Cases reported "Anemia"

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1/35. Management of chronic myeloid leukemia during pregnancy with leukapheresis.

    We describe the successful treatment of a pregnant patient with chronic myelogenous leukemia in chronic phase by using only leukapheresis. Following 20 leukapheresis procedures initiated during the 13th week of gestation and performed over approximately 7 weeks, the patients white blood cell count dropped from 242,000/microl to 19,300/microl. The WBC remained stable over the ensuing 17 weeks until the time of delivery. The patient gave birth by cesarean section to a healthy 2,640 g boy at 37.5 weeks of gestation. This is the second report of the successful use of leukapheresis alone for chronic myelogenous leukemia in chronic phase during the first half of pregnancy. We conclude that where leukapheresis is available, it may provide an alternative treatment to chemotherapy or alpha-interferon, especially in light of their potential teratogenic and leukemogenic side-effects.
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2/35. Myelodysplastic syndrome that progressed to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormality: a case report.

    Myelodysplastic syndrome is a closely related group of acquired bone marrow disorders characterized by ineffective and dysplastic hematopoiesis. These clonal disorders frequently progress to acute leukemia. Acute myelomonocytic leukemia with eosinophilia is characterized by an increase in abnormal eosinophils in the bone marrow, relatively good clinical course and inv (16) chromosomal abnormality. We experienced one case of refractory anemia with excess blasts which progressed to refractory anemia with excess blasts in transformation and finally to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormalities of der (1;7).
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3/35. Abnormal vaginal bleeding as first symptom of acute leukemia.

    We report a case of a 37-year-old woman, presenting with vaginal hemorrhage, together with severe anemia and thrombocytopenia (hematocrit: 10.6%; platelets: 6 G/l) caused by previously unknown acute lymphocytic leukemia.
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4/35. T-cell receptor gammadelta T-cell leukemia with the morphology of T-cell prolymphocytic leukemia and a postthymic immunophenotype.

    T-cell prolymphocytic leukemia (T-PLL) is a postthymic T-cell neoplasm with a characteristic morphology and heterogeneous immunophenotype. Most cases of T-PLL express membrane T-cell receptors (TCRs) of the alphabeta phenotype. We experienced a 30-year-old man suffering from TCRgammadelta T-cell leukemia with morphology compatible to T-PLL with a postthymic phenotype. He was admitted with skin eruption and pancytopenia. Peripheral blood and bone marrow were occupied with medium-sized lymphocytes, which had moderately condensed chromatin with a single nucleolus and sparse, nongranular basophilic cytoplasm. The immunophenotype was CD1a-, CD2-, CD3 , CD4-, CD5 , CD7 , CD8-, and terminal deoxynucleotidyl transferase negative. Hepatosplenomegaly was absent. He was diagnosed as having T-PLL and was treated with combination chemotherapy. Six months later the leukemic cell became chemoresistant. Although the patient showed transient improvement in response to pentostatin, he died 13 months after the diagnosis. To our knowledge, this is the first case of T-PLL with a TCRgammadelta phenotype.
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5/35. Abnormalities of chromosome 1 in myeloproliferative disorders.

    Three patients with myeloproliferative disorders showed a similar chromosome abnormality, accompanied by other abnormalities that were different in each case. Marrow cells from all three patients were trisomic either for the entire chromosome 1 or for its long arm. Patient 1 had a brief period of anemia and thrombocytopenia which preceded a terminal acute leukemia; Patient 2 had polycythemia vera (P.V.) that terminated in acute leukemia; and Patient 3 has P.V. The detection of an abnormal karyotype in patients 1 and 2 was an important factor in establishing the diagnosis of acute leukemia. Preliminary evidence supports the suggestion that some chromosomal changes are nonrandom in myeloproliferative diseases. Nonrandom abnormalities involving the same chromosome have been observed in several human neoplastic disorders.
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6/35. macrocephaly and epidural involvement of T-cell acute lymphoblastic leukemia.

    We describe a 2-year-old female who presented with macrocephaly and anemia. magnetic resonance imaging revealed a diffusely enhancing epidural lesion over the sagittal frontoparietal region. bone marrow examination confirmed the diagnosis of T-cell acute lymphoblastic leukemia. induction chemotherapy led to hematologic remission and neuroimaging resolution of the mass. This report suggested macrocephaly could be a rare and early manifestation of leukemia in children.
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7/35. Guillain-Barre-Strohl syndrome unraveled as paraneoplastic syndrome of B-cell acute lymphoblastic leukemia in a patient with preceding common variable immunodeficiency syndrome with Evans syndrome.

    A 26-year-old man with a history of common variable immunodeficiency and Evans syndrome (immunthrombocytopenia and immunhemolytic anemia) with sarcoid like lesions and lymph node enlargements in the previous history is described. The patient presented with symptoms resembling Guillain-Barre-Strohl as paraneoplastic syndrome just before the diagnosis of acute lymphoblastic leukemia of the L3 type. The patient was treated according to the B-ALL protocol of the German ALL study group and achieved a complete response after six cycles of chemotherapy together with a resolution of all neurologic symptoms.
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8/35. interleukin-7 (IL-7)-induced proliferation of CD8 T-chronic lymphocytic leukemia cells.

    interleukin-7 (IL-7) is a growth factor for pro-B cells, pre-B cells, and thymocytes and is known to induce the proliferation of normal human peripheral T cells. Moreover, human B and T acute leukemia cells with immature surface markers proliferate in response to IL-7. Here we describe a case of T-chronic lymphocytic leukemia, in which the leukemic cells showed a proliferative response to human recombinant IL-7 in vitro. The patient was a 74-year-old woman with anemia and thrombocytopenia, whose bone marrow was fibrosed and infiltrated with pathologic cells. Surface markers of the leukemic cells were CD2( ), CD3( ), CD5( ), CD7( ), CD8( ), and CD4(-). Both T-cell receptor beta-chain and gamma-chain genes were found to be rearranged by immunogenotypic analysis. The leukemic cells proliferated in response to IL-7 dose dependently. The dna synthesis of CLL cells was stimulated by not only IL-7 but also IL-2 and IL-4. The IL-7-induced proliferation was not inhibited by antibodies to IL-2 receptors or the anti-IL-4 antibody. These findings indicate that IL-7 may induce the proliferation of peripheral CD8 T cells, even on its pathological counterpart.
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keywords = leukemia
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9/35. Bortezomib is an efficient agent in plasma cell leukemias.

    Plasma cell leukemia (PCL) represents the most aggressive form of monoclonal gammopathy for which new treatment approaches are needed. Here we report the effect of Bortezomib on cells from 4 patients with PCL, as well as the in vivo efficacy on a patient with secondary PCL. Bortezomib reduced PCL numbers and was more efficient in cell growth inhibition than dexamethasone or doxorubicin. Treatment with Bortezomib induced procaspase-3 and poly(ADP-ribose) polymerase cleavage and decreased the amount of extracellular signal regulated kinase (Erk1/2) and phospho-Erk1/2. However, Bortezomib did not substantially affect the levels of the Erk1/2 upstream activating kinase (MEK1), p27 or p21. Finally, we had the opportunity to use Bortezomib in a heavily pretreated patient with overt secondary PCL and severe anemia and thrombocytopenia. Following Bortezomib treatment, circulating plasma cells disappeared; what is more striking, the peripheral blood counts returned to normal, becoming transfusion-independent. These data support the inclusion of Bortezomib in the therapeutic armamentarium of PCL.
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keywords = leukemia
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10/35. Acquired inv(9): what is its significance?

    Pericentric inversion of the heterochromatic region of chromosome 9 [inv(9)] is a common heteromorphism in the general population. It is presumed familial as there are no reports of de novo inv(9) chromosomes in constitutional karyotypes. We report 2 cases of acquired inv(9) chromosomes; 1 patient with acute myeloid leukemia, 46,XY,inv(9)(p11q13)[11]/46,XY[9], and a second with severe anemia, 46,XX,inv(9)(p11q13)[14]/46,XX[6]. The acquired nature of the inv(9) was confirmed by constitutional karyotyping and/or molecular analysis. The inv(9) in these patients may be a de novo inversion that cytogenetically mimics the constitutional inv(9) heteromorphism. Alternatively, it may be the result of neocentromere activation in 9q due to epigenetic events associated with the disease in these patients that results in a metacentric chromosome similarly mimicking the constitutional inv(9). One previous report of an acquired inv(9) was in a patient with essential thrombocythemia. The differences in clinical presentation may represent different underlying mechanisms generating the inv(9). The significance of an acquired inv(9) is unknown and will require reporting of additional cases.
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keywords = leukemia
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