Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

7-year-old girl treated for acute lymphoblastic leukaemia since June 1995 after marrow relapse (June 1997) presented with painful oedema of the upper part of left hip and limping, 16 months after allogeneic bone marrow transplantation (BMT). Bone marrow examination excluded medullary relapse. Histopathological investigation of periosteum and bone scrapings revealed massive leukemic infiltration. Radiotherapy resulted in local arrest of the malignant process. However, bone marrow relapse was diagnosed in the child two months later with subsequent death after one month.
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PMID:[Extramedullary (bone) relapse 16 months after allo bmt in a 7-year-old girl treated for acute lymphoblastic leukaemia from the second year of life]. 1200 50

This study sought to develop an in vivo gene therapy to accelerate the repair of bone fractures. In vivo administration of an engineered viral vector to promote fracture healing represents a potential high-efficacy, low-risk procedure. We selected a murine leukemia virus (MLV)-based retroviral vector, because this vector would be expected to target transgene expression to the proliferating periosteal cells arising shortly after bone fracture. This vector transduced a hybrid gene that consisted of a bone morphogenetic protein (BMP)-4 transgene with the BMP-2 secretory signal to enhance the secretion of mature BMP-4. The MLV vector expressing this BMP-2/4 hybrid gene or beta-galactosidase control gene was administered at the lateral side of the fracture periosteum at 1 day after fracture in the rat femoral fracture model. X-ray examination by radiograph and peripheral quantitative computed tomography at 7, 14, and 28 days after fracture revealed a highly significant enhancement of fracture tissue size in the MLV-BMP-2/4-treated fractures compared to the control fractures. The tissue was extensively ossified at 14 and 28 days, and the newly formed bone exhibited normal bone histology. This tissue also exhibited strong immunohistochemical staining of BMP-4. Additional control and MLV-BMP-2/4-treated animals each were monitored for 70 days to determine the fate of the markedly enhanced fracture callus. Radiographs showed that the hard callus had been remodeled and substantial healing at the fracture site had occurred, suggesting that the union of the bone at the fracture site was at least as high in the BMP-4-treated bone as in the control bone. There was no evidence of viral vector infection of extraskeletal tissues, suggesting that this in vivo gene therapy for fracture repair is safe. In summary, we have demonstrated for the first time that a MLV-based retroviral vector is a safe and effective means of introducing a transgene to a fracture site and that this procedure caused an enormous augmentation of fracture bone formation.
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PMID:In vivo bone formation in fracture repair induced by direct retroviral-based gene therapy with bone morphogenetic protein-4. 1281 Jan 66

Extra medullary deposits are often associated with myeloproliferative disorders, especially with myeloid neoplasia. These are called granulocytic sarcoma, aleukemic leukemia cutis, myeloblastoma, chloroleukemia, extra-medullary myeloid tumor, and chloroma. They commonly present in the bone, periosteum, soft tissue, lymph nodes, and the skin but can occur anywhere. The authors present their experience with a patient with no history of neoplasm presented with a nonhealing ulcer of the lower limb that was a chloroma associated with chronic myeloid leukemia.
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PMID:Chronic lower limb ulcer? No--chloroma! 1586 42

Granulocytic sarcoma is an extramedullary tumor composed of immature granulocytic precursor cells. The most common sites of presentation are bone, periosteum, soft tissue, lymph node, skin, and infrequently small intestine. The tumor may develop during the course of acute myeloid leukemia, chronic myeloid leukemia or other myelodysplastic disorders. It can occur without blood or bone marrow manifestations of leukemia and in this case, the diagnosis is difficult. Our patient was initially diagnosed as a case of T-cell non Hodgkin's lymphoma and received one cycle of CHOP with only transient improvement in his symptoms. Subsequently, his biopsy slides were reviewed at our centre and were reported as granulocytic sarcoma.
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PMID:Granulocytic sarcoma in the absence of acute myeloid leukemia: a case report. 1747 71

Granulocytic sarcoma is a rare variant of a myeloid malignancy, which shows an extra-medullary tumour mass which is composed of myeloblasts and myeloid precursors with varying degrees of differentiation. It occurs most commonly in bone, periosteum, soft tissue, lymph nodes, and skin; although it can occur anywhere throughout the body. Here, we are reporting two cases of orbital granulocytic sarcoma in children, which presented clinically with proptosis and periorbital swellings, which were first diagnosed by Fine Needle Aspiration Cytology (FNAC). Later, peripheral blood and bone marrow aspirate examinations revealed the evidence of Acute Myeloid Leukaemia (AML). These cases are being documented to demonstrate the utility and diagnostic accuracy of FNAC in evaluation of this entity, in cases of unsuspected AML. Recognition of this rare entity is important, because giving an early aggressive chemotherapy can cause regression of the tumour and thus improve the patient survival.
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PMID:Granulocytic sarcoma presenting as an orbital mass: report of two cases. 2408 83

Myeloid sarcoma (MS) is a rare extramedullary tumor composed of immature cells of myeloid lineage that destroy the original tissue architecture in which it is found. It is most commonly identified in patients with acute myelogenous leukemia, and less often in myelodysplastic syndromes (MDSs) and other myeloproliferative disorders. It is most commonly reported in the periosteum, bone, skin, and lymph nodes but has been reported in many other sites of the body. Herein, we describe a case of MS involving the periprostatic tissue and review of literature of MS of the prostate. Our patient was initially diagnosed with MDS and was in remission following successful treatment. Six months later, the patient was diagnosed with prostate adenocarcinoma, and MS of the periprostatic tissue was incidentally discovered in the postprostatectomy pathology specimen. An extensive review of literature from 1997 to 2014 revealed a total of eight cases of MS involving the prostate. Of the eight cases of MS of the prostate, four were primary MS (absence of a history of leukemia) and four were secondary MS. Three received local radiation to the prostate with relief of obstructive symptoms, and one of them had a repeat prostate biopsy negative for leukemic cells. Despite being a rare entity, MS should be considered as a differential diagnosis of soft tissue masses, especially in patients with a history of hematological malignancies.
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PMID:Myeloid sarcoma of the periprostatic tissue and prostate: Case report and review of literature. 2745 59

Myeloid sarcoma (MS) is a very rare malignant tumour composed of myeloblasts. It most commonly involves soft tissue, bone, periosteum and lymph nodes, but unusual presentation sites have also been reported. Typically, MS evolves concurrently with active leukaemia or following remission, when it is known as secondary MS. But rarely MS can occur de novo without evidence of concomitant haematological disease. Herein, we report an unusual case of central nervous system-MS in a patient without evidence of concomitant haematological disease. In this case, progressive thoracic and lumbar pain with paraplegia ultimately led to the diagnosis of acute myeloid leukaemia. We also conducted a PubMed search for case reports, case series and reviews of past literature regarding central nervous system-MS and report our findings.
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PMID:Liquid Presenting as Solid: A Rare Presentation of Acute Myeloid Leukaemia as a Solid Epidural Mass. 3319 52