1/14. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/14. Sclerosing extramedullary hematopoietic tumor in chronic myeloproliferative disorders.Sclerosing extramedullary hematopoietic tumor (SEMHT) occasionally may arise in patients with chronic myeloproliferative disorders (CMPDs). Morphologically, these tumors may be mistaken for sarcomas or other neoplasms, especially if the clinical history is unknown. We analyzed four cases to identify features to aid in this differential diagnosis. Clinically, there were four men (mean age, 64.5 years), each with a history of CMPD. Grossly, the SEMHTs formed solitary renal or perirenal masses or multiple mesenteric or omental nodules. Morphologically, each SEMHT had a sclerotic to myxoid background with thick collagen strands and trapped fat. Atypical megakaryocytes, maturing granulocytic and erythroid precursors, and few to no blasts were identified in all cases. The megakaryocytes, granulocytic precursors, and erythroid precursors reacted strongly with antibodies to factor viii, myeloperoxidase, and hemoglobin, respectively, in immunohistochemical studies performed in selected cases. SEMHT is a rare manifestation of CMPD that may be mistaken for a sarcoma, especially sclerosing liposarcoma, Hodgkin's disease, especially lymphocyte depletion type, or a myelolipoma. In a myxoid tumor with trapped fat and atypical cells, morphologic and immunohistochemical identification of maturing hematopoietic precursors helps distinguish SEMHT from sarcoma or Hodgkin's disease. The presence of sclerosis and atypical megakaryocytes helps distinguish SEMHT from myelolipoma.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/14. Combined defect in membrane expression and activation of platelet GPIIb--IIIa complex without primary sequence abnormalities in myeloproliferative disease.Defects in glycoprotein (GP)IIb-IIIa or in its activation may cause abnormal platelet aggregation and a bleeding diathesis. We report studies in a 67-year-old man with a myeloproliferative disease and markedly abnormal platelet responses. By flow cytometry, platelet binding of two complex-specific anti-GPIIb-IIIa monoclonal antibodies (mAbs), A2A9 and 10E5, was approximately 50% of normal. An enzyme-linked immunosorbent assay (ELISA) using immobilized kistrin showed 18% of normal membrane GPIIb-IIIa complex. By immunoblot analysis, GPIIb and GPIIIa levels in platelet lysates and membranes were near normal. Activation of GPIIb-IIIa, monitored with mAb PAC-1, was markedly decreased (< 20% of normal) in response to ADP, thrombin and platelet-activating factor (PAF); expression of ligand-induced binding sites (LIBS) was < or = 30% of normal. signal transduction-independent LIBS expression, induced by echistatin, was approximately 60% of normal, suggesting that the integrin present had intact ligand-binding capability. sequence analysis of GPIIb and GPIIIa cDNA, and platelet mRNA levels for both subunits, were normal. These findings document an acquired combined defect in membrane expression (secondary to a defect in post-translational processing of the complex) and inside-out signalling-dependent activation of the GPIIb-IIIa complex.- - - - - - - - - - ranking = 0.11368163372985keywords = complex (Clic here for more details about this article) |
4/14. An acquired factor viii inhibitor in association with a myeloproliferative/myelodysplastic disorder presenting with severe subcutaneous haemorrhage.We report a 71-year-old man who presented with severe subcutaneous and later psoas muscle haemorrhage in the presence of a raised white cell count and hepatosplenomegaly. A circulating factor viii (FVIII) inhibitor was detected and bone marrow morphology confirmed the presence of a myeloproliferative/myelodysplastic disorder. Initial treatment with high dose FVIII followed by recombinant factor viia was unsuccessful. Haemorrhage was controlled by the administration of activated prothrombin complex concentrate (FEIBA; Baxter healthcare, CA, USA) in combination with prednisolone, cyclophosphamide and i.v. immunoglobulin. The inhibitor became undetectable 14 weeks after presentation. The white cell count responded initially to hydroxyurea and later to cyclophosphamide. There have been only two previous reports of acquired haemophilia A in association with myelodysplastic disorders and no previous report of an association with a myeloproliferative disorder.- - - - - - - - - - ranking = 0.014210204216232keywords = complex (Clic here for more details about this article) |
5/14. myeloproliferative disorders: usefulness of X-linked probes in diagnosis.myeloproliferative disorders are neoplasms of the pluripotent hematopoietic stem cell. Accurate diagnosis and distinction from reactive processes can be difficult therein with cytogenetic analysis only being useful in a minority of patients. Use of X-linked restriction fragment length polymorphism and methylation analysis has enabled clonal analysis to be performed in up to 50% of females, significantly increasing the proportion of analyzable patients over methods dependent on glucose-6-phosphate dehydrogenase heterozygosity. Using hypoxanthine phosphoribosyl transferase and phosphoglycerate kinase probes, we have demonstrated monoclonality of peripheral blood leukocytes in three females with myeloproliferative disorders who had uninformative chromosomal analysis. This technique greatly enhances the diagnosis of early myeloproliferative disorder.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/14. association of the philadelphia chromosome and 5q- in secondary blood disorder.A patient developed a secondary blood disorder 7 years after radiotherapy for a gastric lymphoma. The initial myelodysplastic syndrome evolved to a myeloproliferative phase with transient polycythemia, progressive thrombocythemia, and hyperleukocytosis. Chromosome analysis performed in the terminal phase showed del(5)(q13q31),t(9;22)(q34;q11), and a complex rearrangement involving chromosomes #2 and #3. A correlation between chromosomal abnormalities and hematologic findings could be established. In this case, we have assumed that the Philadelphia translocation is a late event, due to prior mutagen exposure, and its association with a common secondary abnormality (5q-), followed by a progressively developing myeloproliferative phase. Furthermore, the association of Ph and 5q- in a single clone seems to indicate that the same stem cell is affected by these two abnormalities.- - - - - - - - - - ranking = 0.014210204216232keywords = complex (Clic here for more details about this article) |
7/14. Chromosomal changes in secondary leukemias of childhood and young adulthood.The increasing success of antineoplastic therapy has resulted in a growing number of long-term survivors. These people are at risk for complications of the therapy itself. Among these induced acute nonlymphoid leukemia (ANLL) has been both common and often lethal. We reviewed 72 recently reported patients under 30 years of age at the time of initial diagnosis who developed a secondary, karyotypically defined leukemia. Fifty-eight patients contracted ANLL a mean of 4 1/2 years from the initial diagnosis. In 25 patients, this was preceded by a preleukemic phase characterized by a hypercellular bone marrow with abnormal precursors, often accompanied by peripheral pancytopenia, that lasted a mean of 6 months. Three additional patients died in this preleukemic phase. In all 61, the most common chromosomal abnormalities were numerical errors. Twenty-four patients had a hypodiploid karyotype, most often in those in whom the primary diagnosis was lymphoma (22 of 43). The most common chromosomes missing in whole or in part were number 7 (18 patients), number 5 (8 patients), number 17 (5 patients), and number 21 (4 patients). The anomalies were frequently multiple and complex. monosomy 7 figured particularly strongly and may be similar to a karyotypically identical myeloproliferative disorder characterized by micromegakaryocytes, giant platelets, and abnormal granulocyte function arising de novo in children. These findings are similar to those in older patients with ANLL induced by environmental carcinogens or antineoplastic therapy. They are different from the karyotypic changes seen in de novo ANLL in children and young adults, suggesting a different etiology. Also, they reinforce the need to find less leukemogenic treatment programs.- - - - - - - - - - ranking = 0.014210204216232keywords = complex (Clic here for more details about this article) |
8/14. A case of dysmyelopoietic syndrome with hypotetraploid karyotype.We present a patient with dysmyelopoietic syndrome and with a complex, hypotetraploid karyotype with numerous structural aberrations.- - - - - - - - - - ranking = 0.014210204216232keywords = complex (Clic here for more details about this article) |
9/14. hypereosinophilic syndrome and plasmocytoma. Report of a case and review of the literature.The 4th case of plasma cell neoplasm associated with a hypereosinophilic syndrome is described and compared with the previous reports. Hypereosinophilia in the present patient displayed some borderline traits with eosinophilic leukemia. myeloproliferative disorders of the eosinophilic line often present as a precancerous state, but sometimes they seem to acquire malignant independence. In our patient the occurrence of a plasmocytoma with a dramatic course leads to suspect an underlying complex genetic aberration.- - - - - - - - - - ranking = 1.0142102042162keywords = neoplasm, complex (Clic here for more details about this article) |
10/14. Myeloproliferative and lymphoproliferative disorders in the same patient.A 67-year-old man developed a Philadelphia-negative myeloproliferative disorder which initially manifested as marked thrombocytosis, followed two years later by marked leucocytosis. He subsequently developed an IgG lambda plasma cell dyscrasia together with a monoclonal proliferation of circulating lymphocytes with IgM kappa surface immunoglobulin. The lymphoid neoplasm was associated with a rapidly progressive and fatal course.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
| | Next -> |