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)

An unusual case of granulocytic sarcoma with a large retro-orbital tumor mass is described. The tumor had an uncommon cytomorphology and ultrastructure that mimicked a signet ring cell lymphoma. It was negative by chloroacetate esterase (CAE) stain. The patient was treated successfully with CHOP-regimen polychemotherapy and irradiation. Seventeen months after the initial diagnosis of malignant lymphoma, acute myeloid leukemia developed. Additional immunohistochemistry, including an immunoperoxidase staining for lysozyme, clearly demonstrated the early myeloid nature of the original tumor. This case emphasizes the importance of staining for lysozyme and other myeloid markers in addition to CAE staining in cases that demonstrate unusual morphological features.
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PMID:Granulocytic sarcoma (chloroma). Presentation of an unusual case. 195 Nov 90

A 43 year-old man admitted to our hospital because of fever and splenomegaly. Laboratory findings were as follows: Hb 9.5 g/dl, Plts 4.9 X 10(4)/microliter, LDH 2,348 IU/l. Bone marrow findings showed tumor cell 47% with or without phagocytosis. The tumor cells were stained positive lysozyme and alpha 1 antitrypsin. Cytogenetic study was 47, XY, -7, -8, +9, -11, -12, -19, -21, 3q+, 6p+, +6 markers. This case was diagnosed as malignant histiocytosis. Complete remission was achieved with CHOP-E chemotherapy. Remission has been maintained with repeated this therapy. Etoposide deserves a good evaluation in the treatment of malignant histiocytosis. Some cases of malignant histiocytosis with a t(2; 5) (p23; q35) translocation were often reported in Europe and America, while there was no specific chromosomal abnormalities with malignant histiocytosis in Japan.
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PMID:[Malignant histiocytosis with complex chromosomal abnormality: successful treatment with CHOP-E chemotherapy]. 204 Nov 66

The diagnostic findings of malignant histiocytosis (MH) were analysed in 12 consecutive patients in a single institution. Most patients presented with systemic symptoms and lymphadenopathy (92%), splenomegaly (100%) and hepatomegaly (67%). Neurologic symptoms were present in three patients, while involvement of other organs was present in five patients. The incidence of severe thrombocytopenia was 92% of anaemia 92% and of leucocytopenia 67%. Serum angiotensin converting enzyme, alpha 1-antitrypsin and lysozyme were independently increased in 6/9, 3/10 and 1/9 patients respectively. High serum levels of tumour necrosis factor (TNF) were present in 3/10 patients, while serum levels of interleukin-1 were normal in 10/10 patients. Histologic evidence of MH was obtained in all patients by repeated biopsies of involved tissues. Four patients died prior to treatment. Seven patients were treated with combination chemotherapy, consisting of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) or MOPP (chloromethine, vincristine, procarbazine, prednisone), in some cases followed by non-cross-resistant second line chemotherapy, if no complete response was attained. The response rate of treated patients was 57%, and progression was observed in two patients. The median duration of response was 38 months. Three patients are alive without evidence of disease and off therapy (30+, 83+, 85+ months). Although MH is a potentially lethal disease, combination chemotherapy may offer a chance for cure in some patients.
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PMID:Clinicopathological diagnosis and treatment of malignant histiocytosis. 220 2

We had 18 patients (15 males and 3 females) with lethal midline granuloma (polymorphic reticulosis) in the period from 1981 to 1990. This number was about 5.6% of the total number of patients with malignant head and neck tumors that we encountered during this period. An average of 9.1 months separated the first appearance of disease and the beginning of treatment. Most of the 18 patients underwent both radiation therapy and chemotherapy (COP, CHOP, MACOP-B), but, since their disease had reached an advanced stage, 3 underwent radiation therapy only, 3 underwent chemotherapy only, and 1 received no radical therapy at all. Of the 18 patients, 13 died of the disease. In of 6 of these, the disease was confined to the local lesion. The 5-year cumulative survival rate was 15.7% (Kaplan-Meier). Fourteen autopsy studies revealed that tumor cells had invaded the liver (92.8%), lung (92.8%) and spleen (71.4%) and in all cases it was in leukemic patterns. Fifteen cases were studied for tumor surface marker phenotype, but none was found to be positive for L26, CD43, Leu M1 (CD15), or MAC 387. Five cases were positive for UCHL-1 (CD45RO) and 10 cases were positive for lysozyme. All cases were positive for Ki-1 (CD30).
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PMID:[Cases of lethal midline granuloma (polymorphic reticulosis) at our department in a recent 10-year period]. 834 94

Histiocytic sarcoma is a rare malignant neoplasm that occurs in lymph nodes, skin, and the gastrointestinal tract. Many previously published cases were likely misdiagnosed examples of non-Hodgkin lymphoma. Only small numbers of bona fide examples exist in the world literature; cases arising primarily at extranodal sites are not well described and often seem to go unrecognized. To characterize these tumors further, 14 extranodal histiocytic sarcomas were analyzed. Hematoxylin and eosin sections were reexamined, immunohistochemistry was performed, and clinical details were obtained from referring hospitals. Eight patients were female and 6 male (median age, 55 years; range, 15-89 years). All patients presented with a solitary mass, ranging in size from 1.8 to 12 cm (median 6.8 cm). Seven tumors arose in soft tissue (6 lower limb; 1 upper limb), 5 in the gastrointestinal tract (1 involving both stomach and colon, 1 ileum, 2 rectum, 1 anus), 1 in the nasal cavity, and 1 in the lung. Three gastrointestinal tract tumors also involved regional lymph nodes, and 1 involved the liver. Most cases had infiltrative margins. The tumors were generally composed of sheets of large epithelioid cells with abundant eosinophilic cytoplasm, oval to irregular nuclei, vesicular chromatin, and large nucleoli. Binucleated cells were common, and 6 cases contained tumor giant cells. Mitoses ranged from 1 to 64 per 10 HPF (median 11 per 10 HPF). Necrosis was present in 8 cases. Nearly all tumors showed a striking inflammatory infiltrate, most often of neutrophils or lymphocytes. All cases were reactive for LCA, CD45RO, and CD68 (KP1 and PG-M1); 13 of 14 (93%) expressed CD4, 12 of 14 (86%) lysozyme, 8 of 10 (80%) CD31, 7 of 14 (50%) S-100 protein, and 5 of 14 (36%) focal CD1a. Two tumors showed weak, focal cytoplasmic positivity for CD30, and 1 for epithelial membrane antigen. The tumors were negative for ALK-1, CD21, CD35, CD3, CD20, CD34, myeloperoxidase, HMB-45, and keratins. Gastrointestinal tract cases were negative for c-kit and desmin. Six patients were treated with postoperative radiation and 7 with chemotherapy (CHOP or ProMACE-MOPP). Follow-up was available for 10 patients (median, 24 months; range, 4 months to 11 years). Two tumors recurred locally, and 5 patients developed distant spread: 3 to lymph nodes, 1 to lung, and 1 to bone. At the last follow-up, 2 patients have died of disseminated disease, 4 and 5 months following initial diagnosis. The patients who died thus far had the largest primary tumors. Histiocytic sarcoma may arise primarily in soft tissue and shows reproducible histologic features, including abundant eosinophilic cytoplasm and a prominent inflammatory infiltrate. Metastatic carcinoma, metastatic melanoma, and large cell non-Hodgkin lymphomas should be excluded by immunohistochemistry. Histiocytic sarcoma has the potential for an aggressive clinical course, most often with lymph node involvement. However, a subset of cases presenting with clinically localized disease have a favorable long-term outcome. Tumor size may be a prognostic factor.
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PMID:Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. 1531 12

Malignant histiocytosis is a rare neoplasm of the reticuloendothelial system characterized by neoplastic proliferation of tissue histiocytes. We report a case of malignant histiocytosis in a 64-year-old female initially operated on for a mucinous cystadenoma of her liver. Four months after the operation, skin induration on the neck and anterior thoracic wall and systemic lymphadenopathy were noted. Histology and immunohistochemistry of the lymph node and bone marrow specimens showed extensive infiltration with atypical cells, resembling malignant histiocytes (CD45, CD45RO, CD11c, CD68, lysozyme, antitrypsin and alpha1-antichymotrypsin positive; CD1, CD35, B-cell and T-cells markers negative). She was treated with vinblastine, methotrexate and dexamethasone (3 cycles) without response. The therapy was switched to CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisone) with disappearance of lymphadenopathy. Bone marrow infiltration by histiocytes was reduced to 20%. Two months after completion of 8 cycles of CHOP she experienced severe headaches, vomiting, loss of consciousness, and developed paraparesis. A CT scan of the brain was normal but the cerebrospinal fluid cytology showed presence of histiocytes. The patient was then treated with intrathecal methotrexate, prednisolone and cytosine-arabinoside and systemic chemotherapy with etoposide and cyclophosphamide. Her condition improved, she became conscious, her headache diminished, she became mobile but skin and nodal lesions reappeared along with extensive marrow histiocytic infiltration. She finally died 22 months after diagnosis.
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PMID:Malignant histiocytosis with central nervous system involvement and hepatic mucinous cystadenoma in a single patient with review of the literature. 1806 14

We report a rare case of sarcoidosis-lymphoma syndrome with vertebral bone destruction. A 63-year-old woman was previously diagnosed as sarcoidosis by supraclavicular lymph node biopsy, and came to our hospital complaining of back pain. Both serum angiotensin-converting enzyme and lysozyme level had been continuously elevated. Magnetic resonance imaging revealed lumbar vertebral bone destruction. Histopathologic examination of lumbar vertebral tumor obtained by CT-guided biopsy revealed non-caseating epithelioid granuloma with CD 68 (+), AE1/AE3 (-), and no malignant cells. She was admitted to our hospital again for dyspnea and pancytopenia. We diagnosed active sarcoidosis and administered oral 30mg prednisolone daily. One month later, prednisolone became ineffective. Flow cytometry of tumor cells obtained from the gastric ulcer floor showed CD 5 (+), CD 20 (+), K chain monoclonality and we diagnosed B-cell non Hodgkin's lymphoma. She was treated by eight cycles of CHOP plus rituximab chemotherapy and achieved complete response. FDG uptake of the entire body decreased, whereas MRI revealed residual mass in the vertebrae. Sarcoidosis had been diagnosed for two and half years before lymphoma developed. Bone destruction is very rare and sarcoidosis is rarely the cause. This is quite an unusual case presenting histologically proved epithelioid granuloma and vertebral destruction in sarcoidosis-lymphoma syndrome.
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PMID:[Sarcoidosis-lymphoma syndrome with vertebral bone destruction]. 1999 5