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

An in vitro hematopoietic microenvironment was established from explained fragments of bone marrow from adult noninbred NIH Swiss mice with the use of corticosteroid-reconstituted horse serum. Infection with Kirsten murine sarcoma virus (Ki-MuSV) with either a Rauscher murine leukemia virus (R-MuLV) or Balb:virus-1 helper virus coat reduced proliferation of granulocytic and pluripotent hematopoietic stem cells and produced neoplastic transformation of both macrophages and preadipocytes in the adherent cell population within a 4-week period. Ki-MuSV-transformed, virus-releasing macrophages formed clusters of 4-49 cells in 0.8% methylcellulose-containing medium in the absence of added colony-stimulating factor (CSF), synthesized lysozyme, ASD-chloroacetate substrate-specific esterase-M, and CSF, and produced tumors following inoculation iv into adult NIH Swiss mice or ip into newborn NIH Swiss mice. In cultures infected with helper leukemia viruses R-MuLV or Balb:virus-1, gradual transformation of a distinct cell phenotype was observed over a 9-week period with generation of increasing numbers of atypical myeloblasts and promyelocytes which showed dyssynchronous nuclear-cytoplasmic maturation, basophilic granulation, cytoplasmic vacuolation, and formation of incompletely maturing CSF-dependent granulocyte-macrophage colonies in vitro and small spleen colonies in vivo. These data demonstrated that rapid biologic expression of the murine sarcoma virus genome in specific adherent "stromal" marrow cells prevents detection of a more subtle helper-virus-induced dysmyelopoiesis in a distinct nonadherent cell population.
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PMID:Phenotypically distinct target cells for murine sarcoma virus and murine leukemia virus marrow transformation in vitro. 21 35

Granulocytic sarcoma is an extramedullary tumor consisting of immature cells of the granulocytic series known to occur in patients with myelodysplastic syndrome, chronic myelogenous leukemia, or acute myelogenous leukemia. This tumor may involve nodes, cervix, bone and periosteum, and infrequently the small intestine. Granulocytic sarcoma rarely occurs in the colon and has not been previously described endoscopically. We encountered a 73-year-old man with myelodysplastic syndrome who presented with fever, diarrhea, and abdominal pain. Colonoscopic evaluation (focal ulceration, friability, and nodularity) was compatible with Crohn's disease, although histology showed a dense myeloid cell infiltrate characteristic of granulocytic sarcoma. In patients with myelodysplastic syndrome or acute or chronic myelogenous leukemia presenting with diarrhea, abdominal pain, and/or fever, colonoscopy and biopsy are indicated to determine if the colon is affected by granulocytic sarcoma.
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PMID:Granulocytic sarcoma of the colon. 198 52

A 20-year-old male developed both coccygeal and leg pain and followed by rectocystic disturbance. Disc herniation between L5 and S was suspected and laminectomy was performed. At surgery, an easily curretable tumor occupied the epidural space from L5 to the end of the sacrum. In part, the tumor spread out of the vertebral canal and invaded the surrounding muscle tissue. This muscle tissue and part of the lamina were checked histologically. Initial blood analysis revealed 5% blast-like cells, but failed to confirm them as leukemic cells. Histologically, the tumor cells had round or oval nuclei with large nucleoli and scanty cytoplasm without granulocytic differentiation. Malignant lymphoma or Ewing's sarcoma was initially suspected, but the definite diagnosis was uncertain. Immunohistochemical staining with the PAP method and enzyme histochemistry revealed that the tumor cells were positive for lysozyme and naphthol ASD chloracetate esterase. Thus, granulocytic sarcoma was finally diagnosed. Electron microscopic findings supported this diagnosis. Subsequent karyotyping of bone marrow cells revealed 8; 21 translocation, thus the final diagnosis of this patient was myelodysplastic syndrome, refractory anemia with excess blast cells in transformation or acute myelogenous leukemia, M2, by the FAB classification.
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PMID:A case of epidural granulocytic sarcoma preceding acute leukemia. 209 94

A patient with myelodysplastic syndrome (refractory anemia with excess of blasts in transformation, RAEB-T) presented with generalized granulocytic sarcomas involving the skin. The diagnosis was confirmed by skin and bone marrow biopsies. Partial myeloperoxidase deficiency was noted in the circulating polymorphonuclear leukocytes and the more differentiated tumor cells in the granulocytic sarcoma. This observation suggests that such leukocytes may be derived from the abnormal 'leukemic' clone.
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PMID:Myelodysplastic syndrome presenting with generalized cutaneous granulocytic sarcomas. 215 5

Granulocyte-macrophage colony-stimulating factor (GM-CSF) is a 23-kDa glycoprotein with remarkably diverse effects on immune and nonimmune cells. GM-CSF induces differentiation of granulocyte, macrophage, and eosinophil precursor cells. Proliferation of monocyte-macrophages, T lymphocytes, keratinocytes, and endothelial cells is also stimulated by GM-CSF. In addition, GM-CSF alters the functional properties of mature granulocytes, macrophages, eosinophils, and basophils. GM-CSF is produced by T lymphocytes, macrophages, and several cell types in extramedullary sites, where it may act in a paracrine manner to regulate the local response to antigenic challenge. Clinical trials of GM-CSF have been conducted in patients with AIDS, aplastic anemia, myelodysplastic syndromes, and sarcoma and following bone marrow transplantation and accidental radiation exposure. GM-CSF significantly increased circulating numbers of several myeloid cells and produced dose-dependent toxicity consisting primarily of myalgias, fever, fluid retention, and serosal effusions. Additional studies are needed to define the role of GM-CSF in treatment of patients with qualitative and quantitative dysfunction of immune cells.
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PMID:Granulocyte-macrophage colony-stimulating factor: pleiotropic cytokine with potential clinical usefulness. 240 68

Two cases of patients with prostatic granulocytic sarcoma in whom urinary obstruction occurred are presented. The diagnosis was made by tissue examination with hematoxylin and eosin and specific esterase stains. One patient had a myelodysplastic syndrome and the other patient had acute myeloblastic leukemia. In both cases the diagnosis of prostatic granulocytic sarcoma was unexpected. Granulocytic sarcomas should be considered in the differential diagnosis of urinary obstruction in patients with myeloproliferative or myelodysplastic syndromes.
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PMID:Granulocytic sarcoma of the prostate. Two cases causing urinary obstruction. 243 55

A case of granulocytic sarcoma (chloroma) of the temporal bone in a male child is presented. The diagnosis was confirmed by electron microscopic and histologic findings. The patient presented with otologic and neurological manifestations. CAT scan was at first normal but after some months a mass was apparent in the apex of the petrous bone and a manifestation of preleukemia appeared.
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PMID:Granulocytic sarcoma (chloroma) of the temporal bone. 262 92

The incidence of secondary myelodysplastic syndromes (MDS) and acute leukaemias (AL) after chemotherapy and/or radiotherapy is increasing. Most cases have been described in patients with Hodgkin's disease, non-Hodgkin's lymphomas, multiple myeloma, polycythemia vera, ovarian cancer and breast cancer. We report 2 patients with secondary MDS and acute myeloblastic leukaemia after combined chemotherapy and radiotherapy for soft tissue sarcoma. Five more cases have been described in the literature. The data of all patients are summarized. The occurrence of secondary MDS/AL in patients with soft tissue sarcoma may become a problem, in particular in children, who have been cured after combined radiotherapy and chemotherapy.
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PMID:Myelodysplastic syndrome and acute leukaemia following treatment of soft tissue sarcoma. 267 58

In the last 13 years splitting the pubic symphysis has been used in 46 patients 1 to 32 years old for wide access to the bladder neck and entire urethra in female, or the bladder neck and urethra down into the bulb in male patients. Each patient had a complex problem to reconstruct and most had undergone a prior operation. Underlying pathological conditions included exstrophy in 16 patients, epispadias in 11, cloacal exstrophy in 6, posterior urethral valves in 3, severe trauma in 2, bilateral single ectopic ureters in 2, radical surgery for sarcoma in 2, myelodysplasia in 2, cloacal anomaly in 1 and iatrogenic injury of the urethra in 1. The pubis, or interpubic ligament in exstrophy cases, was divided in the midline. The pubic rami were spread with a laminectomy spreader to create a gap sufficient to operate on that segment of the urethra, which is usually not accessible with an intact pubis. No patient had a problem of nonunion or pubic osteitis after using this approach. This series includes 2 patients undergoing simultaneous iliac osteotomies for a widened symphysis pubis. In no patient was a segment of pubis resected, as has been used in transpubic repair of certain traumatic urethral strictures.
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PMID:Splitting the pubis for exposure in difficult reconstructions for incontinence. 274 73

This report describes three unusual patients with lesions due to myeloblasts. In one instance, the patient presented with massive adenopathy. The second patient had bone lesions and a pathologic fracture. The third patient, with myelodysplasia, had diffuse skin lesions infiltrated with myeloblasts. These cases fit the diagnostic category of granulocytic sarcoma. Granulocytic sarcoma is a tumor of immature myeloid cells that may involve any site in the body but that most commonly affects the skin, soft tissues, lymph nodes, bone, and periosteum. Lesions can predate leukemia or occur late in an established chronic granulocytic leukemia or acute granulocytic leukemia. The most common presentation occurs late in the course of acute granulocytic leukemia or in chronic granulocytic leukemia as a herald to blastic transformation. Therapy for localized lesions is radiotherapy, which produces prompt shrinkage of the lesions but relapse occurs subsequently. Systemic chemotherapy also produces satisfactory clinical results. In all instances, therapy can only be considered palliative since virtually all patients have a short survival following the appearance of an extramedullary myeloblastic lesion. Recognition of this pathologic entity at an early stage may give us information on the best management for these patients.
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PMID:Granulocytic sarcoma: three unusual patients. 277 69


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