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Biomedical subjects

F Cervantes

Publications and source records attributed to F Cervantes.

At least 109 records · Page 6Linked to original sources

Neutrophilic pustulosis associated with chronic myeloid leukemia: a special form of Sweet's syndrome. Report of two cases.

Two subjects with Ph-positive chronic myeloid leukemia (CML) in whom pustular Sweet's syndrome was diagnosed are reported. The first patient was a 47-year-old woman who developed fever, painful ulcers of the oral mucosa and vagina and generalized pustulous skin lesions 2 years after the diagnosis of CML. Histologically, the skin lesions consisted of dense neutrophilic infiltrates with perifollicular disposition. The microbiologic studies were negative. The lesions showed a favorable response to corticosteroids, but fever recurred with every attempt of tapering prednisone; it finally disappeared with the addition of oral cyclophosphamide. The second patient was a 45-year-old man who developed fever and disseminated pustules with histologic features consistent with Sweet's syndrome and negative microbiologic studies at 2.5 years after diagnosis of CML. The picture showed a dramatic response to prednisone and did not recur after the drug was discontinued. In both patients, CML remained stable after resolution of Sweet's syndrome.

Cyclophosphamide↗

Polycythaemia vera following non-Hodgkin's lymphoma.

A patient with non-Hodgkin's lymphoma (NHL) who developed polycythaemia vera (PV) is reported. Diffuse large-cell NHL was diagnosed and he was subsequently treated with combination chemotherapy including high dose cyclophosphamide and procarbazine. Four and a half years after chemotherapy splenomegaly developed, coincidently with the appearance of high Hb values, RBC and platelet counts. The screening tests for PV were consistent with this diagnosis, while the search for lymphoma activity was negative. To the best of our knowledge, the present case represents the first well-documented instance of PV following NHL.

Humans↗

[Granulocyte alkaline phosphatase activity in the chronic phase and blastic crisis of chronic myeloid leukemia. Sequential study of 43 patients].

PURPOSE: To evaluate in a sequential fashion the activity of the leukocyte alkaline phosphatase (LAP) in the chronic phase and the blastic crisis of chronic myelogenous leukaemia (CML). MATERIAL AND METHODS: This study is comprised of 43 patients diagnosed of CML according to standard criteria. The initial LAP scores were compared with those recorded in the blastic crisis, using cytochemical methods. The statistical analysis was performed with Student's test and chi-square. RESULTS: The LAP activity at diagnosis was low in 40 of the 43 cases (93%), the score being 0 in 20 instances. In the blastic crisis low scores were found in 27 patients (63%), while LAP activity appeared normal or increased in 16 others (37%). When the LAP scores of the two phases of the disease were compared the differences were found statistically significant (p less than 0.0001 and p less than 0.0007, respectively). CONCLUSIONS: These results confirm that the onset of the blastic crisis of CML is often accompanied by an increase of the LAP activity, although this last persists low in the majority of the patients.

Adolescent↗

[Chronic myeloid leukemia beginning as thrombocythemia. Analysis of 5 cases].

The clinico-haematological and evolutive features of five patients with Ph'-positive chronic myelogenous leukaemia (CML) whose initial profile suggested the diagnosis of essential thrombocythaemia (ET) were analysed. The patients were women with severe thrombocytosis (greater than or equal to 1000 x 10(9)/L) and moderate leucocytosis (less than 25 x 10(9)/L), and only two of them had splenomegaly. Increased basophil count in peripheral blood was present in all cases, and peripheral myelocytosis was seen in three. The molecular analysis showed rearrangement of the BCR gene in the three patients on which it was performed. Increasing leucocyte count was seen in the three patients with extended follow-up, this reaching values in accordance with CML. Finally, the three patients who died suffered a blastic crisis. The analysis of this series, along with others reported in the literature, suggests that such cases correspond to atypical forms of CML rather than actual TE patients.

Adult↗

[T lymphoid blast crisis of Philadelphia chromosome-positive chronic myeloid leukemia: analysis of 3 cases].

BACKGROUND: To investigate the frequency of the T lymphoid phenotype in the blast crisis of chronic myelocytic leukemia (CML) with positive Philadelphia chromosome (Ph) and to evaluate the major clinical and hematologic features of the patients with this phenotype. METHODS: The presence of the TdT enzyme was assessed and specific monoclonal antibodies to the different hemopoietic lines were used to evaluate blast cells in 38 patients with blast crisis during Ph-positive CML. RESULTS: T lymphoid phenotype was found in three of the 38 patients, corresponding in all cases to immature T cells. In one patient, the blast crisis was the presenting feature of CML and in two the localization of the blast crisis was lymphadenopathic. In the two patients who could be followed up a favorable response to chemotherapy regimens including vincristine and prednisone was observed. CONCLUSIONS: Although uncommon, T lymphoid blast crisis in CML should no longer be considered exceptional. This phenotypic finding suggests that, at least in some cases, CML originates in a pluripotential stem cell common to all hemopoietic cells, including T lymphocytes.

Adult↗

[Tuberculosis in chronic myeloproliferative syndromes: its incidence and principle characteristics in a series of 562 patients].

BACKGROUND: Incidence analysis of tuberculosis in a series of 562 patients with chronic myeloproliferative syndrome (CMPS). 344 had chronic myelocytic leukemia, 91 polycythemia vera, 70 idiopathic myelofibrosis and 57 essential thrombocythemia. METHODS: The association between CMPS and tuberculosis was evaluated with the "patient-year" test, using as a control group for the incidence of tuberculosis the population of Catalonia in 1982 from data published by the Catalan government. The comparison with this group was made on the basis of observed/expected distribution. RESULTS: 9 cases of tuberculosis were found, representing a significantly higher frequency than in the general population (observed/expected relation 18.16; p less than 0.000001). In patients with idiopathic myelofibrosis the incidence was clearly higher than in the rest of CMPS. In all patients tuberculosis developed some time after the diagnosis of CMPS. In 4 patients the disease was miliary. In only one of the 5 cases where the microorganisms could be typified it was found to correspond to an atypical mycobacterium. In six patients tuberculosis had an unfavorable outcome despite therapy. CONCLUSIONS: In patients with CMPS the incidence of tuberculosis was higher than in the normal population, with a high frequency of miliary forms.

Chronic Disease↗

Immune hemolytic anemia and renal failure associated with rifampicin-dependent antibodies with anti-I specificity.

A 50-year-old woman with primary biliary cirrhosis developed immune hemolytic anemia and renal failure while receiving rifampicin for the treatment of refractory pruritus. Serological studies revealed the presence of rifampicin-dependent antibodies of the IgM class that, when tested against a wide panel of erythrocytes, had anti-I specificity. Subsequently, rifampicin was withdrawn and prednisone treatment instituted, this resulting in a rapid resolution of the hemolysis, whereas hemodialysis was required for recovery of the renal function. A role is suggested for the anti-I specificity of the antibodies in the development of renal failure associated with rifampicin therapy.

Anemia, Hemolytic↗

A study of the myeloid differentiation antigens of the peripheral blood neutrophil granulocytes in the different evolutive phases of chronic myeloid leukemia.

To ascertain whether progression from the chronic to the accelerated and blastic phases of chronic myeloid leukemia (CML) is associated with the loss of the myeloid differentiation antigens of the neutrophil granulocytes (NG), we analyzed two monoclonal antibodies (PMN 31D8 and PMN 13F6) recognizing normal peripheral blood NG membrane antigens in 49 patients in different evolutive stages of CML. Since five patients were studied twice, a total of 54 studies were carried out. Fourteen patients were evaluated at diagnosis, 12 in the controlled chronic phase within 1 year from diagnosis, 14 in the advanced chronic phase (median evolution 3.25 years), and 14 in the accelerated (five cases) or blastic (nine cases) phases. Fourteen normal subjects served as a control group. At diagnosis, a significant decrease in the positivity for both antigens was observed with respect to controls, probably due to the circulation of incompletely mature NG. In the early chronic phase the values were within the normal range, whereas a significant decrease was registered in the advanced chronic phase and especially in the accelerated/blastic phase. A negative correlation between the NG positivity for both markers and the time elapsed from the moment of obtaining the initial control of the disease was found, suggesting that a progressive loss of the myeloid antigens of the NG occurs during the evolutive course of CML. These results seem to confirm the usefulness of the NG myeloid differentiation antigen study as an evolutive parameter in CML.

Antibodies, Monoclonal↗

Acute transformation in nonleukemic chronic myeloproliferative disorders: actuarial probability and main characteristics in a series of 218 patients.

In a series of 218 subjects diagnosed as having nonleukemic chronic myeloproliferative disorders in a single institution within a 18-year period, 13 instances of acute transformation were registered. They corresponded to 8 out of 70 patients with idiopathic myelofibrosis (IM), 4 out of 91 with polycythemia vera (PV), and 1 out of 57 with essential thrombocythemia (ET). The actuarial probability of developing such a complication at 100 months from diagnosis reached 20.6% in IM, 8.7% in PV, and 4% in ET. Only 1 IM patient whose condition developed into acute leukemia had received prior cytolytic therapy, whereas, in contrast, all PV and ET patients showing this pattern had previously been treated with either 32P or alkylating agents. On the other hand, acute transformation in IM generally had an insidious presentation, contrasting with its abrupt onset in most PV and ET patients. Most acute leukemias (12 out of 13) exhibited a myeloid phenotype. The patients' median survival from diagnosis of the acute transformation was only 3 months, the development of this complication significantly shortening the patients' overall survival.

Actuarial Analysis↗

Liver dysfunction following splenectomy in idiopathic myelofibrosis: a study of 10 patients.

The hepatic abnormalities that developed after the splenectomy in 10 subjects with idiopathic myelofibrosis were analyzed. In all patients in whom a liver biopsy was performed during the splenectomy, extramedullary hematopoiesis was demonstrated, consisting of dysmorphic megakaryocytes primarily localized in the sinusoids, often accompanied by erythroid precursors. Following splenectomy, a significant increase in both the liver size and serum levels of alkaline phosphatase, bilirubin or gamma-glutamyl transpeptidase was found within 6 months, whereas no such increase was observed in the serum aspartate transaminase and alanine transaminase concentrations. In addition, 2 patients developed acute liver failure leading to death at 3 and 4 weeks from splenectomy, respectively. In contrast with these findings, no hepatic alterations were observed in 10 chronic myeloid leukemia patients who were also submitted to splenectomy.

Adult↗

[Causes of death in chronic myeloid leukemia. Analysis of 109 patients].

The cause of death was revised in 109 cases of Ph'-positive chronic myelogenous leukaemia (CML) who died in a 15-year period. The median survival of the series, which included eight patients with initial criteria of blastic crisis, was 33.7 months (ranging between 2 and 192). Eight patients (7.3%) died during the chronic phase of the disease, 7 (6.4%) in the accelerated phase, and 94 (86.3%) in the blastic crisis. The cause of death in the chronic phase was frequently unrelated to CML (a second malignancy, cirrhosis of the liver, suicide, in four cases as opposed to infection, haemorrhage of hyperuricaemic renal failure in four others), but this was not so in the deaths occurred in the accelerated phase or blastic crisis. Thus, most of the deaths appearing in the accelerated phase were due to infection or haemorrhage, whereas in the blastic crisis they were mainly due to infection (54 of the 94 cases), followed by haemorrhage and leucostasis. All in all, these three complications were responsible for 94% of the deaths occurring in that evolutive phase of CML.

Adolescent↗

[T lymphoblastic leukemia with leukemoid reaction or the extramedullary blast crisis of Philadelphia chromosome-negative chronic myeloid leukemia? Comments apropos 2 cases].

Reactive leukocytosis has been reported in patients with non-Hodgkin's lymphoma of different histologic types. On the other hand, the blastic crisis of chronic myelocytic leukemia (CML) can sometimes be localized outside the bone marrow and simulate lymphoma, particularly when the blasts are of lymphoid lineage and the blastic crisis is the presenting feature of the disease. We report two patients in whom the differential diagnosis between lymphoblastic lymphoma with reactive leukocytosis and blastic crisis of CML outside the bone marrow was raised. They were two males aged 32 and 22 years, respectively, with lymphadenopathy (and one with splenomegaly), who were initially diagnosed of T lymphoblastic lymphoma. In both cases, leukocytosis was detected with myelemia and dysgranulopoiesis in the onset in one of them and when lymphadenopathy reappeared after remission in the other one. In addition, one patient had marked eosinophilia. In the bone marrow there was marked granulopoietic hyperplasia, with a reduction of fatty cells, and the granulocyte alkaline phosphatase index was reduced. However, the cytogenetic study did not disclose the existence of Philadelphia (Ph) chromosome, and bcr/abl molecular rearrangement was also not observed in the molecular study of both cases. We discuss the basic aspects of differential diagnosis between T lymphoblastic lymphoma with leukemoid reaction and T lymphoid lymphadenopathic blastic crisis of Ph-negative, bcr/abl-negative CML.

Adult↗

[Blast crisis of chronic myeloid leukemia with positive Philadelphia chromosome: course and clinico-hematologic profile in 80 patients].

The major initial clinical, hematological and cytogenetical features of a series of 80 patients with blastic crisis (BC) in chronic myelocytic leukemia with positive Philadelphia chromosome (Ph) were evaluated, and also were their outcome and response to therapy. Mean age of patients was 45 years (SD: 14.3). Ten patients fulfilled the criteria for initial BC, and 14 had extramedullary blastic infiltration. In one third there was an acceleration phase before the development of BC. The mean leukocyte count was 69 (SD 75) X 10(9)/l. In 40% there was anemia with hemoglobin less than 90 g/l, and 37.5% had thrombopenia with less than 100 X 10(9) cells/l. In most patients, serum lactic dehydrogenase activity was increased, and in one fourth the index of granulocyte alkaline phosphatase was high. In 9 patients, blast cells had a lymphoid phenotype and in 47 (59%) cytogenetic abnormalities in addition to Ph chromosome were found, usually consisting of 8 trisomy, duplication of Ph chromosome, and the presence of a 17q isochromosome. The median survival of the series was 4.8 months. When analyzed as a time-dependent variable, the achievement of a favorable therapeutic response (found in 26% of patients) was associated with a longer survival.

Blast Crisis↗