Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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)

Myeloid sarcoma (MS) is a localized extramedullary mass of immature granulocytic cells that usually occurs in patients with acute myeloid leukemia (AML) or myeloproliferative disorders. It may rarely precede peripheral blood or bone marrow involvement, presenting a diagnostic challenge. Although MS may be found in any location, an intraoral occurrence is rare. In this report we describe a rare case of a patient with nonleukemic MS of the maxillary gingiva. The histologic specimen was first interpreted as non-Hodgkin's lymphoma. The correct diagnosis was reached after extensive immunohistologic studies. The malignant cells were myeloperoxidase positive, lysozyme positive, CD45+, CD68+, CD3-, CD10-, CD19-, CD20-, CD30-, CD34-, CD56-, CD79a-, S100-, and chloroacetate esterase negative. Induction therapy with FLAND (fludarabine, Ara-C, mitoxantrone, and dexamethasone) was started, but the patient did not achieve a remission. Some weeks later, the patient presented pleural effusion and paralysis of the seventh cranial nerve on the left side. She died a few days later. The present case indicates the importance of a correct initial diagnosis for adequate therapy, which is often delayed because of a high misdiagnosis rate. If the MS is treated without intensive chemotherapy for AML as soon as possible, the prognosis will be poor.
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PMID:Myeloid sarcoma occurring in the maxillary gingiva: a case without leukemic manifestations. 1576 82

A 43-year-old woman with a past medical history of breast cancer and an acute myeloid leukemia (AML) presented with headache over a 3-week period. The clinical examination was completely unremarkable. CT and MRI scans showed a contrast enhancing lesion in the left temporal lobe. Histopathologic examination revealed a malignant, hematopoietic tumor with high mitotic activity, areas of necrosis and diffuse infiltration of the brain parenchyma. Positive staining for Chloroacetateesterase and lysozyme of tumor cells identified its myeloid lineage. The diagnosis was granulocytic sarcoma (GS)/chloroma, a metastatic manifestation of AML. Granulocytic sarcoma (GS) most often occurs in patients with AML, myelodysplastic syndromes and myeloproliferative disorders, and can involve any organ. However intracerebral manifestation of GS is a rare event. In this case histopathological features and differential diagnoses of intracerebral GS are discussed.
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PMID:A 43-year-old woman with a temporal mass. 1661 89

Extramedullary myeloid tumors (myeloid sarcomas) are rare neoplasms that are composed of myeloid precursors. They usually arise concurrently with a diagnosis of acute myeloid leukemia, chronic myeloid leukemia, or other myeloproliferative disorders. They may also indicate relapsing disease in a patient with a prior history of leukemia or myeloproliferative disorder. We present our findings of a 63-year-old female diagnosed with extramedullary myeloid tumor first presenting in the gallbladder. She subsequently developed respiratory failure; pre- and postmortem bone marrow studies were negative for leukemia by morphology, flow cytometry, and karyotypic analysis. However, the myeloid neoplasm was disseminated throughout most of her remaining organs. Immunohistochemical stains of the cells indicated a neoplasm of myelomonocytic derivation (CD4, CD43, CD45, CD68, myeloperoxidase, and lysozyme positive). To our knowledge, this is the first report of an extramedullary myeloid neoplasm of the gallbladder with disseminated disease without involvement of the bone marrow.
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PMID:Disseminated extramedullary myeloid tumor of the gallbladder without involvement of the bone marrow. 1694 21

Myeloid sarcoma is a rare tumor of immature myeloid cells in an extramedullary site. Myeloid sarcoma may present in a variety of locations; skin is one of the common sites. It may precede or occur concurrently with acute myeloid leukemia, chronic myeloid leukemia, other forms of myeloproliferative disorders/myelodysplastic syndrome or de novo. We report a case of a 4-month-old female who presented with cutaneous lesions without evidence of leukemia, determined to be de novo myeloid sarcoma. She had erythematous nodules in multiple skin sites. Biopsy revealed a diffuse atypical mononuclear cell infiltrate involving the entire dermis and extending to the subcutis. The infiltrate was diffusely positive for lysozyme, CD43, CD15, CD33, CD68 and CD117 and was negative for CD3, CD20, CD34, CD56, CD79a, CD99, myeloperoxidase, desmin, chromogranin and synaptophysin, supporting a diagnosis of myeloid sarcoma. No leukemic involvement was found on evaluation of peripheral blood or bone marrow aspiration. Chromosomal abnormalities were found at chromosomes 7, 10 and 11. The skin lesions resolved following multiple chemotherapy courses, then recurred requiring additional treatment. De novo myeloid sarcoma involving skin without evidence of leukemia can occur in an infant and may present a diagnostic challenge.
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PMID:De novo myeloid sarcoma in a 4-month-old infant: a case report and review of the literature. 2309 17

The tyrosine phosphatase SHP2, encoded by PTPN11, is required for the survival, proliferation and differentiation of various cell types. Germline activating mutations in PTPN11 cause Noonan syndrome, whereas somatic PTPN11 mutations cause childhood myeloproliferative disease and contribute to some solid tumours. Recently, heterozygous inactivating mutations in PTPN11 were found in metachondromatosis, a rare inherited disorder featuring multiple exostoses, enchondromas, joint destruction and bony deformities. The detailed pathogenesis of this disorder has remained unclear. Here we use a conditional knockout (floxed) Ptpn11 allele (Ptpn11(fl)) and Cre recombinase transgenic mice to delete Ptpn11 specifically in monocytes, macrophages and osteoclasts (lysozyme M-Cre; LysMCre) or in cathepsin K (Ctsk)-expressing cells, previously thought to be osteoclasts. LysMCre;Ptpn11(fl/fl) mice had mild osteopetrosis. Notably, however, CtskCre;Ptpn11(fl/fl) mice developed features very similar to metachondromatosis. Lineage tracing revealed a novel population of CtskCre-expressing cells in the perichondrial groove of Ranvier that display markers and functional properties consistent with mesenchymal progenitors. Chondroid neoplasms arise from these cells and show decreased extracellular signal-regulated kinase (ERK) pathway activation, increased Indian hedgehog (Ihh) and parathyroid hormone-related protein (Pthrp, also known as Pthlh) expression and excessive proliferation. Shp2-deficient chondroprogenitors had decreased fibroblast growth factor-evoked ERK activation and enhanced Ihh and Pthrp expression, whereas fibroblast growth factor receptor (FGFR) or mitogen-activated protein kinase kinase (MEK) inhibitor treatment of chondroid cells increased Ihh and Pthrp expression. Importantly, smoothened inhibitor treatment ameliorated metachondromatosis features in CtskCre;Ptpn11(fl/fl) mice. Thus, in contrast to its pro-oncogenic role in haematopoietic and epithelial cells, Ptpn11 is a tumour suppressor in cartilage, acting through a FGFR/MEK/ERK-dependent pathway in a novel progenitor cell population to prevent excessive Ihh production.
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PMID:Ptpn11 deletion in a novel progenitor causes metachondromatosis by inducing hedgehog signalling. 2386 41

Monocytes are critical components of the innate immune system and they can differentiate into dendritic cells (DCs). Cutaneous neoplasms of dendritic cell origin are uncommon and mostly represented by histiocytic lesions derived primarily from Langerhans cells. The myeloid DC (mDC) while recognized in the immunology literature does not have a well-defined neoplastic cutaneous counterpart. Eleven patients with a diagnosis of cutaneous mDC dyscrasia were evaluated. Routine hematoxylin and eosin stain were performed followed by selective phenotypic studies. The patients were older without a gender predilection and exhibited an asymptomatic papular skin rash with a waxing and waning course. The biopsies demonstrated a dermal based monomorphic small mononuclear cell infiltrate. The cells expressed CD14, CD11c, HLA-DR, as well as granzyme and lysozyme that defines terminally differentiated monocyte/dendritic cells. Expression of BDCA-3 (CD141) by the tumor cells indicated that they were myeloid dendritic cells (mDC2). Each patient had a prior or subsequent diagnosis of an abnormal bone marrow biopsy that included myelodysplastic syndrome, myelofibrosis, chronic myelomonocytic leukemia, and acute myelogenous leukemia. We propose the term cutaneous mDC cell dyscrasia for distinctive infiltrates of differentiated mDCs reflective of underlying myeloproliferative disease. The clinical course is variable and can be indolent although it is strongly correlated with myelodysplastic syndrome that included leukemia.
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PMID:Cutaneous myeloid dendritic cell dyscrasia: A cutaneous clonal monocytosis associated with chronic myeloproliferative disorders and peripheral blood monocytosis. 2780 53

The influence of different parameters on the silicification procedure using lysozyme is reported. When polyethoxysiloxane (PEOS), an internally crosslinked silica reservoir, is used, regular structures with a narrow size distribution could be obtained only via introducing the silica precursor in two steps including initial dropping and subsequent addition of residual oil phase in one portion. We found that mixing sequence of mineralizing agents in the presence of a positively charged surfactant plays a key role in terms of silica precipitation when tetraethoxyorthosilicate (TEOS) is the oil phase. In contrast, well-mineralized crumpled features with high specific surface area could be synthesized in the presence of PEOS as a silica precursor polymer, regardless of mixing sequence. Moreover, introducing sodium dodecyl sulfate (SDS) as a negatively charged surfactant resulted in regular silica sphere formation only in combination with hexylene glycol (MPD) as a specific co-solvent. Finally, it is demonstrated that by inclusion of different nanoparticles even more sophisticated hybrid materials can be generated.
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PMID:Controlling the bio-inspired synthesis of silica. 2783 57

Hematological malignancies can manifest as extramedullary soft tissue masses in relatively rare cases. The rarity of it causes a diagnostic and therapeutic challenge. One of the rarest manifestations is myeloid sarcoma (MS). MS develops as part of acute myeloid leukemia, myeloproliferative neoplasm, or myelodysplastic syndrome or at relapse, especially following allogeneic hematopoietic stem cell transplant. The tumor displays high myeloperoxidase expression, hence the color green, and is called chloroma. It most commonly appears in lymph nodes, skin and soft tissues, bone, testes, gastrointestinal tract, and peritoneum. Immunohistochemistry shows CD68-KP1 as the most commonly expressed marker, then myeloperoxidase, CD117, CD99, CD68/PG-M1, lysozyme, CD34, terminal deoxynucleotidyl transferase, CD56, CD61, CD30, glycophorin A, and CD4. Different chromosomal abnormalities including MLL rearrangement, t(8; 21), monosomy 7, trisomy 8, trisomy 11, trisomy 4, inversion (16), monosomy 16,16q deletion, 5q deletion, and 20q deletion were reported. Most of the literature about MS are case reports and small retrospective studies, thus there is limited clinical knowledge of the cases and their presentation and management plans. Here, we provide a review of what has been reported in the literature about MS in the light of our experiences.
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PMID:Myeloid Sarcoma. 3084 Sep 60


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