Acquired von Willebrand's syndrome associated with hydatid disease of the spleen--disappearance after splenectomy.

A new case of acquired von Willebrand's syndrome (vWS) is described in a 31-year-old woman with a hydatid splenomegaly and with a history of repeated abortions at an advanced stage of pregnancy, a positive serology for syphilis and a mildly elevated titre of antinuclear antibodies, with no family history of bleeding. There is an inhibitory effect on factor VIII: C (antihaemophilic factor) as well as on factor VIIIR: Ag (related antigen) and on factor VIIIR: RCo (ristocetin cofactor), and it is precipitated by rabbit anti-IgG antiserum. This inhibitory effect was demonstrated using the patient's plasma heated to 56 degrees C for one hour so as to dissociate circulating immunocomplexes. All the abnormalities of haemostasis, as well as the positive serology for syphilis, disappeared after splenectomy, and the ANA titre reverted to normal. The clinical and biological peculiarities of the case are discussed, and are interpreted in the light of the findings recorded in patients showing "lupus" anticoagulant.

Autologous bone marrow transplantation as consolidation therapy for non-Hodgkin's lymphoma patients with poor prognostic features.

Theoretical considerations and preliminary results of clinical trials support the earlier use of autologous bone marrow transplantation (ABMT) in poor prognosis non-Hodgkin's lymphoma (NHL). A prognostic analysis of 50 patients with intermediate or high grade NHL younger than 60 years, who achieved at least one complete remission and were not treated with BMT, was performed. Patients with bulky tumor at diagnosis and/or serum LDH greater than or equal to 600 U/l do poorly with conventional chemotherapy. Twelve patients with these high-risk initial characteristics in first complete remission (CR) and six patients in second or third CR were treated with cyclophosphamide (60 mg/kg x 2) and total body irradiation (1000-1200 cGy) followed by ABMT. Overall disease-free survival was 65% at a median follow-up of 35 months. No differences were found between the first and later CR patients. The rate of toxic death was 11%. Disease-free survival after first CR was better for 1st CR ABMT patients than for a historical chemotherapy control group with similar poor prognosis features (p = 0.008). These results support the use of ABMT in selected, high-risk NHL patients in first CR.

[Bone marrow autotransplantation in patients with acute myeloblastic leukemia in primary remission]. / Autotrasplante de medula ósea en pacientes con leucemia mieloblástica aguda en primera remisión.

Fifteen bone marrow autotransplants (BMAT) in patients with acute myeloblastic leukemia (AML) were performed after the first remission. The mean age was 37 years (range 12 to 60 years). According to the morphological classification FAB, 8 patients had monocytic leukemia (M4, M5) and 7 myeloid leukemia (M1, M2, M3). The mean interval elapsed between the date of complete remission and the BMAT was 3.9 months (range 1 to 5-9 months). In 8 patients this interval was longer than 6 months and in 7 cases it was shorter than 6 months. After achievement of the complete remission all patients underwent certain cycles of intensification before the BMAT. Eight patients received only a cycle whereas 7 patients received more than one cycle (between 2 and 4). The conditioning protocol consisted of cyclophosphamide (CP) (60 mg/kg x 2) and total body radiotherapy (TBR) (10 Gy) in 9 patients; CP and busulfan in five; and CP, cytarabine at high doses and melphalan in one case. Marrow extraction was performed after completion of chemotherapy of intensification. In 5 cases the bone marrow was depleted of leukemic cells by previous in vitro treatment with ASTA-Z. There are at present 8 alive patients. The survival free of illness was 51.8%. Seven patients died: 3 cases because relapse of the leukemia, 3 due to attachment failure of the transplantation, and one patient suffered a viral myocarditis. The survival free of illness was significantly longer in those patients transplanted after 6 months of the complete remission.

[Prevention of acute graft versus host disease using 3 prophylactic schemes]. / Prevención de la enfermedad injerto contra huésped aguda con tres esquemas profilácticos diferentes.

During 7 years 81 patients received an allogeneic bone marrow transplant (BMT) for several diseases. The prevention of graft-versus-host disease (GVHD) was undertaken with methotrexate (MTX), MTX plus antilymphocytic gammaglobulin plus prednisone (MTX + ALT + P), and elimination of the T-lymphocytes of the donor's bone marrow with the monoclonal antibody CAMPATH-1. The actuarial survival of the patients who did not develop GVHD was significantly better than that of those who developed grade II-IV GVHD: 56% [95% confidence interval (CI) 39-71%] versus 10% (95% CI 3-25%) (p less than 0.0001). However, actuarial survival was similar in each of the three groups: MTX 35%, MTX + ALT + P 38%, and CAMPATH-1 43%. The incidence of GVH disease, when the sex of the donor and the receptor were different, was significantly higher than in cases where the donor and the receptor had the same sex: 45% (95% CI 31-58%) vs 15% (95% CI 8-28%) (p less than 0.005). By contrast, significant differences were not found between the three groups in the incidence of GVHD: MTX 36%, MTX + ALT + P 34%, and CAMPATH-1 20%. In patients with leukemia, a higher number of relapses occurred in the MTX group, because a higher number of patients in second or third complete remission (CR) or with active disease underwent transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

[Prospective study on the prevalence of iron deficiency in Cantabria among children 6 to 14 years old]. / Estudio prospectivo sobre la prevalencia de ferropenia en Cantabria entre niños de seis a catorce años.

Iron deficiency is the most common worldwide micronutrient deficiency in developed countries. To analyze its prevalence in children and adolescent population from our region, we studied Hb, Hto, red cell volumes, serum iron, total iron binding capacity, transferrin saturation and serum ferritin in a population of 380 individuals. These also fulfilled an epidemiological questionnaire. Mean values and normal reference ranges are shown according to age sex, which is very important to optimize the identification of individuals with iron deficiency. Overall prevalence of iron deficiency was 15.7% and it was higher in adolescent males from 12-14 years (21.8%) and children from 6-8 year (18.8%). Significant differences related to residence or to socioeconomic status were not found. The health consequences of iron deficiency are to be considered in any public health planning project.

[Iron deficiency in female blood donors. Results obtained by applying a prevention protocol]. / Ferropenia en mujeres donantes de sangre. Resultados obtenidos aplicando un protocolo de prevención.

Iron deficiency, often associated to anaemia, is the main cause of deferring women from blood donation: an incidence of 9.5% over a series of 1,437 women was found in our region. The strategy designed to face this problem was based upon precise diagnosis (erythrocyte protoporphyrin values) dietetic counselling, occasional iron therapy, and reduction of the frequency of blood donations. The results attained were encouraging, as the incidence of deferments was 13%; however, the percentage of women who quit blood donation was 86% of the first-time female donors.

[Anti-HCV antibodies among blood donors in Cantabria. Incidence and relation to transaminase levels]. / Anticuerpos anti-VHC en donantes de sangre de Cantabria. Incidencia y relaciòn con niveles de transaminasas.

The incidence of anti-HCV antibodies found in 1,918 blood donors from Cantabria was 0.31%. Taking a level of 80 U/L ALT as a standpoint, 0.21% of samples with lower values correspond to anti-HCV positive cases, versus 6.25% of samples above that figure. Within those donors with ALT greater than 80 U/L, viral and non-viral profiles were established from biochemical criteria, their anti-VHC positivity frequencies being 22.22% and 0%, respectively. Non-viral profile donors represent 1% of the total number who might not be rejected.

Implementation of a strategy to prevent TRALI in a regional blood centre.

Transfusion-related acute lung injury (TRALI) can be a life-threatening complication of transfusion and it is probably underdiagnosed. Human leucocyte antigen (HLA) and granulocyte antibodies are thought to play a major role, but preventive measures are difficult to implement. In our regional blood centre, we implemented a preventive strategy avoiding donor deferral. Previously, pregnant apheresis donors were screened for HLA antibodies, and those with positive results were assigned to a plasma-only protocol. Plasma from these donors and from all previously pregnant whole blood donors was diverted for protein fractionation. Plasma-poor red blood cells (in additive solution, buffy coat removed) and platelets (pools with additive solution) were prepared. Prestorage leucodepletion was also applied. We found HLA antibodies in 18.1% of previously pregnant apheresis donors, and our strategy caused a 6.0% loss of apheresis platelets, a 4.8% increase of apheresis fresh frozen plasma (FFP) and a 7.8% loss of transfusable apheresis FFP. The effect on FFP from whole blood donors could be compensated. The platelet preparation method reduced the mean volume of plasma from each donor to 24.4 mL. Fifteen months after the start of our strategy, no cases of TRALI have been reported. Our experience shows that a practical strategy to prevent TRALI is feasible.

Allogeneic bone marrow transplantation versus intensification chemotherapy for acute myelogenous leukaemia in first remission: a prospective controlled trial.

In 1982 we began a prospective controlled trial to assess the effectiveness of allogeneic bone marrow transplantation and intensive post-remission chemotherapy for patients with acute myelogenous leukaemia in first complete remission. Fourteen patients, 3-45 years of age, who had an HLA-identical sibling donor, received bone marrow transplantation. Twenty-five patients who either lacked an HLA-identical sibling or were over 45 years of age received intensive consolidation chemotherapy including high-dose cytosine arabinoside with or without adriamycin. The actuarial rate of continued complete remission (CCR) at 3 years was significantly higher in the transplantation group than in the chemotherapy group: 70% (95% confidence interval 35-91%) compared with 10% (95% confidence interval 2-30%); P = 0.01. However, the actuarial rate of CCR was not significantly different between the transplantation group and patients under 45 years in the chemotherapy group: 70% (95% confidence interval 35-91%) compared with 17% (95% confidence interval 4-45%), 0.1 greater than P greater than 0.05. The actuarial probability of leukaemia relapse was significantly lower in the transplantation group than in the chemotherapy group: 10% (95% confidence interval 4-21%) compared with 88% (95% confidence interval 70-96%), 0.005 greater than P greater than 0.001. There was no significant difference between both groups if we compare only the patients who died of non-leukaemic causes: 22% (95% confidence interval 9-42%) versus 25% (95% confidence interval 7-59%), P = NS. In summary, this study shows that allogeneic bone marrow transplantation is a better anti-leukaemic treatment than is intensive consolidation chemotherapy in patients with AML in first complete remission.

Complete recovery of hemopoiesis following bone marrow transplant in a patient with unresponsive congenital hypoplastic anemia (Blackfan-Diamond syndrome).

Allogeneic bone marrow transplantation (BMT) was carried out on a 5-year-old boy with congenital hypoplastic anemia (CHA), who did not respond to corticosteroids and who was displaying signs of progressive hemosiderosis. Pretransplant preparation had to be modified because respiratory failure and cerebral edema supervened. This preparatory regimen consisted of busulfan (2 mg/kg for four days), cyclophosphamide (50 mg/kg for one day), and total body irradiation (750 rad). Hemopoiesis was completely restored and is still maintained 650 days after transplantation. This is the second published report on the use of BMT to treat a patient with CHA, and it is the first time it has resulted in long-term survival. BMT should be considered for patients with CHA who do not respond to corticosteroids.

Controlled-rate versus uncontrolled-rate cryopreservation of peripheral blood progenitor cells: a prospective multicenter study. Group for Cryobiology and Biology of Bone Marrow Transplantation (CBTMO), Spain.

BACKGROUND AND OBJECTIVE: Cryopreservation of hemopoietic progenitors for transplantation has been traditionally performed by the use of a controlled-rate freezer. Several groups have reported successful cryopreservation of progenitor cells at -80 degrees C without a controlled-rate freezer. In an attempt to elucidate whether both methods are equally efficient, we compared controlled-rate versus uncontrolled cryopreservation of peripheral blood progenitor cells (PBPC) in a prospective, multicenter study. DESIGN AND METHODS: Apheresis products from patients undergoing PBPC mobilization were split into two aliquots, and cryopreserved simultaneously by both methods, in autologous plasma plus 10% dimethylsulfoxide. Controlled-rate samples were placed into a programmable freezer with a cooling rate of 1-2 degrees C/min. Uncontrolled-rate samples were directly introduced into a -80 degrees C mechanical freezer. After thawing, cell counts, assays for viability, clonogenic cultures and CD34+ cell enumeration were performed. RESULTS: A total of 105 cases were included. No significant differences were found in viability (mean 88.8 +/- 13% in the controlled-rate group vs. 89.7 +/- 12% in the uncontrolled-rate group), nucleated cell loss (23.5 +/- 23% vs. 23 +/- 22%), mononuclear cell loss (19 +/- 23% vs. 19.1 +/- 22%), and loss of CD34+ cells (34.3 +/- 33% vs. 28.6 +/- 34%). On the other hand, recovery of granulomonocytic colony-forming units (CFU-GM), was significantly better with the controlled-rate technique, than with the non-controlled-rate method (104.3 +/- 95 vs. 86.5 +/- 80, respectively; p = 0.048). INTERPRETATION AND CONCLUSIONS: Our results indicate that both techniques are suitable for cryopreservation of PBPC, although a better recovery of committed progenitors is achieved by the controlled-rate method. Therefore, the use of controlled-rate freezer should probably be recommended.