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

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

Malignant histiocytosis (MH) is a progressive systemic neoplastic proliferation of morphologically atypical histiocytes, well characterised in humans and dogs but only recently identified in the cat. In all species, liver, lung, lymph nodes, spleen and bone marrow are infiltrated by atypical histiocytes, and the disease is rapidly fatal. The purpose of this study was to describe the clinical, histological, immunohistochemical and ultrastructural findings of MH in a cat, together with the diagnostic work-up and a list of differential diagnoses. Clinical evaluation included a complete blood-cell count, serum biochemistry, urinalysis, serology and ultrasound examination. The cat had clinical signs of depression, thinness, dehydration, pale mucous membranes and tachycardia. Abdominal ultrasonography revealed generalised splenomegaly and hepatomegaly. Necroscopy showed whitish nodules, randomly scattered throughout the parenchyma in the spleen and liver. The periportal lymph nodes were greatly enlarged and the cut surface was uniformly greyish-white and translucent. Histological examination revealed pleomorphic proliferation of large round tumour cells, with numerous phagocytic vacuoles containing erytrocytes, leukocytes and haemosiderin. By immunohistochemistry, positivity for lysozyme and alpha1-antitrypsin and a scattered positivity for Mac 387 were observed. Ultrastructural features of tumour cells included cytoplasmic lipid droplets, lysosomes and phagolysosomes. MH in the cat needs to be differentiated from diffuse granulomatous disease, non-Hodgkin's lymphoma and Hodgkin's-like disease. The morphological features of the tumour cells, combined with immunohistochemical and ultrastructural observation, are consistent with a diagnosis of MH in the cat.
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PMID:Morphological characterisation of malignant histiocytosis in a cat. 1908 73


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