1/31. Biphenotypic (mixed myeloid/T-cell) extramedullary myeloid cell tumor.A 32-year-old male with a 4-year history of chronic myelogenous leukemia (CML) in chronic phase for 4 years, then myeloid blast crisis for 7 months, developed diffuse bulky lymphadenopathy in association with a white blood count (WBC) of 17,100/mm3 with 70% blasts. biopsy of a cervical lymph node revealed a blastic extramedullary myeloid cell tumor, which showed a biphenotypic (mixed myeloid/T-cell) immunophenotype. Chromosomal analysis revealed karyotypic features of both myeloid and lymphoid lineages. Although extramedullary myeloid cell tumor (EMT, granulocytic sarcoma, chloroma) is well known to occur in chronic myelogenous leukemia (CML), to our knowledge this is the first description of evolution of CML into a biphenotypic EMT.- - - - - - - - - - ranking = 1keywords = white blood, white (Clic here for more details about this article) |
2/31. Acute myelogenous leukaemia and myelomonocytic blast crisis following polycythemia vera in hiv positive patients: report of cases and review of the literature.BACKGROUND: Acute myelogenous leukaemia (AML) and myeloproliferative diseases are rare in hiv-infected individuals and optimal treatment has not been defined. patients AND methods: We report on the cases of two hiv-infected men, one with AML and one with myeloid blast crisis after polycythaemia vera (PV). A comprehensive review of the available literature will be presented. RESULTS: Patient 1, a 57-year-old bisexual man known to be hiv seropositive for more than four years (CDC-category A1), presented with a pulmonary infiltrate. On admission WBC showed leukocytes 5.6 x 10(9)/l and the differential revealed 80% blasts. A diagnosis of AML FAB M0 was made. pneumonia resolved under antibiotic treatment and the patient received induction chemotherapy. However, he once more developed multiple pulmonary infiltrates and died of respiratory failure despite broad spectrum antibiotic and antimycotic therapy. autopsy revealed pulmonary aspergillosis. Patient 2 was a 63-year old hiv-positive hemophiliac (CDC A3) with a 10-year history of PV. On admission his white cell count showed leukocytes 256.6 x 10(9)/l with 82% blasts. Cytochemistry revealed myelomonocytic differentiation. The patient died of tumor lysis syndrome with renal and cardio-pulmonary failure two days later. CONCLUSIONS: This is the first report of an hiv-infected individual with AML M0. The literature describes the cases of 39 hiv patients with AML and only one further case with PV. The association of both, myeloproliferative disease and AML with hiv infection is coincidental. However, the proportion of FAB type M4/5 appears to be higher than in the general population. Despite a high risk of treatment associated mortality durable remissions can be achieved in a small proportion of hiv-infected patients with AML.- - - - - - - - - - ranking = 0.016203365221331keywords = white (Clic here for more details about this article) |
3/31. Essential thrombocythemia transformed to acute myelogenous leukemia with t(3;17)(p24; q12), del(5)(q13q34) after treatment with carboquone and hydroxyurea.In 1991, a 52-year-old woman was diagnosed as having essential thrombocythemia (ET). She was doing well with continuous medication with carboquone (CQ) and subsequently hydroxyurea (HU). However, substantial leukocytosis with leukemic blast cells, anemia and thrombocytopenia developed in 1996. Analysis of peripheral blood showed 4.4 x 10(3)/microl white blood cells with 82% of leukemic blast cells. These blasts showed negative staining with myeloperoxidase by immunostaining, but the myeloperoxidase was positive by electron microscopic analysis. cytogenetic analysis of bone marrow cells revealed a t(3;17)(p24; q12), del(5)(q13q34). On the basis of these findings, the leukemic blast cells were classified as acute myelogenous leukemia (AML:M0) in the FAB classification. The causative agent, CQ and HU in secondary leukemia from ET and chromosomal abnormality related to ET blastic crisis (BC) are discussed.- - - - - - - - - - ranking = 3.6994288599181keywords = white blood cell, white blood, blood cell, white (Clic here for more details about this article) |
4/31. Philadelphia-positive chronic myelogenous leukemia with a 5q-- abnormality in a patient following interferon-alpha therapy.In this report, we describe a rare 5q--/CML association in a patient with Ph-positive chronic myelogenous leukemia (CML) who achieved complete cytogenetic response on interferon-alpha (IFN-alpha) treatment, but who developed a new clone in the blastic crisis. The patient was treated with interferon-alpha beginning in 1996 and a serial chromosome and molecular study was performed over the clinical course of the disease. The patient remained in complete hematologic and cytogenetic remission until November 1998, when a reverse transcriptase PCR study performed on the bone marrow and peripheral blood cells was negative for chimeric BCR/ABL mRNA. The treatment was discontinued until April 1999, when the patient developed acute transformation of the disease. In June 1999, cytogenetic examination showed the development of a new clone, consisting of the deletion of the long arm of chromosome 5 in addition to the standard Ph translocation. The unusual association of a Ph with an abnormality usually observed in a secondary myeloproliferative disease raises the question of whether the new finding is treatment-induced or part of the disease process and casually related to the acute transformation.- - - - - - - - - - ranking = 0.5460190606658keywords = blood cell (Clic here for more details about this article) |
5/31. Megakaryocytic blast crisis as a first presentation of chronic myeloid leukemia.Acute megakaryocytic leukemia (AmegL) corresponds to 5.0-10.0% of all acute myeloid leukemias (AML). blast crisis as the first presentation of chronic myeloid leukemia (CML) accounts for 10.0% of all cases. OBJECTIVE: We report a case of megakaryocytic blast crisis as the first presentation of CML. CASE REPORT: A 25-yr-old-female with a 2-month history of dry cough and a large, non-tender splenomegaly was found to have a hemoglobin concentration of 10.5 g/dL, a hematocritof 33.0%, a white blood cell count (WBC) of 11.4 x 106 L with 38% small blasts, eosinophilia of 5%, basophilia of 8%, and a platelet count of 580 x 109 L. Bone marrow aspiration revealed 24% of blast cells with cytoplasmatic blebs and hyperplastic megakaryocytic lineage with dysplasia. Cytochemical stains were all negative, immunophenotyping studies showed CD41 and CD61 positivity in blast cells. Bone marrow biopsy showed grade II fibrosis. karyotype revealed 46, XX, t(9,22) (q34.1;q11.2)[20] and the reverse-transcriptase-PCR (RT-PCR) gave rise a product with a size corresponding to the 210 kDa protein (p210). No matched donor was found. After induction therapy 5.9% of blast cells persisted. The patient received Imatinib Mesylate and is doing well after a 12-month follow-up. DISCUSSION: AmegL as the first presentation of CML is a rare and often fatal event. Some characteristics point towards the diagnosis of a blast crisis instead of AmegL de novo with t(9,22).- - - - - - - - - - ranking = 3.6994288599181keywords = white blood cell, white blood, blood cell, white (Clic here for more details about this article) |
6/31. Clinical and genetic studies of ETV6/ABL1-positive chronic myeloid leukaemia in blast crisis treated with imatinib mesylate.Most chronic myeloid leukaemia (CML) patients are genetically characterized by the t(9;22)(q34;q11), generating the BCR/ABL1 fusion gene. However, a few CML patients with rearrangements of 9q34 and 12p13, leading to ETV6/ABL1 chimaeras, have also been reported. Here we describe the clinical and genetic response to imatinib mesylate treatment of an ETV6/ABL1-positive CML patient diagnosed in blast crisis (BC). A chronic phase was achieved after acute myeloid leukaemia induction therapy. Then, treatment with imatinib mesylate (600 mg/d) was initiated and the effect was assessed clinically as well as genetically, including by repeated interphase fluorescence in situ hybridization studies. Until d 71 of imatinib mesylate therapy, stable improvements in the clinical and laboratory features were noted, and the frequency of ABL1-rearranged peripheral blood cells decreased from 56% to 11%. At d 92, an additional t(12;13)(p12;q13), with the 12p breakpoint proximal to ETV6, was found. The patient relapsed into BC 126 d after the start of the imatinib mesylate treatment and succumbed to the disease shortly afterwards. No mutations in the tyrosine kinase domain of ABL1 of the ETV6/ABL1 fusion were identified in the second BC. However, whereas the ETV6/ABL1 expression was seemingly the same at diagnosis and at second BC, the expression of ETV6 was markedly lower at the second BC. This decreased expression of wild-type ETV6 may have been a contributory factor for the relapse.- - - - - - - - - - ranking = 0.5460190606658keywords = blood cell (Clic here for more details about this article) |
7/31. life threatening hypophosphataemia in a patient with philadelphia chromosome-positive chronic myelogenous leukaemia in acute blastic crisis.life-threatening hypophosphataemia developed in a 47 year old woman with blastic crisis of chronic myelogenous leukaemia. The patient's hospitalization was characterized by reciprocal relationship between her white cell count and the serum phosphorus levels. The patient did not demonstrate any of the usual causes of profound hypophosphataemia. The postulated mechanism of this patient's hypophosphataemia is uptake by the rapidly dividing leukaemic cells. To the best of our knowledge this is the first case in the English literature of hypophosphataemia associated with blast crisis of philadelphia chromosome-positive chronic myelogenous leukaemia.- - - - - - - - - - ranking = 0.016203365221331keywords = white (Clic here for more details about this article) |
8/31. Lymph node blast crisis in chronic myeloid leukemia mimicking T-immunoblastic lymphoma.BACKGROUND: Chronic myeloid leukemia arises from a somatic mutation in a pluripotent stem cell. It generally terminates with a blastic crisis (BC). One third of BC are lymphoid, and most have a pre-B phenotype. Few cases of T-lymphoid BC have been reported. Here we describe a lymph node blast crisis mimicking T-immunoblastic lymphoma. methods: Bone marrow and lymph nodes were histologically examined by standard methods and by an immunoperoxidase technique. Cytogenetic studies were also performed on lymph node and blood cells. Analysis of T-cell receptor genes and BCR rearrangements were performed on dna extracted from both frozen bone marrow and lymph-node cells. RESULTS: Lymph-node histology showed an infiltration by large lymphoid blasts, consistent with a diagnosis of immunoblastic lymphoma. Blast cells were CD2, CD7, TDT positive, and negative for myeloid and mature lymphoid antigens. The Ph1 chromosome was found in both bone marrow and lymph-node cells. BCR rearrangement was found in the dna from both bone marrow and lymph-node cells. TCR genes were not rearranged. DISCUSSION: The present study provides strong evidence that the lymph-node blast crisis of CML can assume the morphological appearance of immunoblastic lymphoma and may retain the immunological phenotype and genetic features of early T cells with BCR rearrangements.- - - - - - - - - - ranking = 0.5460190606658keywords = blood cell (Clic here for more details about this article) |
9/31. Management of life-threatening pulmonary leukostasis with single agent imatinib mesylate during CML myeloid blast crisis.Pulmonary leukostasis is a rare but serious and often fatal complication of chronic myeloid leukemia (CML) in blast crisis and acute myeloid leukemia. Treatment options are limited for these patients. Imatinib mesylate (STI-571, Gleevec, Novartis) is a potent and selective inhibitor of the BCR-abl tyrosine kinase, the molecular abnormality that causes CML. The case of a 74-year-old man with a history of CML who presented in myeloid blast crisis with pulmonary leukostasis characterized by increasing dyspnea, hypoxemia, fever, and impending respiratory failure is reported. The patient was treated with single agent imatinib mesylate (IM) with rapid decrease in his white blood cell count (WBC) and marked improvement in his respiratory status. No electrolyte abnormalities consistent with tumor lysis syndrome were observed. IM may be an effective single agent therapy for pulmonary leukostasis in patients with CML blast crisis who are at the risk for tumor lysis.- - - - - - - - - - ranking = 3.6994288599181keywords = white blood cell, white blood, blood cell, white (Clic here for more details about this article) |
10/31. Extramedullary pleural blast crisis in chronic myelogenous leukemia: cytogenetic and molecular study.Two patients with Ph1-positive chronic myelogenous leukemia with pleural blastic transformation occurring before medullary involvement are presented. The clonal origin of the pleural cells identified as unclassified blasts in 1 patient and as erythroid blasts in the other was confirmed by the presence of the t(9;22) translocation and their clonal evolution by the presence of duplicated Ph1 and additional chromosome alterations. dna obtained from the pleural blasts and peripheral blood cells of 1 patient showed an identically rearranged bcr configuration, indicating the origin of the pleural blasts from the CML clone and suggesting that this genomic event is not directly linked with the progression of disease.- - - - - - - - - - ranking = 0.5460190606658keywords = blood cell (Clic here for more details about this article) |
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