Cases reported "Myelodysplastic Syndromes"

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1/71. Untreated chronic lymphocytic leukemia concurrent with or followed by acute myelogenous leukemia or myelodysplastic syndrome. A report of five cases and review of the literature.

    Although it has been known that patients with chronic lymphocytic leukemia (CLL) have a higher frequency of second malignant neoplasms, the development of acute myelogenous leukemia (AML) or myelodysplastic syndrome (MDS) in these patients is extremely rare. Most reported cases have been therapy-related. In this article, we report the clinical and immunophenotypic features of 5 cases of untreated CLL concurrent with or followed by the development of AML or MDS. All 5 patients were men, with ages ranging from 57 to 87 years (mean, 73.8 years). Four patients had AML and 1 patient had refractory anemia with ringed sideroblasts. In the 4 cases of AML and CLL, 2 distinct cell populations (i.e., myeloblasts and lymphocytes) were identified morphologically and/or immunophenotypically. Our findings support that this rare concurrence of AML or MDS and untreated CLL may represent 2 separate disease processes.
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keywords = neoplasm
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2/71. Myelodysplastic syndrome with monosomy 5 and/or 7 following therapy with 2-chloro-2'-deoxyadenosine.

    A few cases of secondary neoplasms occurring after treatment with 2-chloro-2'-deoxyadenosine (2CdA) have been reported, mostly in patients previously exposed to other anti-cancer drugs including alkylating agents (AA). Here we report on the occurrence of a myelodysplastic syndrome (MDS) with monosomy 5 and/or 7 in two patients after 2CdA treatment, without or prior to other toxic exposure. In light of a literature review and given the involvement of chromosomes frequently abnormal in secondary leukaemias, we suggest that 2CdA may induce therapy-related MDS (t-MDS).
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3/71. Transient monosomy 7: a case series in children and review of the literature.

    BACKGROUND: monosomy 7 and deletions of the long arm of chromosome 7 [del (7q)] are recurrent, nonrandom chromosomal abnormalities associated with both de novo and therapy-related myelodysplastic syndromes (MDS). The overall prognosis for children and adults with these chromosomal abnormalities is poor. In the current report, the authors present five children with MDS associated with monosomy 7/del(7q) who achieved spontaneous hematologic disease remission as well as a review of the literature. methods: Five children with either de novo or treatment-related MDS who achieved spontaneous hematologic disease remission are presented. Relevant clinical, cytogenetic, and fluorescent in situ hybridization data are included. RESULTS: All patients were boys. Three had de novo MDS whereas two others previously had received chemotherapy for another malignancy. Four patients achieved spontaneous and durable hematologic disease remission that was associated with cytogenetic disease remission in all three patients tested. The fifth patient developed a disease recurrence and died with evidence of clonal evolution after a long interval of hematologic and cytogenetic remission. CONCLUSIONS: A subset of children who develop MDS associated with monosomy 7 or del(7q) achieve spontaneous hematologic and cytogenetic improvement. Although this appears to be uncommon, further data are needed to determine the percentage of patients who improve without therapy and to define clinical characteristics that may predict this clinical outcome. These findings suggest that monosomy 7/del(7q) is insufficient to produce full leukemic transformation.
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ranking = 4.4456591874769
keywords = malignancy
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4/71. thrombocytosis with sideroblastic erythropoiesis: a mixed myeloproliferative myelodysplastic syndrome.

    Some patients with haematological neoplasms have features which overlap between a myelodysplastic syndrome and a myeloproliferative disorder. Two such patients are reported, both having sideroblastic erythropoiesis and thrombocytosis and one sequentially developing features of atypical chronic myeloid leukaemia, idiopathic myelofibrosis and acute megakaryoblastic leukaemia. The prevalence of thrombocytosis among cases of refractory anaemia with ring sideroblasts may be as high as 15-20% and has implications for choice of therapy.
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5/71. Extramedullary myeloid cell tumor of the urinary bladder in a patient with myelodysplastic syndrome.

    We report a case of extramedullary myeloid cell tumor of the urinary bladder in an elderly male with a three year history of myelodysplastic syndrome (refractory anemia with excess blasts), noninvasive papillary transitional cell carcinoma of the urinary bladder, and in situ transitional cell carcinoma of the left ureter. light microscopy demonstrated a poorly differentiated neoplasm composed of medium to large cells with eosinophilic cytoplasm. The tumor cells showed immunohistochemical expression of myeloperoxidase, lysozyme, CD15, CD68 and CD43. bone marrow examination following cystectomy demonstrated refractory anemia with excess blasts (6-10%) and a normal karyotype. cytogenetics, approximately 1 year after cystectomy, demonstrated a deletion of the short arm of chromosome number 12. Four years after presentation, the patient succumbed to pulmonary aspergillosis.
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6/71. A patient with genetic deletion of glutathione-S-transferase T1 and M1 who developed non-small-cell lung cancer and myelodysplastic syndromes.

    glutathione S-transferase (GST) M1 polymorphism is a marker for susceptibility to smoking-related neoplasms, such as lung and bladder cancer. Recently, a genetic deletion of GSTT1, an isoenzyme of GST, has been reported to be associated with myelodysplastic syndromes (MDS). A 59-year-old man with a long-term smoking habit was treated successfully for non-small-cell lung cancer. Four years after the surgical removal of his lung cancer, he developed MDS and died. Using a polymerase chain reaction-based genotyping method, he was found to have a deletion of both the GSTM1 and GSTT1 genes. Screening for the deletion of the GSTM1 and GSTT1 genes may be useful for assessing individual genetic susceptibility to smoking-related lung cancer and MDS.
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ranking = 1
keywords = neoplasm
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7/71. Myelodysplastic syndrome associated with monosomy 7 in a child with bloom syndrome.

    bloom syndrome is a genomic instability syndrome associated with predisposition to development of various types of malignancy. In this report, we described a 7-year-old boy with bloom syndrome (BS) and myelodysplastic syndrome (MDS) associated with monosomy 7 and loss of the y chromosome. To our knowledge, this was the first case with BS showing monosomy 7 and MDS during the early childhood period.
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ranking = 4.4456591874769
keywords = malignancy
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8/71. Relapse of acute myelogenous leukemia as a cerebellar myeloblastoma showing megakaryoblastic differentiation.

    Myeloblastomas (granulocytic sarcomas) occurring within the central nervous system (CNS) are extremely rare lesions that may develop in patients with acute or chronic myeloproliferative disorders. The majority of such lesions involve brain or spinal cord by contiguous spread from meningeal or bony sites, rather than originating within the CNS parenchyma. We describe a patient with acute myelogenous leukemia in remission, who developed a purely intraparenchymal cerebellar myeloblastoma with megakaryocytic differentiation. The neoplastic cells expressed the megakaryocytic markers factor viii-related antigen and platelet glycoprotein-IIIa (CD61), and showed ultrastructural features that were indicative of megakaryocytic differentiation. Clinically, myeloblastomas of the CNS invoke a broad differential diagnosis that includes abscess, hemorrhage, and metastatic neoplasms because of their intraparenchymal location and radiologic features. Although they are rare, myeloblastomas should be included in the histopathologic differential diagnosis of a poorly differentiated neoplasm occurring within the CNS, particularly in a patient with a history of myeloproliferative or myelodysplastic disease.
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keywords = neoplasm
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9/71. trisomy 15, sex chromosome loss, and hematological malignancy.

    We report 6 patients with myelodysplasia who, on routine cytogenetic studies, demonstrated trisomy 15. Four of these also had sex chromosome loss. A review of the literature revealed 6 other cases of trisomy 15 with sex chromosome loss and 22 cases of trisomy 15 as the sole chromosomal abnormality. All cases had hematologic malignancy or myelodysplasia. trisomy 15 is uncommon but tends to be associated with myelodysplasia in older subjects, and with sex chromosome loss in about one third of cases.
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ranking = 22.228295937385
keywords = malignancy
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10/71. Successful bone marrow transplantation in an adult patient with reactive hemophagocytic syndrome associated with myelodysplastic syndrome.

    We report an adult case of reactive hemophagocytic syndrome (RHS) associated with myelodysplastic syndrome (MDS) who received emergency bone marrow transplantation (BMT). Despite methylprednisolone pulse therapy, high-dose gamma-globulin, and chemotherapy containing etoposide, the pancytopenia progressed. After informed consent, the patient underwent syngeneic BMT using melphalan as the conditioning regimen. The patient has been well without relapse of RHS and MDS for more than 2 years after BMT. This result suggests that the above strategy, including BMT, should be considered for the treatment of adult RHS associated with hematological malignancy.
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ranking = 4.4456591874769
keywords = malignancy
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