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Biomedical subjects

F Cervantes

Publications and source records attributed to F Cervantes.

At least 181 records · Page 10Linked to original sources

Sequential appearance of immunoblastic lymphoma and acute nonlymphocytic leukemia in a patient with angioimmunoblastic lymphadenopathy.

Immunoblastic lymphoma developed in a patient diagnosed as having angioimmunoblastic lymphadenopathy 5 years earlier. Chemotherapy schedules including alkylating agents were administered for 1 year, resulting in subsequent remission of the lymphoma. Thirty months after the start of chemotherapy, preleukemic changes were observed in peripheral blood and bone marrow, and acute nonlymphocytic leukaemia (M5 of the FAB classification) was diagnosed shortly afterwards. The possible role of both chemotherapy and the immunodeficiency associated to angioimmunoblastic lymphadenopathy in the pathogenesis of the leukemia is discussed.

Acute Disease↗

Prognostic discrimination among younger patients with chronic granulocytic leukemia: relevance to bone marrow transplantation.

To obtain information relevant to the question of bone marrow transplantation, we examined the prognostic significance of disease features recorded at the time of diagnosis among 625 patients, aged 5 to 45, with Philadelphia chromosome-positive, nonblastic chronic granulocytic leukemia. The actuarial death rate for this population was 5% during the first year after diagnosis, 12% during the second year, and averaged 22.5% per year during the next eight years. Multivariable regression analysis of features recorded in nearly all cases indicated that sex, spleen size, hematocrit, platelet count, and percentage of circulating blasts were significant prognostic indicators. Analyses of additional data available in 113 to 421 cases suggested that serum lactic dehydrogenase activity, percentage of blasts in marrow, nucleated RBCs in blood, and percentage of basophils plus eosinophils might also provide useful prognostic information. A Cox model, generated with five variables representing features recorded regularly (the first five listed), permitted segregation of these patients into three groups with significantly different survival patterns. The high-risk group exhibited an actuarial mortality of 30% during the first two years after diagnosis and an annual risk of 30% thereafter. In contrast, the most favorable group had a two-year actuarial mortality of 9% and an average risk thereafter of 17% per year, with a median survival of 5 1/2 years. We conclude that it should be possible to classify potential candidates for bone marrow transplantation according to risk with conventional therapy. Such information may be useful in making decisions regarding early v deferred marrow transplantation.

Adolescent↗

Myeloproliferative disease in two young siblings.

Two siblings developed a myeloproliferative disease. One of them was a 9-year-old girl with a short history of bleeding, whose initial clinical and hematologic features were concordant with idiopathic myelofibrosis. Shortly afterwards this disorder evolved to a leukemic phase which subsequently resulted in the death of the patient. Four years later, at the age of 16, her brother was diagnosed as having acute myelofibrosis, and some months afterwards he died from fulminant pneumonia coincidently with pancytopenia and presence of scarce blast cells in peripheral blood. From the pathogenetic point of view, neither exposure to bone marrow toxins nor bone marrow chromosome abnormalities could be detected. HLA-typing disclosed the same haplotype (A2, A9, B5, B21, C5) in both patients.

Acute Disease↗

Iron stores in chronic granulocytic leukaemia at presentation.

In 87 patients with Ph1 positive chronic granulocytic leukaemia (CGL), the bone marrow iron content was studied on smears obtained at diagnosis. A low sideroblast score and a decreased or absent marrow iron on semiquantitative estimation were found in 91% and 85% of cases, respectively. These findings did not correlate with blood parameters reflecting iron status such as Hb concentration, mean corpuscular volume, mean corpuscular haemoglobin, serum iron, total iron-binding capacity and transferrin saturation, which were normal in most cases. In 30 patients, initial serum ferritin was estimated, normal or slightly increased levels being as a rule found. In 24 of such patients, serum ferritin was again measured in remission following busulphan and, although values remained normal, a significant decrease was observed with respect to the initial levels (P less than 0.001). Thus, in spite of the consistent marrow pattern of iron depletion, initial iron stores appear to be normal in CGL. It seems, however, that the disease activity may partially influence the serum ferritin levels.

Adolescent↗

Acute myeloblastic leukaemia presenting with superior vena cava syndrome due to mediastinal mass.

A patient with acute leukaemia presenting with superior vena cava syndrome due to a large mediastinal mass is reported. The presence of blast cells with Auer rods in peripheral blood and bone marrow allowed the diagnosis of acute myeloblastic leukaemia. Following intensive chemotherapy the patient achieved a complete remission, with disappearance of the signs of venous obstruction and normalization of the chest roentgenogram. Although no histopathological documentation of the mediastinal tumor was available, its myeloblastic origin was assumed in view of the clinical course.

Acute Disease↗

[Immunoblastic sarcoma associated with dermatitis herpetiformis].

Association between dermatitis herpetiformis and gluten enteropathy is well established. An increased incidence of malignant disease has been reported in patients with coeliac disease and also in dermatitis herpetiformis. Linear IgA dermatitis herpetiformis has a much lower incidence of associated enteropathy compared with patients with papillary dermatitis herpetiformis, but the risk of malignancy could be the same. We report a case of linear IgA dermatitis herpetiformis in which immunoblastic sarcoma subsequently developed.

Celiac Disease↗

Prognostic discrimination in "good-risk" chronic granulocytic leukemia.

The prognostic significance of disease features recorded at the time of diagnosis was examined among 813 patients with Philadelphia chromosome-positive, nonblastic chronic granulocytic leukemia (CGL) collected from six European and American series. The survival pattern for this population was typical of "good-risk" patients, and median survival was 47 mo. There were multiple interrelationships among different disease features, which led to highly significant correlations with survival for some that had no primary prognostic significance, such as hematocrit. Multivariable regression analysis indicated that spleen size and the percentage of circulating blasts were the most important prognostic indicators. These features, and age, behaved as continuous variables with progressively unfavorable import at higher values. The platelet count did not influence survival significantly at values below 700 X 10(9)/liter but was increasingly unfavorable above this level. Basophils plus eosinophils over 15%, more than 5% marrow blasts, and karyotypic abnormalities in addition to the Ph1 were also significant unfavorable signs. The Cox model, generated with four variables representing percent blasts, spleen size, platelet count, and age, provided a useful representation of risk status in this population, with good fit between predicted and observed survival over more than a twofold survival range. A hazard function derived from half of the patient population successfully segregated the remainder into three groups with significantly different survival patterns. We conclude that it should be possible to identify a lower risk group of patients with a 2-yr survival of 90%, subsequent risk averaging somewhat less than 20%/yr and median survival of 5 yr, an intermediate group, and a high-risk group with a 2-yr survival of 65%, followed by a death rate of about 35%/yr and median survival of 2.5 yr.

Adolescent↗