Cases reported "Preleukemia"

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1/15. Myelomonocytic leukaemia with a preleukaemic syndrome and Ph1 chromosome in monozygotic twins.

    A case of monozygotic twins with a preleukaemic phase of 3 1/2 years is reported. The final haematological picture was that of myelomonocytic leukaemia. The karyotype investigated in one twin during the final period of the disease showed a Ph1 chromosome.
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2/15. Sequential development of distinct clonal chromosome abnormalities in a patient with preleukaemia.

    Preleukaemia has been identified as a clonal haemopathy in which progression to acute leukaemia involves conservation of the preleukaemic karyotype in the blast cells or the development of new abnormalities superimposed on the original clone. In this report, a case of childhood preleukaemia is presented in which two cytogenetically distinct clones developed over 2 years in a dysplastic marrow that was initially karyotypically normal. One clone with 47 chromosomes (47,XY, 21), disappeared without therapy. Predominance of the cytogenetically abnormal clone, 45,XY,-12,-17,t(12;17)(p11;q11) was associated with the development of acute myelogenous leukaemia and myelofibrosis. The development of independent clonal abnormalities in the unstable preleukaemic marrow may occur more commonly than has been previously recognized. Implications of the progression of the karyotypic abnormalities are discussed.
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3/15. Serial cell culture and cytogenetic studies of marrow cells from a patient with monosomy 7 syndrome.

    Serial bone marrow cell culture studies, with cytogenetic characterization, were performed in an 18-month-old boy with monosomy 7 syndrome--a condition that usually converts to nonlymphoblastic leukemia. Over a 12-month course, his marrow demonstrated: decreased colony formation and increased cluster formation in vitro with disease progression (consistent with preleukemia); persistence of only the monosomy 7 karyotype in direct marrow studies; and in contrast, proliferation of both 46,XY and monosomy 7 cells in in vitro marrow cultures. The findings indicate that certain dormant marrow cell populations may be expressed under in vitro culture conditions and suggest the need for determining factors which would allow their expression in vivo.
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4/15. Translocation 1;7 in preleukemic states.

    A translocation t(1;7) interpreted as t(1;7)(p11;p11) was first reported by Scheres et al. in eight patients with various hematologic disorders. The karyotype of the abnormal cells was trisomic for 1q and monosomic for 7q. Those investigators reported having found four other cases in the literature. We report herein studies of two patients with the same t(1;7).
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5/15. Restoration of normal hematopoiesis by bone marrow ablation and allogeneic marrow transplantation in a case of Hodgkin's disease therapy-related preleukemia.

    Therapy-related leukemias are generally preceded by a preleukemic phase of several months duration, characterized by pancytopenia, abnormal bone marrow findings, and nonrandom chromosomal abnormalities in almost all cases. No specific therapeutic guidelines are recommended in this preleukemic phase or any other type of preleukemia; aggressive combination chemotherapy is usually withheld until the full expression of leukemia. A 22-yr-old man with therapy-related preleukemia following treatment of Hodgkin's disease received as primary treatment ablative chemotherapy followed by marrow transplantation from his histocompatible sister. At day 316, the patient is still in complete bone marrow recovery with a normal donor karyotype. In the light of the very poor results obtained with conventional chemotherapy regimens once the leukemic phase is established, we suggest that bone marrow transplantation, if undertaken before leukemic conversion, may be the treatment of choice in young adults with therapy-related preleukemia.
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6/15. Treatment of therapy-induced preleukemic syndrome.

    Two young adult patients with therapy-induced preleukemic syndrome and Hodgkin's disease as primary malignancy were treated with aggressive antileukemic regimens before the establishment of leukemic conversion. Pretreatment clinical staging procedures did not reveal recurrence of Hodgkin's disease. One of the regimens consisted of an HLA-identical allogeneic bone marrow transplant and the other of high dose cytosine arabinoside. Both therapeutic approaches have proved successful in restoring normal hematopoiesis with reversal to normal karyotypes and unmaintained remissions 556 and 192 days post-treatment. The rationale for such a therapeutic approach in the preleukemic stage of therapy-induced leukemogenesis is discussed.
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7/15. A complicated but nonrandom karyotype in preleukemia.

    A patient with preleukemia who possessed a complicated hypodiploid karyotype in 100% of bone marrow cells is described. The clinical, hematologic, and cytogenetic features showed a marked similarity to a patient with preleukemia described by Watt et al. [1]. Both patients terminated their disease in acute nonlymphocytic leukemia. Another patient with similar cytogenetic features, but who presented in the acute leukemic phase of polycythemia rubra vera, also is described. All three patients possessed a translocation involving chromosome #11 at band p15. This, together with many numerical and structural abnormalities, particularly those involving chromosomes #5, #7, and #17, may prove useful in defining a variety of preleukemia with a poor prognosis.
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8/15. Hematologic and cytogenetic study of two cases of acute leukemia associated with breast cancer.

    The authors present two cases of patients with breast cancer with lymph node extension and who both had surgery. As a pancytopenia with hypercellular bone marrow was discovered at the same time in the first patient, she received no complementary treatment; 4 months later, she presented with an acute lymphocytic leukemia (ALL) for which a remission was easily induced, but she died of a pulmonary infection. The second patient received local radiotherapy (50 grays) and adjuvant chemotherapy (Alkeran for 26 months). Forty-seven months after the diagnosis of breast cancer and 16 months after the end of the treatment, an acute nonlymphoblastic leukemia (ANLL; M6) was diagnosed after 8 months of a preleukemic state. Treatment did not produce any results and death occurred on the 17th day. Cytogenetic studies on the bone marrow cells of both patients were performed. In the first patient in the ALL phase normal cells coexisted with a 47 chromosome clone, the extra chromosome being a D ( 13?). In the second patient, several karyotype abnormalities were already present in the preleukemic state and also during the acute leukemic phase. No normal mitoses were found; hypodiploidy was present as well as major abnormalities such as markers, rings, and, among others, the systematic loss of a #5 and a #7. The first patient seems to have presented with a de novo ALL, associated with the malignant tumor; whereas, the second patient showed all the characteristics of an induced ANLL. The clinical, hematologic, and cytogenetic characteristics of these two patients are analyzed and compared to those of other cases in the literature.
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9/15. Dermal necrosis and chromosome Iq abnormality in a man with a familial myeloproliferative disorder.

    A 33 year old man, with pre-existing psoriasis and a family history of multiple occurrence of acute myeloid leukemia and other myeloproliferative disorders, developed steroid-responsive ulcerating skin lesions, pancytopenia, marrow hypoplasia, hyperglobulinemia and polyarthritis. An abnormal karyotype (47,XY i(1q] was detected in the bone marrow, and comparison with a case previously reported by Lee et al. Suggested that this abnormality may be significant. His sister, who developed chronic leucocytoclastic vasculitis, had pre-existing psoriasis, variable mild leucopenia and marrow dysplasia. review of available records of other affected family members documented the occurrence of steroid responsive pancytopenia, knee swelling and terminal lipoid pneumonia in a first cousin. Four other relatives died with acute myeloblastic leukemia and another died with myelofibrosis. Two healthy first degree relatives were subjected to laboratory investigations with essentially negative findings.
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10/15. Translocation 1;7 in dyshematopoiesis: possibly induced with a nonrandom geographic distribution.

    Eight patients with various hematologic disorders had an identical chromosomal aberration in their bone marrow or unstimulated peripheral blood, a translocation t(1;7) interpreted as t(1;7)(p11;p11). The translocation chromosome replaced one normal chromosome #7; therefore, the karyotype of the abnormal cells was trisomic for 1q and monosomic for 7q. Including four cases from the literature, a total of 12 patients (4 women, 8 men) with this translocation are known at the moment. The translocation does not seem to be associated with a specific disorder, but almost all patients had a preleukemic syndrome during some stage of their disease. It is very remarkable that 11 of the 12 patients lived in the netherlands, and 7 patients had a history of iatrogenic exposure to alkylating agents or irradiation; one patient was a radiation worker and another one had a history of toxic exposure to chloramphenicol. It is suggested, therefore, that the t(1;7) is a possibly induced chromosomal aberration with a clearly nonrandom geographic distribution.
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