Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.2.1.17 (lysozyme)
 21,489 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a 70-year-old man with cutaneous granulocytic sarcoma who presented with numerous cutaneous nodules but without any leukaemic involvement of the peripheral blood. The tumour cells were positive for lysozyme, peroxidase, CD11a, CD11c, CD33 and HLA-DR, and weakly positive for CD4 and CD14, suggesting granulocytic differentiation. The bone marrow at admission showed dysplasia of the erythrocytic and granulocytic lineage and complex chromosomal abnormalities in association with an increase in monocytes. The patient was diagnosed as having granulocytic sarcoma of monocytic lineage with concomitant myelodysplastic syndrome. In this case, tumour cells also expressed the neural cell adhesion molecule (CD56), which has been suggested as a possible risk factor for developing granulocytic sarcoma in acute myelogenous leukaemia.
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PMID:A case of CD56+ cutaneous aleukaemic granulocytic sarcoma with myelodysplastic syndrome. 1097 33

Langerhans cell histiocytosis (LCH) represents a poorly defined pathologic entity characterized by diverse clinical appearence and falling into two major categories namely a restricted and an extensive disease. Since the outcome and the course of the disease is variable, we postulated that this might be reflected by the phenotype of the Langerhans cells. We have selected 11 adult restricted cases and 10 extensive childhood cases and compared the phenotype of LCH cells by immunohistochemistry on paraffin sections. Morphometric analysis indicated a significantly higher expression of histiocytic (CD68, S-100, lysozyme) markers in the adult restricted cases compared to the extensive form of the disease. Both groups were equally positive for LCH marker CD1a and negative for T cell marker CD4. On the other hand, HLA-DR expression was significantly higher in LCH cells of the extensive childhood cases suggesting higher activation. These data suggest that LCH cells have a different phenotype in the extensive childhood and restricted adult LCH where the latter is characterized by a more differentiated histiocytic phenotype.
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PMID:Differential Expression of Markers in Extensive and Restricted Langerhans Cell Histiocytosis (LCH). 1117 5

Here, we report on an elderly woman with sarcoidosis and Hashimoto's disease who later developed myasthenia gravis. She was 68-year-old with a long history of Hashimoto's disease who had a clinical diagnosis of sarcoidosis with uveritis at the age of 66 years. On laboratory examination, angiotensin-converting enzyme, lysozyme and gamma-globulin were elevated and there was bilateral hilar lymphoadenopathy. She was admitted to our hospital because of left blepharoptosis and mild fatigability in the proximal muscles at the age of 68 years. Myasthenia gravis, type IIa, was confirmed by elevated titer of anti-acetylcholine receptor antibody in serum (0.8 nmol/l, normal < 0.6), positive edrophonium test and decremental EMG response. Oral prednisolone therapy was effective. Her muscle biopsy revealed HLA ABC-positive fibers in all fascicles, and HLA-DR positive fibers in the perifascicular areas. Myasthenia gravis complicated by sarcoidosis and Hashimoto's thyroiditis is extremely rare, suggesting that the common underlying immunological abnormalities for the three disorders such as a certain defective cellular immunity are responsible for the pathomechanism to induce the patient condition.
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PMID:[A case of myasthenia gravis following sarcoidosis and Hashimoto's thyroiditis]. 1121 99

The proper loading of exogenous peptide antigens affects the transport and cell surface expression of MHC class II molecules. In the present study, the goal was to determine to what extent this step determines the cell surface expression level of MHC class II molecules, such as the HLA-DR. EBV-transformed B-cells, were cultured either in a serum- and protein-free medium, or in a medium that contained different concentrations of exogenous antigens. Using HLA-DR-specific antibodies, the induction of the MHC class II expression was observed in cells that were cultured under serum-and protein-free conditions, when compared to those cultured with exogenous protein antigens. This upregulation was completely suppressed to the normal level by the addition of a high concentration of hen egg lysozyme to the serum- and protein-free medium. This indicates that exogenous proteins regulate the HLA-DR expression. To further examine whether this modulation is controlled at the transcription level, the expression of the HLA-DR beta-chain mRNA was analyzed by reverse transcription-PCR and Northern blots. The same levels of HLA-DRB mRNA were detectable in both culture conditions, indicating that the present observation is dependent on some regulatory mechanisms at the post-transcriptional level. This might include a different pathway for trafficking of HLA-DR molecules to the cell surface, since peptide-binding assays revealed that a high proportion of cell surface HLA-DR molecules under the serum- and protein-free condition were transported to the cell surface without associated peptide antigens.
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PMID:HLA-DR expression in B-lymphocytes in vitro is not suppressed by the absence of exogenous antigens. 1171 May 16

Immature-type CD56(+) natural killer (NK)-cell neoplasms are classified as either myeloid/NK-cell precursor acute leukemia or blastic NK-cell lymphoma. We identified two cases of immature-type CD56(+) NK-cell neoplasms that were not categorizable as either of these entities. The first case involved a 74-year-old woman presenting with skin eruptions and pancytopenia due to bone marrow necrosis. Skin biopsy specimen revealed CD4(+), CD7(-), CD34(-), CD43(+), CD56(+), CD68(+), muramidase (lysozyme)(+), and myeloperoxidase (MPO)(-), and immunophenotyping of peripheral blood showed CD4(+), CD7(-), CD13(+), CD33(+), CD34(-), CD43(+), CD56(+), cytoplasmic (cy)CD68(+), CD123(+), and HLA-DR(+). The second case involved a 62-year-old man who had bilateral optic nerve tumor and presented with malignant cells in peripheral blood. Cell surface markers of malignant cells showed CD4(+), CD7(-), CD13(+), CD33(+), CD34(-), CD43(+), CD56(+), cyCD68(+), and HLA-DR(+). The phenotypes of tumor cells in both cases were compatible with blastic NK-cell lymphoma, except for the expression of myeloid antigen. Clinical presentations of these cases showed characteristics of both blastic NK-cell lymphoma and myeloid/NK-cell precursor acute leukemia.
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PMID:Uncommon cases of immature-type CD56+ natural killer (NK)-cell neoplasms, characterized by expression of myeloid antigen of blastic NK-cell lymphoma. 1910 30

We present a case of a histiocytic sarcoma incidentally detected in peripheral lung tissue resected for a spontaneous pneumothorax. Furthermore, we discuss the practical approach to pulmonary Langerhans cell histiocytosis, the main differential diagnosis of this lesion in the lung, based on morphological and immunohistochemical features. A 23-year-old male patient presented with recurrent pneumothoraces. The pulmonary tissue showed a single round granuloma-like lesion measuring 4 mm in diameter in close neighbourhood to a bronchial wall. The granuloma consisted of histiocytic cells with enlarged pale nuclei, plasma cells, lymphocytes and scanty eosinophilic granulocytes giving the impression of a granuloma of pulmonary Langerhans cell histiocytosis on haematoxylin and eosin (H&E) stains. Immunohistochemically, the histiocytic cells were negative for CD1a and S-100. They were positive for CD68, HLA-DR, CD14, CD4, CD11c, CD45LCA and lysozyme. MIB1 (Ki67) showed a nuclear staining of approximately 10% of the histiocytic cells. In summary, these findings were in keeping with a histiocytic sarcoma, a rare haematopoetic neoplasm. By demonstrating this particular case, we emphasise the importance of proving the diagnosis of pulmonary Langerhans cell histiocytosis by means of immunohistochemistry. In case of a negative CD1a reaction in a histiocytic lesion, further immunohistochemical studies have to be performed in order not to misdiagnose a malignant haematopoetic lesion.
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PMID:Pulmonary histiocytic sarcoma mimicking pulmonary Langerhans cell histiocytosis in a young adult presenting with spontaneous pneumothorax: a potential diagnostic pitfall. 1956 69

A 70-year-old male was admitted because of back pain due to peri-vertebral tumors. The histologic picture of a needle-biopsied tumor specimen showed pleomorphic large cell infiltration into the collagen fibers. On immunohistochemistry, these abnormal cells were positive for CD68, CD163 and lysozyme but negative for CD1a, 21, 30, and S100. Flow cytometric analysis also demonstrated that these cells were positive for CD13, 14, 38, 45, 56, and HLA-DR. A bone marrow aspirate showed the marked infiltration of abnormal large cells with the same surface antigens as described above. A diagnosis of HS was made. He showed monocytosis in the peripheral blood of more than 1.0 x 10(9)/L from presentation. The karyotype of bone marrow cells was 46,XY,+8. Fluorescent in situ hybridization (FISH) analysis with a probe for chromosome no. 8 showed that all these monocytes carried +8, indicating that he had another disorder of chronic myelomonocytic leukemia (CMML). FISH analysis with a probe for chromosome no. 12 demonstrated that the abnormal large cells in the bone marrow were all tetraploid, while analysis with the chromosome no. 8 probe showed more than 8 signals per cell, indicating that HS cells carried octasomy to decasomy of chromosome no. 8. These findings strongly suggest that HS in the present patient originated from underlying CMML.
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PMID:Histiocytic sarcoma and underlying chronic myelomonocytic leukemia: a proposal for the developmental classification of histiocytic sarcoma. 2053 95

Chronic lymphocytic leukaemia (CLL) cells convert CD14(+) cells from patients into 'nurse-like' cells (NLCs). CLL cells can also convert CD14(+) peripheral blood mononuclear cells (PBMCs) from healthy donors into cells with morphological similarities to NLCs (CD14(CLL) -cells). However it is unclear whether only CLL cells induce this conversion process. This study showed that CD14(+) PBMCs from healthy donors could also be converted into differentiated cells (CD14(B) -cells) by non-malignant B-cells. In order to identify changes specifically induced by CLL cells, we compared gene expression profiles of NLCs, CD14(CLL) -cells and CD14(B) -cells. CD14(+) cells cultured with CLL cells were more similar to NLCs than those cultured with non-malignant B-cells. The most significant changes induced by CLL cells were deregulation of the antigen presentation pathway and of genes related to immunity. NLCs had reduced levels of lysozyme activity, CD74 and HLA-DR in-vitro while expression of inhibitory FCGR2B was increased. These findings suggest an impaired immunocompetence of NLCs which, if found in-vivo, could contribute to the immunodeficiency in CLL patients.
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PMID:Nurse-like cells show deregulated expression of genes involved in immunocompetence. 2161 84

Salivary gland cysts are often concurrent with GM1 gangliosidosis in Shiba dogs. Although the etiology is unknown, these cysts may be misdiagnosed as malignant due to the accumulation of foamy cells. The present study investigated the cytological, histopathological, immunohistochemical and electron microscopic characteristics of salivary gland cysts in a Shiba dog affected with GM1 gangliosidosis. The salivary gland masses were surgically enucleated and examined clinicopathologically and pathologically in a 7-month-old Shiba dog with GM1 gangliosidosis. Many large cells with rich cytoplasm including vacuoles of various sizes, i.e., foamy cells, were observed in stamp smears from the cut-surface of the masses and histopathologically in major parts of the cyst wall. Some of these foamy cells presented features similar to a spider-web appearance. The foamy cells were confirmed to have originated from macrophages based on marked immunohistochemical expression of vimentin, HLA-DR, lysozyme and Iba1. An ultrastructural study demonstrated electron-dense vesicular structures in the vacuolated cells. Therefore, the masses were diagnosed pathologically as benign salivary gland cysts with accumulation of foamy cells. In conclusion, the histopathological features of the salivary gland cysts in this Shiba dog were similar to those of lipoma and/or liposarcoma. In such cases, immunohistochemical and ultrastructural examinations were useful in the differential diagnosis. Practitioners, clinical pathologists and pathologists should take GM1 gangliosidosis into consideration when they encounter salivary gland cysts in Shiba dogs.
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PMID:Pathological features of salivary gland cysts in a Shiba dog with GM1 gangliosidosis: a possible misdiagnosis as malignancy. 2208 95

Myeloid sarcoma is a tumor which consists of myeloblasts or immature myeloid cells. This tumor presents in the lymphoid organs, bone, skin, soft tissue, various mucosae and organs, and the central nervous system. Granulocytic sarcoma, an extramedullary acute myeloid leukemia, is also referred to as chloroma (GS) because of its greenish surface color. Granulocytic sarcoma is rare and difficult to diagnose. We can easily misdiagnose this tumor as lymphoma or sarcoma, especially when there is no evidence of hematologic disorders. Immunohistochemical studies are helpful in determining the correct diagnosis. Antibodies to myeloperoxidase, lysozyme, and chloroacetate esterase are used for the diagnosis of granulocytic sarcoma. In addition, detection of cell surface markers such as CD 33, CD 34, CD 68, CD 99, and HLA-DR may be useful. We describe a case of GS that presented with bluish nodules on the right cheek of a 54-year-old woman with immunohistochemical findings for correct diagnosis.
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PMID:Primary granulocytic sarcoma of the face. 2214 54


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