A novel syndrome of radiation-associated acute myeloid leukemia involving AML1 gene translocations.

AML1 is a transcriptional activator that is essential for normal hematopoietic development. It is the most frequent target for translocations in acute leukemia. We recently identified 3 patients in whom pancytopenia developed almost 50 years after high-level radiation exposure from nuclear explosions during or after World War II. In all 3 patients, acute myeloid leukemia (AML) eventually developed that had similar characteristics and clinical courses. Cytogenetics from the 3 patients revealed a t(1;21)(p36;q22), a t(18;21)(q21;q22), and a t(19;21)(q13.4;q22). By fluorescent in situ hybridization (FISH), all 3 translocations disrupted the AML1 gene. Two of these AML1 translocations, the t(18;21) and the t(19;21), have not been reported previously. It is possible that the AML1 gene is a target for radiation-induced AML. (Blood. 2000;95:4011-4013)

Immature teratoma of the ovary--an unusual case.

A 30-year-old female was found to have a malignant immature teratoma of the ovary 14 years ago. This was treated with surgery followed by chemotherapy. Two years later, hepatic metastasis was discovered and treated with different multiple-agent chemotherapy, resulting in transformation or evolution into a mature teratoma form of disease. Twelve years later, this disease underwent malignant degeneration and transformed into a metastatic adenocarcinoma. This case report illustrates that malignant ovarian immature teratoma, teratocarcinoma, like its testicular counterpart, can evolve or differentiate into an apparently benign form of disease, but ultimately leads to death due to subsequent malignant transformation into new forms of malignancy arising from any of the three primordial germ cell layers.

Monitoring of anticoagulant therapy during open-heart surgery in children with congenital heart disease.

A significant reduction (30%) in the amount of postoperative blood loss from 1.12 to 0.70 ml/kg/h was noted in pediatric patients subjected to cardiopulmonary bypass for congenital heart disease surgery when the activated whole blood clotting time was utilized to monitor anticoagulant therapy. However, no difference in the incidence (11%) of postoperative hemorrhage was noted. Extensive preoperative laboratory hemostasis testing and routine immediate postoperative use of platelet transfusion and fresh frozen plasma (in cyanotic heart defects) failed to predict or correct those patients with significant postoperative hemorrhage.

Chronic benign idiopathic neutropenia in Jordanians.

Bone marrow granulocyte reserves and the bone marrow storage pool were evaluated in 10 Jordanian adult patients with chronic benign idiopathic neutropenia, by measurement of the peripheral blood granulocyte increment following the administration of hydrocortisone and lithium. The increment in each case was statistically significant, indicating retention of effective neutrophil production in stressful situations and thus the benignity of this disorder.

Desacetyl vinblastine amide sulfate induced ineffective erythropoiesis.

Ineffective erythropoiesis occurred during desacetyl vinblastine amide sulfate (VDS) therapy of a patient with metastatic Ewing's sarcoma. The peripheral blood was characterized by anisocytosis, poikilocytosis and reticulocytopenia. Bone marrow showed megaloblastic red cell hyperplasia with nuclear fragmentation, binuclearity and abnormal metaphases. Radioiron incorporation into red cells was markedly decreased. Electron microscopy showed red cell surface changes previously called ropalocytosis. Normal erythropoiesis promptly resumed when VDS treatment was withheld. Although less common than leukopenia, red cell abnormalities are part of the spectrum of vinca alkaloid toxicity.