the primary skin reaction to mosquito bites and might play a key role in reactivation of latent EBV infection in NK cells. This viral reactivation contributed to the pathogenesis of the infectious mononucleosis-like systemic symptoms of HMB in our present case.
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5/20. Preoperative diagnosis of lymphocytic hypophysitis (adenohypophysitis) unresponsive to short course dexamethasone: case report.

    Lymphocytic hypophysitis (adenohypophysitis) is a rare lymphocytic infiltration of the pituitary gland that usually occurs during pregnancy. Because of its rarity, it has seldom been diagnosed preoperatively, and no trials of therapeutic treatment have been reported to date. We describe a 29-year-old woman with a pituitary mass and visual-field defects during pregnancy. The patient's pituitary profile revealed an abnormal thyroid axis and relatively low prolactin for her stage of pregnancy. This finding suggested adenohypophysitis, and the patient was given a trial course of dexamethasone. The progression of the visual-field defects, however, indicated that the steroids, both in dosage and duration, were not effective. Thus, the patient underwent a partial hypophysectomy for decompression. The pathology report confirmed adenohypophysitis, and steroids were continued for the remainder of the pregnancy, with slow resolution of the visual-field defects to normal. This report is the first case of adenohypophysitis where the diagnosis was suspected preoperatively, and a trial course of steroids was given. The steroids at the dosage and duration used were not effective. Further evaluation of the use of steroids in this disease is warranted.
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6/20. Lymphocytic hypophysitis with diabetes insipidus: improvement by methylprednisolone pulse therapy.

    Lymphocytic hypophysitis is a rare inflammatory disorder in the pituitary gland. The lesion is usually confined to the adenohypophysis. Although the involvement of the posterior pituitary gland or the stalk is rare, such patients with diabetes insipidus have been reported. Surgery has been used to make the definitive diagnosis. Recent studies suggest, however, that the pathologic diagnosis may not be necessary always. We reported a case of Lymphocytic hypophysitis managed by methylprednisolone pulse therapy. A 50-year-old premenopausal woman with Lymphocytic hypophysitis and diabetes insipidus was treated with methylprednisolone pulse therapy. Her adenopituitary lesion disappeared and the diabetes insipidus resolved. The optimal management for patients with lymphocytic hypophysitis may be the high index of the suspicion prior to the extensive surgical resection. In addition, methylprednisolone pulse therapy may improve the clinical and MRI findings.
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7/20. Myositis in infiltrative lymphocytosis syndrome: clinicopathological observations and treatment.

    Diffuse infiltrative lymphocytosis syndrome (DILS) is characterized by the presence of persistent circulating CD8 lymphocytosis and visceral CD8 lymphocytic infiltration, predominantly in the salivary glands and lungs in a subset of human immunodeficiency virus (HIV) patients. Myositis has been rarely documented in DILS. We report here the histopathological abnormalities and treatment response of an HIV patient with DILS associated myositis. Muscle biopsy revealed features of polymyositis but electron microscopy study showed intranuclear filamentous inclusions of 18 nm diameter, without intracytoplasmic inclusion. Treatment with intravenous immunoglobuline was useful.
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8/20. A case of lymphocytic hypophysitis with masked diabetes insipidus unveiled by glucocorticoid replacement.

    Lymphocytic hypophysitis may involve the pituitary gland and various hormonal abnormalities. A 72-year-old man presented with euvolemic hyponatremia caused by glucocorticoid deficiency. After glucocorticoid replacement, hypernatremia in the presence of dilute urine was found. Central diabetes insipidus (DI) was confirmed later by a significant increase in urine osmolality after vasopressin administration. brain magnetic resonance imaging showed a pituitary mass and loss of hyperintense signal in the posterior pituitary gland on T1-weighted imaging. The patient underwent a transsphenoidal adenectomy, and pathological examination of dissected tissues showed a typical finding of lymphocytic hypophysitis. Two months after surgery, the patient's central DI had resolved sufficiently that 1-desamino-8- d -arginine vasopressin therapy was discontinued without polyuria. However, he was kept on glucocorticoid and levothyroxine therapy. In conclusion, lymphocytic hypophysitis may feature a concealed central DI caused by glucocorticoid deficiency-associated hyponatremia.
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9/20. Concurrent lymphocytic hypophysitis and pituitary adenoma. Case report and review of the literature.

    Lymphocytic hypophysitis (LyH) is an uncommon intrasellar lesion characterized by lymphocytic infiltration of the adenohypophysis. Evidence suggests that the cause is autoimmune, and the symptoms are usually related to either a mass effect or endocrine dysfunction. Lymphocytic hypophysitis has been described rarely in the setting of other simultaneous pathological processes that involve the pituitary and sella turcica, and is postulated to arise from an intrinsic inflammatory response. The authors report the case of a 43-year-old woman who presented with a 2-month history of galactorrhea and pseudohyperprolactinemia secondary to a 10-mm lesion within an enlarged pituitary gland. She was nulliparous and had no contributory medical history. Serial neuroimaging performed over a 2-year period demonstrated lesion growth, and visual deficits had developed; together these warranted surgical intervention. A transsphenoidal resection was performed. Microscopic and immunohistopathological examinations revealed a nonsecreting pituitary adenoma with concurrent lymphocytic adenohypophysitis. This is the first documented case of LyH in the setting of a null-cell pituitary adenoma. The authors review the related literature and outline potential mechanisms for the concurrent development of LyH and a pituitary adenoma.
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10/20. A diffuse infiltrative CD8 lymphocytosis syndrome in human immunodeficiency virus (HIV) infection: a host immune response associated with HLA-DR5.

    STUDY OBJECTIVE: To describe the clinical, immunologic, and immunogenetic features of a diffuse infiltrative lymphocytic disorder resembling Sjogren syndrome in persons infected with human immunodeficiency virus (HIV). DESIGN: Clinical case study. SETTING: University-affiliated hospitals and outpatient clinics. patients: Consecutive sample of 17 patients. MEASUREMENTS AND MAIN RESULTS: All of the 17 patients had bilateral parotid gland enlargement; 14 had xerostomia and 6 had xerophthalmia. Of the 17 patients, 14 had generalized lymphadenopathy, 10 had histologically proved lymphocytic interstitial pneumonitis, 4 had neurologic involvement, and 3 had lymphocytic infiltration of the gastrointestinal tract. gallium scanning in all of 11 tested patients showed abnormal salivary gland uptake. Minor salivary gland biopsies showed more than 2 lymphocytic foci per 4 mm2 tissue in all of 11 tested patients, the infiltrate consisting predominantly of CD8 cells. Fifteen patients had circulating CD8 lymphocytosis; the principal phenotype of these cells was CD8 CD29 . Rheumatoid factor and antinuclear antibodies were infrequent, and none of the patients had anti-Ro/SS-A or anti-La/SS-B antibodies. HLA-DR5 was significantly more frequent in the black patients (10 of 12) compared with controls (13 of 45). Only one patient developed an opportunistic infection during 544 patient-months of study, and none has died of AIDS. CONCLUSIONS: A distinct syndrome primarily characterized by parotid gland enlargement, sicca symptoms, and pulmonary involvement occurs in HIV infection. This disorder is associated with CD8 lymphocytosis and the presence of HLA-DR5, and appears to be a genetically determined host immune response to HIV.
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