Follow-up of anti-Aspergillus IgG and IgA antibodies in bone marrow transplanted patients with invasive aspergillosis.

A total of 89 patients at risk for, or with invasive aspergillosis (IA) were recruited from bone marrow transplantation (BMT) units in two Lisbon hospitals, and followed for 2(1/2) years to monitor their immune response. Of these patients, six developed probable IA, from which five died. The presence of serum IgG or IgA antibodies against seven Aspergillus recombinant antigens was assessed in patients with IA, using an enzyme-linked immunosorbent assay (ELISA). In parallel, the serum levels of galactomannan (GM) were also monitored, using the Platelia Aspergillus kit (Sanofi Pasteur, Marnes-la-Coquette, France). Superoxide dismutase (Sod) and 94 kDa were the most immunogenic antigens for IgA, while the IgG pattern of recognition changed from patient to patient. From our results we conclude that although follow-up of antibodies against these antigens should not be used as a diagnostic method, patients with IA do produce an immune response that may influence disease outcome.

[Aplastic anemia. Alternative treatment by bone marrow transplantation]. / Anemia aplástica. Tratamento alternativo à transplantação medular.

We report a case of a 25-year-old woman, with the diagnosis of severe aplastic anemia without a histocompatible sibling donor for bone marrow transplantation. The patient has been treated successfully with a combination of two immunosuppressive agents, cyclosporine and antilymphocyte globulin, after not responding to primary therapy with corticosteroids and growth factors. She showed a complete response to treatment, with transfusional independence, after a follow-up of 14 months. The pathophysiology of aplastic anemia, the mechanism of action and secondary effects of these treatments are discussed.

[Treatment of acute promyelocytic leukemia with trans-retinoic acid. Experience of the Santa Maria Hospital, Medical School of Lisbon]. / Terapêutica da Leucemia promielocítica aguda com ácido Trans-Retinóico. Experiência do Hospital de Santa Maria, Faculdade de Medicina de Lisboa.

Acute promyelocytic leukemia (APL) is a rare subtype of acute myelogenous leukemia that is usually associated with a fatal hemorrhagic diathesis. All trans-retinoic acid (ATRA) is an active metabolite of vitamin A that differentiates the malignant cell clone, corrects the coagulopathy, and induces complete remission in the vast majority of patients with APL. Between June 1992 and September 1993, 8 patients with APL (4 previously untreated, 3 in first relapse and 1 in second relapse) received ATRA. Complete remission was achieved in 7 patients; in 5 with ATRA alone and in 2 with ATRA followed by cytotoxic chemotherapy due to the development of asymptomatic hyperleukocytosis. The earliest signs of response were the correction of the coagulopathy and an increase in the white blood cell count. Sequential morphological and immunophenotypical analyses of the bone marrow revealed differentiation of the malignant cell clone, in the absence of bone marrow hypoplasia. 4 of 5 patients treated only with ATRA until complete remission had late leukopenia. The most frequent adverse effects were dryness of skin and mucosae, hypertrigliceridemia and hypercholesterolemia, and a moderate increase in liver transaminases. An increase in the white blood cell count was common, and in two cases exceeded 35.0 x 10(9)/l. One of these patients developed multiple thrombosis of the extremities after cytotoxic chemotherapy. We frequently observed an increase in lactic dehydrogenase levels that was concomitant with the peak in the white blood cell count. The only patient on whom complete remission was not achieved was 60 years old, had chronic obstructive pulmonary disease, and died in the third week of therapy with a pulmonary distress syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

[Acute promyelocytic leukemia. The therapeutic advances]. / Leucemia promielocítica aguda. Avanços terapêuticos.

Acute promyelocytic leukemia (APL) is a rare subtype of acute myelogenous leukemia. It is frequently associated with a life-threatening hemorrhagic diathesis, often aggravated by induction cytotoxic chemotherapy. Patients with APL have bone marrow infiltration by abnormal promyelocytes, usually with prominent cytoplasmic granulation. These patients have a unique cytogenetic abnormality, a balanced reciprocal translocation between the long arms of chromosomes 15 and 17. The nuclear retinoic acid receptor alpha gene, on chromosome 17, is translocated to the PML gene region, on chromosome 15, resulting in the synthesis of two fusion messenger ribonucleic acids, PML/RAR-alpha and RAR-alpha/PML, easily detected by the reverse transcriptase polymerase chain reaction. This assay is extremely useful in the diagnosis and detection of minimal residual disease in APL patients. All trans-retinoic acid (ATR) differentiates the malignant cell clone and corrects the coagulopathy associated with this disease. The most important adverse effect is a respiratory distress syndrome, treatable with steroids, if detected at its onset. ATR yields durable remissions in patients with APL, after consolidation with cytotoxic chemotherapy.

[Bone marrow transplantation. Preliminary results]. / Transplantação da medula óssea. Resultados preliminares.

From May 1989 to March 1991 60 bone marrow transplants were performed--41 allogeneic and 19 autologous--in patients with malignant and nonmalignant hemopathies and solid tumors. The transplant related mortality was 19.4% and 10.5% for allografts and autografts, respectively. The projected relapse free survival at 22 months in patients with malignant hemopathies who underwent allotransplantation in early phase was 81% and, in patients in advanced phase 21.4% (p less than 0.001). In patients with aplastic anemia the actuarial survival at 22 months is 60%. In autografted patients, only one failed to have full engraftment and is dependent on platelet support at day 79. Follow-up in all autografts is less than one year. These preliminary results of our bone marrow transplant program show low morbidity and mortality related to transplantation and suggest significant therapeutic effect.

[Psychiatric disorders in hospitalized patients with hematologic neoplasms]. / Distúrbios psiquiátricos em doentes internados com neoplasias hematológicas.

We report the results of a year of regular psychiatric support in the Hematology Department (UTI-DH) at Santa Maria Hospital. During a six-month period, the total group of hospital inpatients suffering from leukemia, Hodgkin's disease or non-Hodgkin's lymphoma, were assessed, using a semi-structured interview. A prevalence of 30% of adjustment disorders (depression and/or anxiety) and 2% of organic mental syndromes was found employing the DSM-III-R diagnostic system. In the second six-month period only patients referred by their doctor and/or nurse were observed. The two rates are discussed. A lower prevalence was found if compared with other studies in cancer patients in general. Possible causes will be focussed. General problems related to the nature of the cancer were identified. The consequences of the omission of cancer diagnosis to patients are analysed. The communication between physician and patient which is often neglected irrespectively of the culture or country, is stressed.