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AK phenotypic changes observed in some hematologic diseases.

Determination of AK types in 372 patients of Hematologic Clinic, revealed in many cases changes in electrophoretic pattern of AK1 type namely the occurrence of an additional protein band. These changes were observed mostly in the acute granulocytic leukemia, lymphoblastic leukemia, and aplastic anemia. In the chronic granulocytic leukemia they were present as a rule, during the blastic crisis. Phenotypic changes were transient and the repeated examinations showed disappearance of an additional band in some patients. Etiology of the changes observed is still unclear.

Adenylate Kinase↗

[Dental care for patients with hematological diseases].

Clinical and cytological studies in 41 hematological patients aged 19-70 years showed appreciable shifts in the immune status and nonspecific reactivity virtually in all examinees. Layers of cellular epithelium of late (V-VI) and early (III-IV) stages were more often detected in smears impressions than individual cells. Cells of stages I-II were scarce and never formed layers. In patients with leukemia, signs of dissociation of epitheliocyte nucleus and cytoplasm maturation were observed ("mature" cytoplasm and "young" nuclei). These specific shifts should be borne in mind when rendering dental care to hematological patients.

Acute Disease↗

[The performance of (1, 3)-beta-D-glucan and Aspergillus galactomannan measurement for early diagnosis of invasive aspergillosis in patients with hematological diseases].

We analyzed the performance of (1, 3)-Beta-D-glucan (measurement by the alkaline-kinetic chromogenic Limulus method (FUNGITEC G test-MK, Fungitec) and the kinetic turbidimetric Limulus method [Beta-Glucan test WAKO, Wako]) and we carried out Aspergillus galactomannan antigen detection (enzyme-linked immunosorbent assay, ELISA test) for the early diagnosis of invasive aspergillosis in patients with hematological diseases at the time of febrile episodes of unknown origin that did not respond to antibacterial therapy for more than 3 days. During a one-year period (April 2002 to March 2003), a total of 69 febrile episodes in 58 patients were studied; 8 cases of invasive aspergillosis were diagnosed according to the definition of the European Organization for Research and Treatment of Cancer/Mycosis Study Group, and 61 cases were found to be non-mycotic diseases. Based on the analysis of 69 results with confirmed disease status, the overall performance of the Fungitec, the Wako, and the ELISA test were as follows: sensitivity was 0.88, 0.63, and 0.50, respectively, whereas the specificity was 0.85, 0.98, and 1.0, respectively. Moreover, there was a strong relationship between the log-transformed values of the (1, 3)-Beta-D-glucan levels measured by the two methods (r = 0.92 [95%CI, 0.89-0.94] ; p<0.001). For the statistical analysis of these serological tests a receiver operating characteristic curve (ROC) was used, as well as the resulting area under the ROC curve (ROC AUC). The ROC AUC and the cut-off values that gave the highest accuracy were as follows: 0.92, 24.9 pg/ml for the Fungitec, 0.84, 7.3 pg/ml for the Wako, and 0.89, 0.9 COI for the ELISA test, respectively. In conclusion, these results indicate that both of the two (1, 3)-Beta-D-glucan measurement approaches served equally well as surveillance tools for determining the extent of invasive aspergillosis; in addition, the log-transformed value of these tests can be used for comparison. Moreover, the ELISA test was found to have clinical utility, both as a surveillance and as a diagnostic tool when invasive aspergillosis was suspected. It should be noted that the galactomannan assay had sensitivity-related limitations; lowering the cut-off value is expected to increase the diagnostic value for use in cases of invasive aspergillosis.

Adult↗

[Laparoscopic splenectomy in hematological diseases].

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is the most common indication for elective splenectomy. The laparoscopic approach has been used over the past ten years. AIM: To report our experience with laparoscopic splenectomy. PATIENTS AND METHODS: Retrospective review of 27 patients subjected to splenectomy due to hematological diseases. Among them, 17 patients (78% female, age range 17-70 years old) were subjected to a laparoscopic splenectomy. Eligibility criteria were the presence of benign disease, an informed consent by the patient, a spleen size of less than 20 cm by ultrasound and absence of previous surgery in the upper left quadrant. The rest of the patients were subjected to an open splenectomy. RESULTS: Seventy one percent of patients subjected to laparoscopic splenectomy had an ITP. Mean operating time was 184 minutes. The mean spleen size was 11 cm and the mean weight was 186 g (70-450). No patient died or had complications. No patient required a conversion to an open surgery. Transfusions were not required. The median hospital stay was 3 days. CONCLUSIONS: Elective laparoscopic splenectomy is a safe and low risk surgical procedure.

Adolescent↗

[Clonal chromosome abnormalities in malignant hematological diseases using fluorescence in situ hybridization].

Fluorescent in situ hybridization (FISH) is a rapid, sensitive and reliable method for the identification of complete chromosomes, or segments of them, during metaphase or nuclear interphase. The present study shows the results of the analysis of 32 bone marrow aspirates from patients with malignant hematological diseases (11 AML, 7 ALL, 12 CML and 2 CLL), referred to the Medical Genetics Unit of the Faculty of Medicine, Zulia University, Maracaibo, Venezuela between 1994 and 1996. All samples were studied by conventional and molecular techniques (FISH), using probes of total chromosomes, alpha-satellites and locus specific. In patients with AML and ALL and FISH technique detected clonal chromosomal abnormalities, that were not found by the conventional cytogenetic technique. Furthermore, the PML-alpha RARA complex was identified in the promyelocytic acute leukemias. The presence of the molecular complex ABL-BCR was also demonstrated in CML. The present study demonstrates the usefulness of the FISH technique in the detection of clonal chromosomal abnormalities, which are important when considering the clinical care of patients with these pathologies.

Adolescent↗

High prevalence of endocrine dysfunction in long-term survivors after allogeneic bone marrow transplantation for hematologic diseases.

BACKGROUND: The progressively increasing number of long-term survivors after allogeneic bone marrow transplantation (allo-BMT) led researchers to focus on the early and late complications of this procedure. Endocrine dysfunction occurred mostly in patients who had undergone total body irradiation (TBI) as part of pretransplantation treatment. The extent to which chemotherapy and immune system derangement affect endocrine function in allo-BMT recipients is still unclear. METHODS: Forty consecutive patients (21 women, 19 men) with hematologic diseases surviving 12 or more months after allo-BMT from HLA-identical siblings were studied. Patients' age at transplantation ranged from 13 to 45 years and their post-BMT follow-up lasted 12-62 months. The conditioning regimen BUCY2 was employed. Graft versus host disease (GVHD) was observed in the acute form in 13 patients and in the chronic form in 26. The function of hypothalamic-pituitary-gonad, thyroid, somatotrophic, and adrenal axes was assessed. RESULTS: The most common endocrine dysfunction was ovarian insufficiency (95% of women), followed by an increase in follicle-stimulating hormone in 47% of men, indicating spermatogenesis damage. Hormone replacement therapy was contraindicated in three women because of chronic liver GVHD and it was ineffective partially in four others because of reduced intestinal or cutaneous absorption. Thyroid dysfunction occurred in 47.5% of patients and included low T3 syndrome, chronic thyroiditis, and transient subclinical hyperthyroidism and subclinical hypothyroidism. Adrenal function was abnormal in 10%, mostly related to the prolonged corticosteroid treatment. IGF-I was lower than age-reference values in 27% of all patients and in 38% of those with chronic GVHD. Thyroid, adrenal, and IGF-I impairments were more frequent in patients with chronic GVHD than in patients without this disease (P = 0.048). CONCLUSIONS: A high prevalence of endocrine dysfunction was detected in a cohort of allo-BMT recipients not treated by TBI. Although gonadal failure was likely related to intensive myeloablative treatments, thyroid, adrenal, and IGF-I impairments were late events, suggesting that immunosuppressive treatment and immune system derangement may play a role in the development of endocrine dysfunction after allografting.

Adolescent↗

[Mucous lesions associated with hematologic diseases].

Mucosal lesions are a frequent finding in patients with hematologic malignancies. Early diagnosis and institution of adequate therapeutic modalities can significantly reduce the morbidity and even mortality in these patients. Primary lesions are the consequence of infiltration of the oral tissues by malignant cells. Secondary lesions result from the condition of bone marrow suppression induced by replacement of the blood forming tissue by malignant cells. Lesions caused by the oncologic treatment, directly or indirectly, are called tertiary lesions. This article describes and illustrates the different types of lesions.

Adolescent↗