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)

The occurrence of adenopathy in patients with myelodysplastic syndrome-associated extramedullary myeloid cell tumors has rarely been reported. We describe a 7-year-old girl with juvenile myelomonocytic leukemia who showed the novel chromosomal abnormality t(9;12)(p22;q24.1) and who developed severe adenopathy of the cervical lymph nodes, tonsils, and adenoids that was manifested as granulocytic sarcoma. Following chemotherapy, the patient underwent a conditioning regimen of busulfan, cyclophosphamide, and total body irradiation followed by successful allogeneic bone marrow transplantation from her single HLA locus-mismatched mother at 6 months after her diagnosis. The patient continues to be well and in remission 3 years after stem cell transplantation.
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PMID:Granulocytic sarcoma presenting with severe adenopathy (cervical lymph nodes, tonsils, and adenoids) in a child with juvenile myelomonocytic leukemia and successful treatment with allogeneic bone marrow transplantation. 1548 50

This 2002 European Group for Blood and Marrow Transplantation (EBMT) activity survey concentrates on current status, increase and decrease in haematopoietic stem cell transplantation (HSCT) activity in Europe and investigates the association of transplant rates with team density. In 2002, there were 20 207 HSCT, 6915 allogeneic (34%), 13 292 autologous (66%) and 3947 additional re- or multiple transplants collected from 586 centres in 39 European countries. Main indications were leukaemias (6523 (32%; 76% allogeneic)); lymphomas (10 760 (53%; 92% autologous)); solid tumours (1913 (9%; 92% autologous)) and nonmalignant disorders (874 (4%; 92% allogeneic)). Compared to 2001, there were increases (>10%) for AML, ALL 1st CR, CML not 1st cP, MDS, SAA and CLL in allogeneic HSCT and for MDS, Ewing's sarcoma, soft-tissue sarcoma and ovarian cancer in autologous HSCT. Decreases (>10%) were observed in autologous HSCT for acute leukaemias beyond 1st CR, CML cP, glioma, breast cancer and lung cancer. Correlation of transplant rates (number of transplants per 10 million inhabitants) with team density (number of transplant teams per 10 million inhabitants) suggests different diffusion patterns for autologous compared to allogeneic HSCT. These data describe current practice for blood and marrow transplantation in Europe and give some hints about mechanisms involved in HSCT rates.
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PMID:Haematopoietic stem cell transplantation (HSCT) in Europe 2002. Changes in indication and impact of team density. A report of the EBMT activity survey. 1551 6

Our objective was to study the outcome of allogeneic hematopoietic stem cell transplantation (HSCT) for Shwachman-Diamond Syndrome (SDS). Among 71 SDS patients included in the French Severe Chronic Neutropenia Registry, 10 received HSCT between 1987 and 2004 in five institutions. The indications were bone marrow failure in five cases, and myelodysplastic syndrome (MDS) or leukemia in five cases. The median follow-up of patients who survived without relapse is 6.9 years (3.1-16.8 years). The conditioning regimen consisted of a busulfan-cyclophosphamide combination (n=6) or total body irradiation plus chemotherapy (n=4). Six patients received stem cells from unrelated donors and four from identical siblings. Engraftment was complete in eight patients and unassessable in two patients. These latter two patients died of infections 32 and 36 days after HSCT, with grade IV graft-versus-host disease and multiorgan dysfunction. A third patient died from an acute respiratory distress syndrome 17 months after HSCT with progressive granulocytic sarcoma. One patient had an MDS relapse 4 months after HSCT and died 10 months later. The overall 5-year event-free survival rate is 60+/-15%. We conclude that HSCT is feasible for patients with SDS who develop bone marrow failure or malignant transformation.
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PMID:Hematopoietic stem cell transplantation for Shwachman-Diamond syndrome: experience of the French neutropenia registry. 1615 25

Myeloid sarcoma (MS) is a malignant tumour of myeloblasts rarely occurring in the maxillary bone. The tumour may precede or be concurrent with leukaemic infiltration of the bone marrow or herald blastic transformation of a myelodysplastic syndrome or a chronic myeloproliferative disorder. Myeloid sarcoma is uncommon in the oral cavity, but it can involve the palate, gingiva, extraction socket, and cheek. Recognition and diagnosis of myeloid sarcoma involving the soft tissues of the oral cavity in an otherwise asymptomatic patient is important and mandates an appropriate haematological diagnostic workup. We herein report on a new case without any evidence of haematological disorders. We discuss the pathological diagnosis and the therapeutical approaches.
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PMID:Myeloid sarcoma of the maxillary bone. 1651 76

A 43-year-old woman with a past medical history of breast cancer and an acute myeloid leukemia (AML) presented with headache over a 3-week period. The clinical examination was completely unremarkable. CT and MRI scans showed a contrast enhancing lesion in the left temporal lobe. Histopathologic examination revealed a malignant, hematopoietic tumor with high mitotic activity, areas of necrosis and diffuse infiltration of the brain parenchyma. Positive staining for Chloroacetateesterase and lysozyme of tumor cells identified its myeloid lineage. The diagnosis was granulocytic sarcoma (GS)/chloroma, a metastatic manifestation of AML. Granulocytic sarcoma (GS) most often occurs in patients with AML, myelodysplastic syndromes and myeloproliferative disorders, and can involve any organ. However intracerebral manifestation of GS is a rare event. In this case histopathological features and differential diagnoses of intracerebral GS are discussed.
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PMID:A 43-year-old woman with a temporal mass. 1661 89

Granulocytic sarcomas (chloromas) are rare extramedullary tumors consisting of primitive granulocytic cells. They arise de novo, or are associated with other hematologic disorders such as acute myeloid leukemia, myelodysplastic syndrome, or myeloproliferative disorders. We report here on a case of a 62-year-old woman who presented with a large swelling in her right groin and leg. The mass was confirmed by biopsy to be a granulocytic sarcoma. Bone marrow examination showed mild hypercellularity but no evidence of increase in blast count. However, cytogenetic examination of the marrow showed t(9;22), indicating an unexpected diagnosis of chronic myeloid leukemia.
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PMID:Case report: A large extramedullary granulocytic sarcoma as the initial presenting feature of chronic myeloid leukemia. 1661 45

We identified a reciprocal translocation between chromosomes 3 and 8, with breakpoints at bands 3q26 and 8q24, in five patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). The t(3;8)(q26;q24) was the sole cytogenetic aberration in two patients, was associated with trisomy 13 in one patient, and occurred with monosomy 7 in two patients. In three patients, the AML or MDS developed 36, 52, and 57 months following chemotherapy for soft tissue sarcoma, mantle cell lymphoma, and diffuse large B-cell lymphoma, respectively; in these three patients, the neoplasms were considered to be therapy-related. All five patients displayed marked trilineage dysplasia and variable degrees of cytopenias, with marked thrombocytosis noted in one patient and a normal platelet count in another patient. All patients were treated with combination chemotherapy; at writing, four were still alive and one had died during a follow-up period ranging from 1 to 16 months. We conclude that the t(3;8)(q26;q24) is a recurrent translocation associated with therapy-related MDS/AML or de novo AML, and is frequently associated with monosomy 7.
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PMID:Translocation (3;8)(q26;q24): a recurrent chromosomal abnormality in myelodysplastic syndrome and acute myeloid leukemia. 1661 15

Granulocytic sarcoma rarely involves the orbit. It is the local proliferation of immature myeloid cells and occurs most often in patients suffering from myelodysplastic syndromes. We report a case of granulocytic sarcoma that revealed acute myeloid leukemia in a patient suffering from chronic myelomonocytic leukemia. At the time of diagnosis, the lesion involved the nasal fossae, the ethmoidal sinus, and the orbit, with subperiosteal tumoral infiltration toward the skin. The treatment involved surgery and radiotherapy. The patient died 3 months later of complications of the hematological disease. This observation is original for the occurrence of granulocytic sarcoma during chronic and well-known leukemia, the age of onset, and the fatal outcome in just a few weeks. In this observation, the ophthalmologist revealed the acute nature of the myelodysplastic syndrome.
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PMID:[Granulocytic sarcoma in a 90-year-old patient]. 1707 18

Thirty-two cases of granulocytic sarcoma (GS) are reported in this paper. Age range was from 16 - 70 years. GS was accompanied by AML in 13 cases, ALL (My+) in one case, CML in 11 cases and MDS in two cases. GS was diagnosed simultaneously with leukemia in five cases and preceded the leukemia in eight. Lymph node and soft tissue were the most commonly detected localizations. Seven cases had first been diagnosed as NHL. Histopathologically blastic, immature and mature variants were found in 11, nine and 11 cases respectively and overall survival was shortest in the blastic type. Myeloperoxidase and lysozyme were found to be positive in 30 and 24 cases respectively. Therapy was radiation in five cases and surgery in three. Systemic chemotherapy was given to the cases. The clinical outcome of the patients after the diagnosis of GS was poor. GS is a unique entity; prognosis is poor but it is important to detect the signaling pathways associated with migration of myeloid cells to the extra-medullary tissues. The critical factors for detecting this interesting tumor are to be aware of this disease, cooperation between clinician and pathologist and the application of special stains to detect the myeloid origin.
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PMID:Granulocytic sarcoma: 32 cases and review of the literature. 1716 97

Non-Hodgkin lymphomas (NHLs) are often characterized by specific cytogenetic abnormalities. We evaluate the utility of routine cytogenetic studies in 261 "lymphoma work-ups". These include 4 non-hematolymphoid malignancies and 257 hematolymphoid processes submitted over 3 years, including even those initially appearing benign by morphology and immunophenotyping. About 64/257 yielded no results and 5/78 "lymphoid hyperplasia/lymphadenitis" were abnormal; 3 of these 5 appeared clonal [1, shortly followed by follicular lymphoma (FL)]. Increasing FL grades showed decreased t(14;18)/increased del 6q abnormalities. 1/4 Burkitt lymphomas (BL) (i.e., an atypical BL) showed t(8;14) and t(14;18) (i.e., a double-hit). 1/4 post-transplant lymphoproliferative disorders (PTLD) showed abnormalities, confirming clonality. One "extramedullary hematopoiesis" with a previous myelodysplastic syndrome and 1 erythroblastic sarcoma showed abnormalities, confirming myelodysplasias (MDS). A monocytic sarcoma revealed a t(9;11)(p22;q23). Routine cytogenetic studies aid in "lymphoma work-ups" by (1) detecting rare abnormalities in cases without apparent malignancy, indicating close follow-up, (2) detecting abnormalities in FL correlating with increasing grade, (3) detecting co-existent t(8;14) and t(14;18) in BL, indicating a worst prognosis, (4) establishing clonality in PTLD and (5) establishing diagnoses of MDS or chloroma in tissues. The findings by the conventional karyotyping studies in many of these cases added significant data to the diagnostic cases, beyond morphologic and immunophenotypic findings, and were not amenable to directed fluorescent-in-situ hybridization studies.
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PMID:Usefulness of routine conventional cytogenetic analysis in tissues submitted for "lymphoma work-up". 1820 1


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