muramidase and Myelodysplastic-Syndromes

Granulocytic sarcoma (GS), or chloroma, is a rare extramedullary tumor composed of immature myeloid cells. It most commonly involves bone, soft tissue, lymph nodes and skin and develops during the course of or preceding myelogenous leukemia (ML). Involvement of other organs has been rarely reported including ovary, uterus and cervix, lung and the gastrointestinal tract; however, GS presenting as upper and lower gastrointestinal (GI) bleeding from ulcerated gastric mass and concurrent bleeding vaginal mass is an unusual rare manifestation of GS. We describe a case of GS in a 70 year old black woman who presented with a bleeding "lump" in the vaginal wall and suffered fatal GI bleeding from an ulcerated gastric lesion. She was diagnosed with myelodysblastic syndrome a few months earlier. From the review of the available English literature, this is a unique presentation of GS. It is important to include this entity in the differential diagnosis when encountering GI bleeding particularly in a patient previously diagnosed with myeloid leukemia or preleukemia. The importance of Naphthol Chloracetate Esterase (NCAE) stain and lysozyme immunoperoxidase stain in establishing the diagnosis is breifly discussed.

Topics: Aged; Coloring Agents; Diagnosis, Differential; Fatal Outcome; Female; Gastrointestinal Hemorrhage; Humans; Immunoenzyme Techniques; Leukemia, Myeloid; Muramidase; Myelodysplastic Syndromes; Naphthol AS D Esterase; Stomach Ulcer; Uterine Hemorrhage; Vaginal Diseases

Myelodysplastic syndromes (MDS) are stem cell disorders of clonal origin in which infections and leukemic transformation are quite frequent. Neutrophils from 28 patients with MDS were analysed by flow cytometry for the expression of the two complement receptors CR1 and CR3, the antigenic reactivity of some granule constituents--myeloperoxidase, lysozyme, elastase, lactoferrin--and functional activities, such as locomotion, respiratory burst and cytotoxicity. The results were correlated with the FAB disease subtypes, grouped as low risk (RA) and high risk patients (RAEB, RAEB-t, CMML) and with 30 healthy subjects. A significant reduction in the percentage of neutrophil CR1, CR3 positivity and chemotaxis induced by endotoxin-activated serum was detected in the high risk group when compared with the low risk group and healthy controls. Furthermore, the high risk group also showed a low amount of myeloperoxidase, elastase, lysozyme and superoxide anion, but both low and high risk groups displayed reduced cellular cytotoxicity in comparison with the control. This work indicates that MDS patients belonging to the more advanced FAB categories frequently show multiple abnormalities in the expression of neutrophil complement receptors, and granular components (> 3), as well as in cell functions, suggesting the possibility of using these phenotypic abnormalities in the monitoring of disease progression.

Topics: Chemotaxis, Leukocyte; Cytoplasmic Granules; Flow Cytometry; Humans; Lactoferrin; Muramidase; Myelodysplastic Syndromes; Neutrophils; Pancreatic Elastase; Peroxidase; Phagocytosis; Receptors, Complement; Receptors, Complement 3b; Superoxides

By means of the immunocytochemical method, the level of cytoplasmic lysozyme in leukocytes from healthy volunteers (n = 50) and from patients with uremia (n = 50), leukocytosis (n = 50), various forms of leukemia (n = 36) and myelodysplastic syndrome (MDS) (n = 7) were analysed, and compared with that of simultaneously assayed serum lysozyme. Both the cytoplasmic and serum levels of lysozyme in uremia and leukocytosis were significantly higher than normal subjects (p < 0.001). No correlation, however, was found between their cytoplasmic and serum levels of lysozyme. Morphological analysis for various kinds of leukemia and MDS indicated that myelocytic and monocytic cells became highly positive for lysozyme staining with maturation, and that lymphocytes, leukemic myeloblasts and monoblasts were negative. The cytoplasmic and serum lysozyme levels of leukemias or MDS having a number of lysozyme-positive cells were elevated as compared with those of normal individuals. Among them acute myelocytic leukemia (FAB M4) revealed an excellent correlation between the lysozyme levels in cytoplasm and in serum. The rest whose serum lysozyme level tend to be lower than the cytoplasmic one gave poor correlation. Thus, serum lysozyme level is not fully reflected by the cytoplasmic level. The dual determination of cytoplasmic and serum lysozyme is suggested to be helpful on estimating leukemia types, the degree of cellular maturation and total cell mass, and might also provide a valuable tool for prediction of prognosis for these disorders.

Topics: Cytoplasm; Humans; Leukemia; Leukocytes; Leukocytosis; Muramidase; Myelodysplastic Syndromes; Uremia

Intracellular contents and serum levels of neutrophilic granule protein components, including myeloperoxidase (MPO), lactoferrin (LF) and lysozyme (LYZ), were investigated in 30 cases of myelodysplastic syndromes (MDS), with 59 healthy adult donors as controls. Both neutrophilic MPO and LF decreased significantly, suggesting the transformation of abnormal clones. Both serum levels of LF and LYZ exhibited a considerable fluctuation which may reflect reflect granulopoiesis in the bone marrow. We are of the opinion that measurement of intracellular MPO, LF, LYZ and their serum levels may aid in the diagnosis and prognosis of MDS and is proved to be of great clinical significance.

Topics: Adolescent; Adult; Child; Female; Humans; Lactoferrin; Male; Middle Aged; Muramidase; Myelodysplastic Syndromes; Neutrophils; Peroxidase