1/132. Translocation (4;15)(p16;q24): a novel reciprocal translocation in a patient with BCR/ABL negative myeloproliferative syndrome progressing to blastic phase.A patient with BCR/ABL negative myeloproliferative syndrome with a 46,XY,del(3)(q21), t(4;15)(p16;q24) karyotype is described. fluorescence in situ hybridization performed with chromosomes 4 and 15 painting probes confirmed a novel reciprocal (4;15) translocation. The absence of crkl tyrosine phosphorylation, no activation of the abl kinase as measured by autophosphorylation, and a normal-size abl transcript suggest an alternative mechanism for leukemogenesis to that operative in Ph positive BCR/ABL positive chronic myeloid leukemia. A number of genes potentially relevant to tumorigenesis, some involving the ras signaling pathway, map to the 4p16 and 15q24 chromosome regions.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/132. ALL- and CML-type BCR/ABL mRNA transcripts in chronic myelogenous leukemia and related disorders.Using the reverse transcription polymerase chain reaction, we investigated acute lymphoid leukemia (ALL)-type, and chronic myelogenous leukemia (CML)-type BCR/ABL mRNA expression in a total of 66 patients with chronic myeloproliferative disorder (CMPD). Thirty-six of 37 patients with CML were positive for CML-type mRNA. Thirteen of the 25 CML had ALL-type mRNA expression. The patients with ET, PV, MF, and CMML did not have any detectable BCR/ABL expression. The most remarkable finding was that two patients, a Ph1-positive CML patient and a patient with a presumptive diagnosis of essential thrombocythemia (ET), showed only ALL-type chimeric mRNA expression.- - - - - - - - - - ranking = 53.38999626848keywords = chronic myelogenous leukemia, chronic myelogenous, leukemia, myelogenous leukemia, myelogenous (Clic here for more details about this article) |
3/132. A group of previously not recognized cytogenetic abnormalities in myeloid hematological malignancies.We have identified a group of previously not reported chromosome abnormalities related to myeloid hematological malignancies. Cases 1 and 2 were observed to have an additional i(4)(p10) as the sole anomaly with similar clinical features of myeloid disorders; that is, acute nonlymphocytic leukemia (ANLL-M2) and myelodysplastic syndrome (MDS)-refractory anemia with an excess of blasts in transformation, respectively. fluorescence in situ hybridization studies with the use of a 4p-specific microdissection probe further confirmed the presence of an i(4)(p10) in these patients. Case 3 was diagnosed with ANLL-M1 and had an additional i(8)(p10) as the only change, also confirmed by a whole-chromosome painting procedure. In cases 4-6, deletions of 18q at breakpoints q12, q23, and q21 were identified as the sole anomaly in a myeloproliferative disorder (MPD), MPD, and MDS, respectively. X-autosome translocations other than t(X;10)(p11;p11) and t(X;11)(q13;q23) have not been reported as recurrent or primary changes in hematological disorders. In the present study, a t(X;9)(q26;q22) and t(X;5)(q13;q33) as the sole anomaly were found in cases 7 and 8, respectively. Both cases had the same diagnosis of MDS. Considering that trisomies 4 ( 4) and 8 ( 8) are common anomalies in MDS and ANLL, our findings strongly indicate that amplification of genes on 4p and 8p, but not on 4q and 8q, may play a crucial role in the pathogenesis of MDS and ANLL. In addition, genes on 18q12-23 and on Xq13-26 may be involved in the pathogenesis of myeloid disorders.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
4/132. Cutaneous ulcers associated with hydroxyurea therapy in myeloproliferative disorders.hydroxyurea (HU) is an established chemotherapeutic agent in the treatment of myeloproliferative disorders (MPD) including chronic myelogenous leukemia (CML), polycythemia vera and essential thrombocythemia (ET). It is well tolerated, has minimal toxicities, and produces hematological response in most patients treated. Side effects of hydroxyurea are few and include myelosuppression, oral ulcers and skin rashes. Cutaneous toxicity is rare. This study aims to describe the occurrence of cutaneous ulcerations attributed to HU therapy in patients with MPD, and familiarize the oncology community with this unusual but disturbing toxicity of HU. Five patients with MPD receiving HU therapy at doses of 0.5 to 4 g/day who developed skin ulceration were reviewed (median age was 53 years). Three patients had philadelphia positive CML, and two had ET. Cutaneous ulcers developed after a long period of HU therapy (median 36 months, range 7 to 96 months). The time after discontinuation of HU to the healing of the ulcers was 1 to 4 months. Ulcers developed mainly in the lower extremities particularly adjacent to the malleoli, indicating a possible relation to trauma. In conclusion, cutaneous ulceration represents a poorly recognized and rare HU-related side effect. Discontinuation of HU usually leads to slow resolution of the ulcers over several months. The etiology of this rare side effect remains poorly understood.- - - - - - - - - - ranking = 10.477999253696keywords = chronic myelogenous leukemia, chronic myelogenous, leukemia, myelogenous leukemia, myelogenous (Clic here for more details about this article) |
5/132. sjogren's syndrome terminating as a myeloproliferative disorder.A 59 year old female was diagnosed as having sjogren's syndrome in 1963. A short time later, she developed a refractory sideroblastic anemia. In 1972, she was found to have a preleukemic state with a persistent "shift to the left" of the granulocytic series, terminating early in 1974 as acute myeloblastic leukemia. Although several cases of malignant lymphomata have been described in association with Sjorgen's syndrome, to our knowledge no other example of Sjorgen's syndrome has been described in association with a myeloproliferative disorder. Defective immune surveillance produced by the Sjorgen's syndrome may have permitted the development of the myeloproliferative syndrome. Alternatively, both disorders may have developed from a hemopoietic stem cell defect.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
6/132. A chronic myelogenous leukemia-like myeloproliferative disorder accompanied by T-cell lymphoblastic lymphoma with chromosome translocation t(8;13)(p11;q12): a Japanese case.A 40-year-old male patient presented with leukocytosis and mild splenomegaly. Bone marrow aspirate showed myeloid hyperplasia and eosinophilia resembling chronic myelogenous leukemia in the chronic phase. Cytogenetic examination of bone marrow cells revealed an unusual karyotype, t(8;13)(p11;q12), in 20/20 metaphases. Not the BCR/ABL, but the ZNF198/FGFR1 chimeric mRNA was detected by reverse transcription-polymerase chain reaction. Since 1992, 12 patients with a similar atypical myeloproliferative disorder with T-cell non-Hodgkin's lymphoma or eosinophilia, associated with a t(8;13) translocation in both bone marrow and lymph node specimens, have been described. The present case is an additional one that should be classified into this new clinicopathologic entity.- - - - - - - - - - ranking = 52.38999626848keywords = chronic myelogenous leukemia, chronic myelogenous, leukemia, myelogenous leukemia, myelogenous (Clic here for more details about this article) |
7/132. Transient myeloproliferative disease of the newborn: case report with placental, cytogenetic, and flow cytometric findings.Transient myeloproliferative disease (TMD) of the newborn is a rare hematologic abnormality associated with trisomy 21. It is frequently difficult to distinguish the disorder from true congenital leukemia (TCL). Unlike leukemia, which has a clinically aggressive course, TMD generally resolves within weeks to months. We present a case of TMD of the newborn diagnosed on the basis of peripheral blood studies and describe the pertinent pathological findings within the placenta. Flow cytometric analysis of the blasts in the peripheral blood showed phenotypic heterogeneity with features consistent with megakaryocytic differentiation. Cytogenetic studies showed trisomy 21 within the blastic cells. The placenta showed villous dysmaturity with associated chorangiosis and prominent intravascular aggregates of primitive-appearing cells with focal, early vascular wall invasion. The neonate recovered fully and shows no evidence of disease at 2 years of age.- - - - - - - - - - ranking = 2keywords = leukemia (Clic here for more details about this article) |
8/132. Immunophenotype of a transient myeloproliferative disorder in a newborn with trisomy 21.Cytologic, immunologic, and cytogenetic studies were performed on the blast cells of a newborn with down syndrome and transient myeloproliferative disease. This hematologic disorder is uncommon, and occurs primarily in infants with down syndrome. This boy presented with a high white blood cell count and a high percentage of blast cells, without anemia or thrombocytopenia. Chromosome analysis showed a constitutional trisomy 21 without any other clonal abnormality. A three-color flow cytometric analysis was performed and revealed two different CD45 dim, CD34( ), CD117( ), CD56( ) immature subpopulations: the normal immature myeloid precursor and an immature blast cell population that expressed CD41, CD42, CD61, CD36, CD13, CD1a, and CD2. We postulate that this population could be the leukemic precursor involved in the acute megakaryoblastic leukemia frequently observed in children with down syndrome.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
9/132. 5q- syndrome presenting chronic myeloproliferative disorders-like manifestation: a case report.A 28-year-old Japanese woman with suspected essential thrombocythemia (ET) had marked thrombocytosis, mild leukocytosis with normal neutrophil alkaline phosphatase activity, and no anemia. She was monitored without being given any medication. Eleven years later, complete blood counts showed no remarkable changes but some non-lobulated mononuclear megakaryocytes were found in the bone marrow. cytogenetic analysis revealed deletion of the long arm of chromosome 5 (5q-). Subsequently, hemoglobin and platelet counts decreased gradually, splenomegaly appeared and progressed, after which myelofibrosis developed. Acute leukemia developed 16 years after the first documentation of thrombocytosis. 5q- syndrome is known to be a myelodysplastic syndrome (MDS) with unique clinical features and cases with this syndrome presenting with thrombocytosis of more than 1,000 x 10(9)/L but without anemia are rare. Furthermore, it is noteworthy that in this patient transition to acute leukemia occurred following development of myelofibrosis and marked splenomegaly, which are generally observed in blastic crises resulting from chronic myeloproliferative disorders (CMPD). The patient showed features indicative of CMPD rather than of MDS in spite of presenting with 5q- chromosomal abnormality. This case supports the concept of "mixed myelodysplastic and myeloproliferative syndromes" and suggests the possibility of the appearance of CMPD-like manifestations in 5q- syndrome.- - - - - - - - - - ranking = 2keywords = leukemia (Clic here for more details about this article) |
10/132. A review of myeloproliferative disease with presentation in the head and neck region.The diagnosis of an essential thrombocytosis is demonstrated in this presentation of a well-looking 53 year old man who had a five-year history of increasing facial asymmetry as evidenced by deviation of his mandible to the right and malocclusion. The enlarged mandibular condyle was the first manifestation of his underlying myeloproliferative disorder. His management will be discussed. Neoplastic diseases of the multipotent haematopoietic stem cells result in four major diseases: chronic myelogenous leukaemia (CML); polycythaemia vera (PV); agnogenic myeloid metaplasia with myelofibrosis (AMM/MF); essential thrombocytosis (ET). CML: demonstrates increased production of neutrophils and marked splenomegaly. It is divided into a chronic phrase typified by hyperplasia of mature bone marrow elements and a blastic or acute phase which evolves into a proliferation of immature marrow elements and can develop into acute myelogenous leukaemia. PV: associated with increased production of all myeloid cells but dominated by increased red blood cells with splenomegaly. AMM/MF: allows the neoplastic stem cells to proliferate and lodge in multiple sites outside the bone marrow. splenomegaly and fibrosis of marrow spaces also occurs. ET: resulting in a markedly elevated platelet count in the absence of a recognizable stimulus. Treatment revolves around measures to maintain hydration, to relieve arthralgias, to prevent thrombotic episodes, and to prevent infections.- - - - - - - - - - ranking = 2.9379047016963keywords = chronic myelogenous, myelogenous (Clic here for more details about this article) |
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