1/14. The benign lymphoepithelial lesion--a harbinger of neoplasia. The patient presented had well-documented benign lymphoepithelial lesion, many years before the appearance of neoplastic disease. The relationship between this type of lesion and the later development of neoplasia is unclear, but evidence points to its existence, and further study is needed to clarify its frequency and significance. It appears that in some patients the benign lymphoepithelial lesion is a forerunner of the neoplastic process, and thus may serve as another clinical clue to the early diagnosis of neoplasia, especially that on the lymphoreticuloendothelial cell type. ( info) |
2/14. Mikulicz syndrome associated with a malignant large cell gastric lymphoma: a case report and review of the literature. There is much confusion in the literature regarding the differences between Mikulicz disease and Mikulicz syndrome. This may be because there seems to be a connection between the disease and disease processes associated with the syndrome. This article provides historical data discussing the emergence of Mikulicz disease, confusions regarding its definition, and finally offers an explanation to the interrelationships of these two entities. This case report hypothesizes that the non-Hodgkin's lymphoma (NHL), which in association with sjogren's syndrome and Mikulicz disease in this patient comprised the Mikulicz syndrome, may have transformed into his malignant large-cell gastric lymphoma. It supports conclusions in the literature that Mikulicz disease, benign lymphoepithelial lesion, and sjogren's syndrome may all be a part of the same disease process as graduated variants of one another, with malignant lymphoma being a recognized complication of these entities. ( info) |
3/14. Fine needle aspiration cytologic findings in a benign lymphoepithelial lesion with microcalcifications. A case report. Aspiration cytologic findings in a case of benign lymphoepithelial lesion (BLL) of the parotid gland are presented. The aspirate contained a polymorphous lymphoid population, histiocytes, myoepithelial and ductal epithelial cells and numerous bluish, calcified bodies. A cytologic diagnosis of benign nonneoplastic lesion, consistent with chronic sialadenitis and microlithiasis, was made. The clinical impression of neoplasia was inconsistent with the cytologic findings. Subsequent histologic examination showed classic BLL with microcalcifications as an unexpected feature. The value and limitations of fine needle aspiration cytology in the diagnosis of nonneoplastic salivary gland lesions and the differential diagnosis of BLL are discussed. ( info) |
4/14. Mikulicz's disease and Mikulicz's syndrome: analysis of the original case report of 1892 in the light of current knowledge identifies a MALT lymphoma. OBJECTIVE: To remove the confusion surrounding the terms "Mikulicz's disease" and "Mikulicz's syndrome" by discovering the nature of the disease that Mikulicz described. STUDY DESIGN: Investigation of the original 1892 publication by Mikulicz in the light of current knowledge. RESULTS: Mikulicz described bilateral painless swellings of the lacrymal, major salivary, and minor salivary glands that were present for 14 months without a reduction in salivary flow before the patient, a man of 42 years, died. A print of a microscopical field of one of the submandibular glands contains a uniform lymphocytic infiltrate of centrocyte-like cells typical of MALT lymphoma, which is strongly supported by the reported clinical features. CONCLUSION: Mikulicz published the first case report of a MALT lymphoma of salivary glands, and the terms "Mikulicz's disease" and "Mikulicz's syndrome" should no longer be used. ( info) |
| PURPOSE: To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management with a review of the literature. methods: Retrospective nonrandomized consecutive case series, Jules Stein Eye Institute, David Geffen School of medicine at UCLA. RESULTS: Mikulicz's disease is characterized by symmetric lacrimal, parotid, and submandibular gland enlargement with associated lymphocytic infiltrations. The authors noted two cases of Mikulicz's disease. The diagnosis of Mikulicz's disease was based on the following criteria: 1) symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands (parotid and submandibular); and 2) the exclusion of other diseases that may mimic this presentation, such as sarcoidosis, viral infection, or lymphoproliferative disorders. CONCLUSIONS: Mikulicz's disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions. The condition is self-limiting and most often, the diagnosis is a clinical one. Previously, Mikulicz's disease was often considered as a subtype of sjogren's syndrome (SS). Clinical and immunologic differences between Mikulicz's disease and SS may warrant further consideration of Mikulicz's disease as a specific autoimmune phenomenon separate from SS, and Mikulicz's disease may be amenable to different treatment modalities than those employed in patients with SS. ( info) |
6/14. A case of Mikulicz's disease complicated with interstitial nephritis successfully treated by high-dose corticosteroid. A 40-year-old woman who had bilateral swelling in the eyelids and submandibular region was admitted. Clinical findings suggested she had primary sjogren's syndrome. Laboratory data showed glucosuria, positive CRP (0.50 mg/dl), liver dysfunction (AST 53 U/l, ALT 101 U/l, gamma-GTP 241 U/l, ALP 914 U/l, LAP 496 U/l), hyperglycemia, hypergammaglobulinemia (IgG 3450 mg/dl, IgA 91 mg/dl, IgM 80 mg/dl), hypocomplementemia (C3 73 mg/dl, C4 2 mg/dl, CH50 < 19.0 U/ml), renal tubular dysfunction (urine N-acetyl-beta-D: -glucosaminidase 8.6 U/l, urine (beta2)-microglobulin 83 microg/l), and urinary concentration defect. ammonium chloride loading test was normal. gallium-67 scintigram indicated abnormal uptake in bilateral lacrimal glands, submandibular glands, and kidneys. A diagnosis of Mikulicz's disease and interstitial nephritis was made, since biopsy specimens of her lacrimal gland and minor salivary gland showed diffuse infiltration of lymphocytes. Renal biopsy specimens also showed severe interstitial infiltration of lymphocytes. Symptoms and laboratory data normalized in response to methylprednisolone pulse therapy and prednisolone 60 mg/day. This case of Mikulicz's disease complicated with interstitial nephritis was successfully treated by high-dose corticosteroid. ( info) |
7/14. Mikulicz disease of a minor salivary gland. The third recorded instance of Mikulicz disease of a minor salivary gland is described; this is the first case in which a surgical specimen, in addition to the biopsy specimen, was obtained for microscopic examination. A brief description of the disease and its treatment is also presented. ( info) |
8/14. Polycystic disease of the parotid glands: case report of a rare entity and review of the literature. We report a case of polycystic disease of the parotid glands. This is a rare disorder and we know of only two previous documented cases in the literature. The disease presents with painless enlargement of one or both parotid glands and is not associated with any clinical abnormality of salivation or with any apparent anomaly of the other salivary glands. Histologically, the overall glandular architecture is preserved but the lobules are all markedly distended by epithelial-lined cysts, which appear to be derived from the intercalated ducts. Characteristic congophilic laminated spheroliths are present within the cystic spaces. The condition must be differentiated from cystic neoplasms, particularly papillary cystic adenocarcinoma, and from various non-neoplastic disorders including sialectasia, retention cysts and lymphoepithelial cysts. ( info) |
9/14. Chemotherapy of progressive hairy-cell leukaemia. 5 patients with hairy-cell leukaemia were treated with chemotherapy. 3 of them received multiple courses of CHOP (cyclophosphamide-doxorubicin-vincristine-prednisolone); 1 patient received cyclophosphamide monotherapy for 6 months. The 5th patient developed a Mikulicz's syndrome after splenectomy and was initially treated with cyclophosphamide monotherapy, then received 1 course of CHOP, and finally 9 courses of intermediate-dose methotrexate. 1 complete remission (45 months ) and 3 partial remissions (75 , 68, 32 months) were observed, while 1 patient had progressive disease. Our results indicate that chemotherapy may be a valuable alternative to alpha-interferon in the treatment of hairy-cell leukaemia progressing after splenectomy. ( info) |
10/14. Mikulicz disease and subsequent lupus erythematosus development. In a 12-year-old boy with clinical and histologic features of Mikulicz disease, systemic lupus erythematosus (SLE) developed two years later. Atrophic plaques and nodules in the skin along with substantial increase in salivary gland enlargement characterized the onset of SLE. There was lack of renal and joint manifestations, and no evidence of keratoconjunctivitis sicca or xerostomia was seen. Histopathologic changes in the skin lesions included mucinous infiltrates, chronic vasculitis, and dermal-epidermal junction findings typical of SLE. Direct immunofluorescene studies of skin lesions and normal-appearing skin showed granular deposition of immunoglobulins and complement at the dermal-epidermal junction zone. Level of anti-dna antibodies was noticeably elevated, and the serum C3 value was depressed. The patient's response to systemic corticosteroid therapy was dramatic. ( info) |