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At least 19 recordsLinked to original sources
PubMed1975

Patho-anatomical features of so-called Ph1- chronic myeloid leukemia.

Chronic myeloid leukemia without the Philadelphia chromosome (Ph1-CML) is described and distinguished from chronic myeloid leukemia with the Philadelphia chromosome (Ph1+CML) on the basis of clinical and autopsy findings of four cases. Ph1-CML showed clinical, hematological, and patho-anatomical features which could be regarded as typical. Patho-anatomically Ph1-CML differed from Ph1+CML in the variable maturation of the leukemic proliferation in the bone marrow and extramedullary infiltrates. Up to the terminal phase Ph1-CML can be of an extremely mature cell type. However, it can also show myeloblastic transformation after an initially mature cell stage. Ph1-CML infiltrates are found in tissues and organs which Ph1+CML usually does not infiltrate or only to a low degree until a blastic crisis. On the basis of its course and clinical and patho-anatomical features Ph1-CML looks like an atypical chronic myeloid leukemia. However, it is better called an acute myeloid leukemia of the mature cell type.

Aged↗
PubMed2026

Cytogenetic Diversity of Variant Philadelphia Translocations in Chronic Myeloid Leukemia.

INTRODUCTION: Chronic myeloid leukemia (CML) is a disease characterized by Philadelphia (Ph) translocations. These translocations can be classical or variant. The structural features and diagnostic implications of variant Philadelphia translocations remain incompletely defined, and they display considerable cytogenetic heterogeneity. METHODS: In this retrospective study, variant Ph translocations identified by conventional cytogenetic analysis and fluorescence in situ hybridization (FISH) were systematically classified among 639 patients diagnosed with CML. A total of 35 patients with variant Ph translocations were included in the analysis. Molecular follow-up data, when available, were assessed using RT-qPCR analyses in a subset of patients. RESULTS: Chromosome analysis revealed 2 simple and 33 complex variant Ph translocations. FISH analysis, performed in 20 patients, identified deletions involving BCR, ABL1, or both in a limited number of cases. Additional chromosomal abnormalities and secondary translocations accompanied variant Ph translocations in four patients. The partner chromosomes involved in variant Ph translocations showed marked diversity, involving multiple chromosomal loci. CONCLUSION: Variant Philadelphia chromosome translocations in CML exhibit substantial cytogenetic diversity, reflecting the complexity of their underlying genomic architecture. The rarity and heterogeneity of these rearrangements complicate their classification and interpretation in routine diagnostic practice. Descriptive reporting of variant Ph translocations may contribute to a better understanding of their diagnostic complexity and support more accurate cytogenetic interpretation in CML.

Humans↗
PubMed1979

[Spleen irradiation and splenectomy for treatment of hypersplenism in chronic myeloid leukemia and chronic lymphatic leukemia (author's transl)].

Hypersplenism is characterized by cytopenia, splenomegaly (possibly hyperplastic bone-marrow), and lienal hypersequestration. It is necessary, in view of the rather important surgical risks of splenectomy, to clarify if the hypersplenism may be influenced by splenic irradiation in case of chronic myeloid leukemia (CML) or chronic lymphatic leukemia (CLL). References in literature are rare and do not present a reliable differentiation of the syndrome, according to its present definition. Of our patients, three cases with hypersplenism verified by radioiron studies are reported: in one patient with chronic myeloid leukemia, irradiation of the spleen had no effect, whereas pancytopenia was completely repaired by means of splenectomy. The same results were seen in a patient with chronic lymphatic leukemia. In the last patient, however, who had chronic lymphatic leukemia, fractionated irradiation of the spleen led to a distinct improvement of anemia and thrombopenia, but the recovery lasted only six months. This effect is due to transient normalization of the lienal hypersequestration.

Aged↗
PubMed1978

The relevance of extramedullary hemopoiesis to the staging of chronic myeloid leukemia.

In chronic myeloid leukemia (CML) the spleen and the liver can lodge, trap and release a high proportion of leukemic cells, and are active sites of (leukemic) hemopoiesis throughout the disease. Evidence is provided that spleen and liver volume have a prognostic value, and that marrow, spleen, and liver differ as to cell composition, cell kinetic patterns, functional cell characters, and cell karyotype. In the majority of the patients, extramedullary hemopoiesis seems to be more abnormal than marrow hemopoiesis, supporting the hypothesis that spleen and/or liver can play an important role in the evolution of CML towards the blastic metamorphosis. However, present information on extramedullary hemopoiesis does not allow to identify specific patterns and to establish its relevance to the course of the disease.

Bone Marrow↗
PubMed1975

Chromosome band analysis in 19 cases of chronic myeloid leukemia: 9 chronic, 10 blastic, two with Ph1 (22q-) translocation on 17 short arm.

The results of Giemsa band analyses of the marrow cells of 19 cases of CML in the chronic or blast phase are reported. All but one of the 14 Ph1 positive cases showed the 9q+pattern, the exceptional one being 16p+. All 10 blast cases showed additional rearrangements, which are described. In two of the cases, one blastic, one chronic, which were -22, Ph1 negative, the 22q- member was identified as a translocation on the short arm of one 17 which was dicentric.

Chromosome Aberrations↗
PubMed1975

Meningeal leukemia after blastic transformation of chronic myeloid leukemia.

In two patients with blastic transformation of chronic myeloid leukemia complete remission was induced, but meningeal leukemia subsequently developed. The recognition of blast cell crisis as the presenting feature of chronic myeloid leukemia is discussed, together with treatment of this condition and its rare complication, meningeal leukemia. The development of meningeal leukemia in this disease is almost certainly due to the increased survival that has been produced by advances in therapy for blastic transformation.

Adult↗
PubMed1978

Splenectomy in chronic myeloid leukemia.

We review splenectomy in chronic myeloid leukemia, emphasizing recent studies that have evaluated early splenectomy during the chronic phase of the disease. Despite current interest in splenectomy in chronic myeloid leukemia, uncontrolled clinical trials to date suggest that the operation during the early phase neither delays the onset of blastic transformation nor prolongs survival. Immediate operative mortality of splenectomy during the chronic phase is low, generally less than 1%. The morbidity of the procedure, however, remains formidable due to infectious and thromboembolic complications. Splenectomy during chronic-phase chronic myeloid leukemia permits easier control of the disease in patients who are thrombocytopenic due to busulfan toxicity or sensitivity. Splenectomy during the chronic phase may also palliate those patients who suffer from acute splenic events or massive splenomegaly. Splenectomy in blast-phase disease should be considered a heroic measure providing little benefit to most patients.

Aged↗
PubMed1975

Consecutive cytochemical staining for the analysis of the blastic population in the acute phase of chronic myeloid leukemia.

Blastic crisis in chronic myeloid leukemia is characterized by several cytological alterations which may represent some abortive attempts to differentiate along various cell lines as a consequence of a maturation defect of the myelopoietic cells. These changes of the hematological picture are associated with alterations of the karyotype and with cytochemical abnormalities of the blast cells, possibly related to their metabolic anomalies. In this regard 14 patients with blastic crisis were investigated to achieve an evaluation of the composition of the cell population during the acute phase. A sequence of three cytochemical reactions applied consecutively on the same slide (alpha-naphthyl-acetate esterase + AS D-chloro-acetate esterase + PAS) proved to be useful for the detection of differently oriented blast cells. During the acute phase of chronic myeloid leukemia only about one half of the blast cells were expressing granuloblastic differentiation. The data may be important for some clinical and prognostic factors, since the heterogeneity of the blastic population may be associated with a particular resistance to therapy.

Adolescent↗
PubMed1976

Specific xenogenous antiserum to cells of chronic myeloid leukemia (CML) in blastic crisis.

The antiserum was obtained from horses immunized with cells from patients with blastic crisis of CML and completely absorbed with normal white blood cells (WBC). The absorbed antiserum remained cytotoxic to blast cells from nearly half of the patients in blastic crisis and did not react with WBC from patients with Acute Myeloid Leukemia (AML), Acute Lymphoid Leukemia (ALL), Chronic Lymphoid Leukemia (CLL) and Chronic Myeloid Leukemia (CML) in its chronic phase as well as with cells of human normal bone marrow or fetal liver.

Antigens, Neoplasm↗
PubMed1978

Chromosome abnormalities and clinical and morphologic manifestations of chronic myeloid leukemia.

Forty cases of chronic myeloid leukemia (CML) were studied and subgroups of cases with similar chromosomal abnormalities in terminal stage were defined. Certain correlations were observed between the type of chromosomal changes, and clinical and morphologic manifestations of the disease: (1) It seems that, in cases without any karyotype changes other than translocation (9;22), the terminal stage is longer and milder than in cases with additional chromosomal abnormalities; (2) cases with marker i(17q) are clinically and morphologically rather uniform and are characterized by distinct signs of myeloid differentiation of blast cells, absent in other cases; (3) in cases with various atypical chromosomal abnormalities, the course of the terminal stage is the most rapid and grave. The blast cells differ from myeloblasts and resemble lymphoid elements.

Adolescent↗
PubMed1975

Trisomy 11 in acute phase of chronic myeloid leukemia.

A female patient with chronic myeloid leukemia in acute phase was found to have trisomy 11 in her bone marrow cells. The contemporary existence of different clones suggests the following clonal evolution: 46,XX leads to 46,XX,Ph' leads to 47,XX,Ph', +11 leads to 48,XX,Ph', +11, +Ph'. It is suggested, that the cytogenetic events leading to trisomy C in advanced cases of chronic myeloid leukemia are of a different character than those which often results in trisomy C in other myeloid disorders.

Acute Disease↗
PubMed1978

Phase II clinical trial with vindesine for remission induction in acute leukemia, blastic crisis of chronic myeloid leukemia, lymphosarcoma, and hodgkin's disease: absence of cross-resistance with vincristine.

Vindesine, an analog of vinblastine and vincristine, has been submitted to a phase II trial, the results of which are judged in terms of remission induction. A high proportion of remissions were obtained in acute lymphoid leukemia and blastic crisis of chronic myeloid leukemia, and a few responses have been registered in lymphosarcoma and Hodgkin's disease. A continuous 48-hour iv infusion may induce a remission where an iv push of the same dose has failed. The most remarkable characteristic of vindesine is the absence of cross-resistance with vincristine as documented in acute lymphoid leukemia.

Acute Disease↗
PubMed1976

[Toxoplasmosis and chronic myeloid leukemia].

A patient suffering from chronic myeloid leukemia and used as a donor of leucocytes was found to have toxoplasmosis associated with a high level of IgG without an elevation of IgM which was maintained for several months. This case raises several points for discussion. Toxoplasmosis is a frequent complication of malignant hematological disease, it is often severe with neurological complications in more than half the patients affected. It is often difficult to diagnose. The parasite is arduous to isolate and serology is problematic to interpretate without reference to previous samples. This suggests it would be wise to measure the titre of anti-toxoplasma antibodies as part of the initial investigations of every malignant hemopathy and to repeat the measurements during the evolution of the disease. Any patient showing a high serological titre, even though it is stable and without IgM should be given systematically a specific treatment for toxoplasmosis, for as in the patient presented in this paper, such a serological picture is compatible with an increasing infection. The frequence of high serological titres seems to be greater among patients suffering from chronic myeloid leukemia than among another people. Then, transfusion of leucocytes of such patients may be the source of parasite transmission; this paper relates one case of post-transfusional toxoplasmosis.

Antibodies↗
PubMed1978

Osteolysis in chronic myeloid leukemia.

Four patients affected by chronic myeloid leukemia who developed osteolytic lesions in the course of the disease are described. According to the literature, the appearance of these alterations seems to signify an unfavorable prognosis, since they occur slightly before or even at the same time as the blastic transformation of the disease. However, in one case bone biopsy showed a metastasis of a solid tumor, emphasizing the importance of this procedure in order to give a more precise clinical evaluation, both diagnostic and therapeutic.

Biopsy↗
PubMed1976

Discordant patterns of chromosome changes and myeloblast proliferation during the terminal phase of chronic myeloid leukemia.

A patient with Ph1 positive chronic myeloid leukemia (CML) developed blastic transformation which by morphologic criteria appeared to be localized to the lymphatic system. Chromosome analysis at this time, however, revealed new chromosomal abnormalities in addition to the existing Ph1 in all tissues studied (lymph node, blood, and bone marrow) consisting primarily of extra chromosome numbers 19 and 9 and a second Ph1. Therapy resulted in clinical remission with significant decrease in the aneuploid cell lines. However, these reappeared with recurrence of the blast crisis. Colony formation in semisolid culture of blood and marrow cells at the time of initial blast crisis yielded growth patterns characteristic of CML. On recurrence of the blast crisis after therapy, growth patterns were characteristic of CML in blast crisis or acute myeloblastic leukemia even though blood and marrow still showed relatively low levels of myeloblasts and promyelocytes. Possible explanations are discussed for the disparity in distribution between morphologic and chromosomal abnormalities in this patient.

Adult↗
PubMed1978

Effect of early splenectomy and cyclic acute leukemia-like chemotherapy on the course of chronic myeloid leukemia.

This report provides information on the progress of two trials by the Italian Cooperative Study on Chronic Myeloid Leukemia (CML). Both trials were designed in order to test the effect of early splenectomy and of cyclic administration of either arabinosyl cytosine and vincristine plus prednisone, or arabinosyl cytosine plus thioguanine plus daunomycin, on the course of CML and on the survival. A preliminary analysis, performed on September, 1977, suggests that neither splenectomy nor the chemotherapy scheduled have resulted in a significant decrease of the rate of blastic transformation of CML, during the first 2 to 3 years from diagnosis.

Adult↗
PubMed1977

Chronic myeloid leukemia: physical and functional similarities to acute leukemia.

Blast crisis, closely resembling acute leukemia, is the usual terminal event in chronic myeloid leukemia. Using physical ("fingerprint") and cultural (colony-forming) methods, we have demonstrated distinctive patterns in the stable phase of chronic myeloid leukemia and in blast crisis. An unusual fingerprint alteration preceding the onset of the terminal phase is noted, and cell culture perturbation is evident at different stages of the disease. Our findings indicate that the application of these methods to the study of hemopoietic disorders is valid, and suggest that the use of such techniques may allow a better understanding of the complex cellular events occurring in the course of chronic myeloid leukemia.

Acute Disease↗