Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A new hematopoietic cell line derived from a patient with Philadelphia chromosome (Ph1)-negative myeloblastic leukemia arising from a form of myelodysplastic syndrome (MDS) is described. This cell line, designated TMM, consists of immature cells with the morphological characteristics of young myeloblasts and grows in suspension culture with a doubling time of about 30 hours. By cytochemical analysis the cultured cells were positive for acid phosphatase. They were free of the Epstein-Barr virus-associated nuclear antigen as well as terminal deoxynucleotidyl transferase. Further phenotypic analysis revealed the expression of the myelomonocytic-specific antigen Leu-M1 and receptors for the Fc portion of IgG. Partial differentiation of these cells could be induced by dimethyl sulfoxide, tetradecanoyl phorbol acetate, or hypoxanthine and resulted in cells of the myeloid series expressing lysozyme and receptors for the C3b complement protein. The karyotype was 46,XY, lacked the Ph1 chromosome, and displayed no abnormalities at the light microscopic level. No rearrangement of the bcr-c-abl gene complex was found. This cell line should be useful for studying an important type of the heterogeneous population constituting Ph1-negative myeloblastic leukemia, arising in this instance from MDS, as well as for studying differentiation and proliferation of human pluripotent stem cells.
 ...
PMID:Establishment and characterization of a human myeloid cell line from Philadelphia chromosome-negative myeloblastic leukemia arising in a patient with myelodysplastic syndrome. 347 6

Granulocytic sarcoma (GS) is a solid tumor of extramedullary localization constituted by immature precursors from the granulocytic series. GS may be diagnosed in different malignant blood diseases involving the granulocytic series, acute non lymphoblastic leukemia (ANLL) being the most frequent, followed by myelodysplastic syndromes (MDS) and chronic myeloproliferative syndromes, specially chronic myeloid leukemia (CML) in blastic crisis. Although the diagnosis of GS is suspected with conventional cytologic and anatomopathologic studies, histochemical staining and immunohistochemical techniques are often required for definitive diagnosis. Five cases (4 males, 1 female; age range 22-77 years) diagnosed with GS in one center over a period of nine years (1984-1993) are described. The GS were located in the lymph nodes, the jaw, paravertebral region, gallbladder and retroperitoneum, respectively. Two patients had refractory anemia with excess of blasts (RAEB). Three patients had ANLL; in one GS constituted the form of relapse, in another GS presented at the time of diagnosis and in the remaining patient GS preceded the diagnosis of ANLL. All the patients died from 2 to 8 months after diagnosis of GS with no response to treatment being observed. Immunohistochemical study of the tumor was performed in 4 patients, being positive for lysozyme and the monocytic MAC-387 monoclonal antibody. Immunocytochemical study of the tumor blasts was carried out with positivity for CD15 being observed. Although uncommon, GS should be suspected in patients with ANLL or MDS with tumors of any localization and at any time during its evolution. Immunocytochemical and immunohistochemical studies are of great value to differentiate GS from other tumors, particularly anaplastic non Hodgkin's lymphomas.
 ...
PMID:[Granulocytic sarcoma: a study of 5 cases]. 770 32

A systematic morphological analysis of cutaneous infiltrates in acute myelogenous leukemia and myelodysplastic syndrome revealed that in many cases the infiltrating cells have a different phenotype from those in the bone marrow. This study sought to answer two questions: (a) How wide is the range of cytological features and immunoreactivity of the cutaneous infiltrates and what danger is there of misinterpretation? (b) What are the possible causes of the wide spectrum of differentiation of the cells infiltrating the skin? Skin biopsy specimens from 16 patients with myelogenous leukemia or myelodysplastic syndrome were investigated. The diagnosis was acute myelomonocytic leukemia (M4, according to the French-American-British/FAB system of classification of acute leukemias) in eight cases, acute monocytic leukemia (M5) in four cases, aleukemic leukemia cutis as a recurrence of M2 leukemia after treatment in one case, and myelodysplastic syndrome in three cases, including one case of myelodysplasia with an excess of bone marrow blasts (RAEB-T) and two cases of chronic myelomonocytic leukemia, one of which presented as aleukemic leukemia cutis. Reactivity with the macrophage-associated antibodies anti-CD68, Ki-M1p, and anti-lysozyme was the most consistent. However, the naphthol AS-D chloroacetate esterase reaction and staining with DAKO-M1, Ki-My2p, anti-neutrophil elastase, and anti-CD34 were found to be of little value for identifying the cutaneous infiltrate as myelogenous. Some antibodies (e.g., anti-S100 protein and MB2) even produced staining in a few cases that could have led to a mistaken diagnosis of histiocytic neoplasm or malignant lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Skin involvement in myelogenous leukemia: morphologic and immunophenotypic heterogeneity of skin infiltrates. 754 88

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.
 ...
PMID:Neutrophils from patients with myelodysplastic syndromes: relationship between impairment of granular contents, complement receptors, functional activities and disease status. 806 92

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.
 ...
PMID:[The interrelation of serum lysozyme level and cytoplasmic lysozyme level]. 815 64

We report herein a case of extramedullary myeloid tumor arising bilaterally in the testes of a 66-year-old man, who had previously been diagnosed with myelodysplastic syndrome. Light microscopy of the testicular neoplasm demonstrated a tumor composed of large, slightly polygonal cells with pale blue to weakly eosinophilic cytoplasm. The tumor cells were immunoreactive for CD45, myeloperoxidase, lysozyme, CD43, and MB2. Many of the cells also expressed chloroacetate esterase. Peripheral blood and bone marrow findings were consistent with chronic myelomonocytic leukemia (FAB-CMML), particularly in the most recent material, which showed clear cellular dysplasia and an increase in the percentage of blasts in the bone marrow (15% to 20% of all nucleated cells). This case of extramedullary myeloid tumor is unusual in view of the patient's age and the testicular location. It emphasizes the importance of including extramedullary myeloid tumor in the differential diagnosis of histologically undifferentiated large-cell tumors, as well as a need to use a broad panel of immunohistochemical stains in such cases.
 ...
PMID:Testicular extramedullary myeloid cell tumor in a patient with myelodysplastic syndrome. 861 53

Granulocytic sarcoma (GS) is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. Most GS reported in large series were not associated with overt acute myelogenous leukemia. Gastric perforation occurred during prednisolone therapy in a 72-year-old Japanese male with a four-month history of a myelofibrosis-like state. Subtotal gastrectomy was performed for a suspected gastric ulcer perforation. Gastric histologic, immunohistochemical and cytochemical examination revealed diffuse infiltration by sheets of myeloblasts and promyelocytes with scant or moderately abundant cytoplasm including a few eosinophilic myelocytes. Bone marrow study done in one month after the operation disclosed refractory anemia with excess of blasts (RAEB). Leukemic transformation occurred two months later, and a subcutaneous tumor appeared on the forehead. The forehead tumor predominantly consisted of myeloblasts without evidence of maturation. Both the stomach and forehead tumors were examined immunohistochemically with a panel of monoclonal antibodies (LCA, L26, MT1, UCHL1, OPD4, LN-1, LN-2, LN-3, MB1, Leu-M1, PM) and polyclonal antibodies (lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin, S-100 protein, lactoferrin), as well as naphthol-ASD-chloroacetate esterase staining to investigate and characterize the reliable marks for GS, and the patient was diagnosed as GS. We found that gastric GS may occur in a myelofibrosis-like state followed by RAEB of myelodysplastic syndrome and that naphthol-ASD-chloroacetate esterase staining and immunohistochemical detection of MT1, lysozyme, and alpha 1-antitrypsin were the most reliable markers for confirming the diagnosis of GS.
 ...
PMID:Unsuspected gastric granulocytic sarcoma in a patient with myelodysplastic syndrome. 870 73

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.
 ...
PMID:Fatal gastrointestinal bleeding as the primary manifestation of granulocytic sarcoma in a patient with myelodysblastic syndrome. 906 37

We report the cases of two men, aged 48 and 71 years, with granulocytic sarcoma of the testis. Both presented with left testicular swelling and underwent orchiectomy, which revealed cream-colored to yellow-tan, rubbery-to-firm, testicular tumors with extensive paratesticular spread. The tumor in the younger patient was composed of a uniform population of primitive cells with scant cytoplasm and was initially misinterpreted as malignant lymphoma. Staging revealed no extrascrotal spread. The patient was treated with radiation and chemotherapy and remained free of disease for 12 years, at which time he died of unrelated causes. The older patient had a history of a myelodysplastic syndrome. His tumor contained cells with bright eosinophilic, occasionally granular cytoplasm, consistent with myeloid lineage. Because of a prominent component of myelocytes, with round, eccentric nuclei and moderately abundant cytoplasm, and because of an associated chronic inflammatory cell infiltrate that contained mature plasma cells, the tumor was initially misinterpreted as a plasmacytoma, although it was reinterpreted as a granulocytic sarcoma before initiation of therapy. Tumor cells in both cases were positive with a chloroacetate esterase stain. Immunohistochemical staining revealed expression of myeloperoxidase, lysozyme, leukocyte common antigen, and CD43, but not of B-cell-specific or T-cell-specific antigens in both cases. Granulocytic sarcomas are apt to be misinterpreted as other hematolymphoid tumors, which may result in a significant error in management. The diagnosis should at least be thought of any time the diagnosis of malignant lymphoma or plasmacytoma of the testis is being considered.
 ...
PMID:Granulocytic sarcoma of the testis: a report of two cases of a neoplasm prone to misinterpretation. 911 Feb 93

A 66-year-old man was given a peripheral blood test because of low grade fever. Leukocytosis was detected, and the blood and bone marrow findings were consistent with those of chronic myelomonocytic leukemia. Three months later the hematological findings were: WBC 58,800/microliter (19% blastoid cells, 22% monocytes), Hb 9.0 g/dl, and a platelet count of 116 x 10(4)/microliters. A bone marrow examination revealed the presence of 52.6% blastoid cells and dysmegakaryocytopoiesis, including micromegakaryocytes. Serum and urinary lysozyme levels were elevated. Karyotypic analysis detected t(1; 3) (p36;q21), but not major bcr/abl mRNA. The patient was given a diagnosis of acute transformation of chronic myelomonocytic leukemia. Despite treatment, he died about 3 months later. t(1;3) is occasionally observed in cases of myelodysplastic syndrome (MDS) and leukemia. Patients with t(1;3) often exhibit dysmegakaryocytopoiesis; furthermore, acute leukemia develops more readily in those who also have MDS. Cases of long-term survival are rare.
 ...
PMID:[Acute transformation of chronic myelomonocytic leukemia with t(1;3) (p36;q21) abnormality]. 975 Apr 60


<< Previous 1 2 3 4 Next >>