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Biomedical subjects

A Iriondo

Publications and source records attributed to A Iriondo.

59 records · Page 4Linked to original sources

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.

Anemia, Aplastic↗

Acute lymphatic leukemia with mediastinal involvement.

Mediastinal involvement was found in 11 (group A) of 43 patients affected with acute lymphatic leukemia when early thoracic roentgenograms of these patients were reviewed. Several clinicobiological characteristics of these patients were compared with those with a normal mediastinum as shown in their roentgenograms (group B). Statistically significant differences were observed between group A and group B not only as far as age was concerned (13 years group A, 7.4 years group B) but also in the ratio males/females (10:1 group A, 10:20 group B) and with regard to the leukocyte counts (266 x 10(9)/liter group A, 20 x 10(9)/liter group B). In addition, patients from group A showed a greater 'tumoral mass' and a more prominent extrahematological involvement (45% group A, 15% group B). In these cases 'convoluted' cells were frequently discovered and the blastic cells exhibited significantly lower scores of PAS-positivity and more marked acid phosphatase activity than those in group B. Although the rate of complete remissions (CR) obtained in both groups was similar (80% group A, 93% group B), marked differences were observed not only in the duration of CR but in the period of survival after CR as well, both factors being more prolonged in group B patients. 50% of patients with mediastinal involvement (group A) relapsed in the first 6 months of the evolution of the disease.

Adolescent↗