1/9. Acute basophilic leukemia: case report.The term "basophilic leukemia" has been in use for 75 years. However, consistent diagnostic criteria are lacking. This is due to the rarity of the disease and to the routine unavailability of special tests that are often required to confirm a diagnosis. We report an unusual case of acute basophilic leukemia in a child who was referred to our Center, arriving with partially treated acute lymphoblastic leukemia. Basophilic differentiation on light microscopy was evident from the coarse basophilic granules in blasts, a progressive maturation of blasts toward basophils, and toluidine positivity on cytochemistry. Blasts showed a myeloid immunophenotype (CD13 , CD33 , CD117 ) with a characteristic dual positivity for CD34 and CD25, highly suggestive of basophilic nature of the blasts. Conventional cytogenetic studies revealed translocation t(8;21)(q22;q22). A diagnosis of acute basophilic leukemia with t(8;21) was made. review of pre-therapy slides showed features consistent with AML-M2 with basophilia. There were no basophilic blasts. With these features, a diagnosis of acute basophilic leukemia secondary to AML-M2 was made. In our patient, basophilic leukemia appears to have evolved from selective clonal proliferation of "basophil-committed blasts" during the course of the disease in a case of AML-M2 with basophilia.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/9. Bilateral granulocytic sarcoma (chloroma) of the breast in CML in blast crisis: a case report.A 35 year old lady was diagnosed as having chronic myeloid leukemia in May 1999 and thereafter started on chemotherapy. Three years later the patient presented with bilateral breast masses. FNAC from both the breast lesions showed leukemic infiltration (granulocytic sarcoma). The peripheral blood picture showed blastic transformation. breast is an uncommon site for development of granulocytic sarcoma. We present this case because of its unusual location and bilateral nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/9. Extramedullary blast crisis in a patient with philadelphia chromosome-positive chronic myelogenous leukemia in complete cytogenetic remission.Treatment of Philadelphia (Ph) chromosome-positive chronic myelogenous leukemia (CML) with recombinant interferon-alpha (IFN-A) results in complete disappearance of the Ph chromosome in about 10% to 15% of patients in early chronic phase. This group has a long survival and very low incidence of blast crisis. The first known case is reported of extramedullary blastic transformation in a patient with medullary complete cytogenetic response (0% Ph-positive metaphases) to IFN-A. Four episodes of extramedullary blast crisis have occurred in this patient. The first three episodes were lymphoid by morphology and cytochemical stains. Molecular analysis confirmed breakpoint cluster region rearrangement. The most recent transformation was myeloid in nature and involved bone and pulmonary parenchyma. The patient is currently undergoing a second autologous transplantation with stored bone marrow that is Ph negative. The patient has survived more than 18 months since the first episode of blast crisis, and the bone marrow is normal.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/9. Extramedullary blast crisis in chronic myelogenous leukemia. Demonstration of T-cell lineage and philadelphia chromosome in a paraspinal tumor.A case of chronic myelogenous leukemia (CML) with penetrance of the Ph1 molecular alteration into the T-cell lineage of a paraspinal tumor is reported. The T-cell nature of the paraspinal tumor was documented by immunochemical study and genotyping. The philadelphia chromosome was also detected by genotyping in the same tumor cell population. To the authors' knowledge, this is the first case of extramedullary T-cell blast crisis in CML demonstrated in an extranodal tumor. The clinical significance of detecting extramedullary blast crisis, and the theories for the scarcity of T-cell transformation in CML are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/9. Refractory anemia terminating in acute megakaryoblastic leukemia (M7).A 72-year-old man with refractory anemia (RA) developed overt megakaryoblastic leukemia after the course of RA with excess of blasts. The blasts were positive for platelet peroxidase activity and had platelet glycoproteins (GPs) such as GPIIb/IIIa and GPIIIa. The bone marrow biopsy at terminal stage disclosed marked fibrosis. The nature of the megakaryoblasts was investigated. The blasts did not differentiate morphologically into mature megakaryocytes with TPA addition. in vitro colony assay showed the failure of colony-forming unit, megakaryocyte growth in peripheral blood. The pathogenesis of myelofibrosis in our patient is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/9. Chronic myeloid leukemia: manifesting as spontaneous splenic rupture and terminating in megakaryoblastic transformation.We report a case of chronic myeloid leukemia with spontaneous splenic rupture as the initial presenting feature; there was a successful surgical outcome, but this case terminated in megakaryoblastic transformation. Results are reported based on morphological, immunological, cytochemical, ultrastructural, immunocytochemical studies, and in vitro liquid culture studies. The megakaryocytic nature of the blast cells was identified through the demonstration of platelet peroxidase activity by ultrastructural cytochemistry and the presence of platelet and megakaryocyte-specific antigen using monoclonal antibody, as well as the anti-factor viii antibody by immunocytochemical technique.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/9. Progression of essential thrombocythemia to blastic crisis via idiopathic myelofibrosis.We report a 61-year-old man with essential thrombocythemia (ET) whose clinical course was followed for 12 years. The ET evolved into true idiopathic myelofibrosis (IM) 6 years after the initial diagnosis and progressed to myeloid blastic transformation 6 years later. The cytogenetic analysis showed a normal karyotype during the ET phase but subsequent analysis revealed an abnormal karyotype during the IM phase which evolved clonally at blastic crisis with constant involvement of chromosome 13q and chromosome 7. The close monitoring of essential events, using clinical, morphologic, immunologic and cytogenetic parameters, allowed us to carefully identify the transition from one chronic myeloproliferative disease (MPD) to another. This is only the second case reported showing a clinical evolution of this nature. The clinical and biological aspects of the disease are briefly discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/9. Ph-positive chronic myeloid leukemia mimicking essential thrombocythemia and terminating into megakaryoblastic blast crisis: report of two cases with molecular studies.Two patients with chronic myeloid leukemia (CML) presenting with the hematologic features of essential thrombocythemia (ET) are reported. At diagnosis they showed extremely high platelet counts (4985 and 2800 x 10(9)/l, respectively) and moderate leukocytosis (21 and 17 x 10(9)/l, respectively). In both cases, in addition to the philadelphia chromosome (Ph), a rearrangement within the major breakpoint cluster region on chromosome 22 was demonstrated, with the breakpoint in the 3' extreme. In patient 1 the disease initially responded to radioactive phosphorus and hydroxyurea, but during the evolutive course a progressive increase in the white blood cell counts was noted, reaching values typical of the chronic phase of CML, and the patient eventually died from blast crisis 45 months after diagnosis. In patient 2, although good control of the platelet counts was achieved with hydroxyurea, the disease also evolved into a blast crisis four months after diagnosis. In both cases monoclonal antibodies and electron microscopy studies demonstrated the megakaryocytic nature of the blast cells. The above features are not consistent with the present and similar cases being Ph-positive ET. Instead, they should be regarded as a special form of CML characterized by a marked protagonism of the megakaryocytic component.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/9. Acute lymphoblastic leukemia in a case of essential thrombocythemia.Essential thrombocythemia is a myeloproliferative disorder that infrequently evolves into acute leukemia. Leukemic transformation is frequently preceded by therapy with alkylating agents or radioactive phosphorus (32P), and is virtually always myeloid in nature. In this report, the authors describe a case of acute lymphoblastic leukemia arising in a patient with long-standing essential thrombocythemia.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |