Each of three families of northern European origin contains a mentally retarded son with hemoglobin H (Hb H) disease. One parent is a carrier of mild alpha-thalassemia and the other is normal, suggesting that this form of Hb H disease results from the interaction between an inherited defect of alpha-chain production and one member of the pair in chromosome 16 and a new mutation on the other. Restriction-enzyme analysis indicated that the new mutation was not the same in the other three patients, and demonstrated at least two hitherto undescribed lesions involving the alpha-globin gene cluster. Unless the association between the Hb H disease and mental retardation is fortuitous, the new mutations may also be related to the development changes in these children. Since the mutations only came to light because there was concurrent inheritance of an additional alpha-thalassemia determinant, this type of mutation of chromosome 16 may have been overlooked in other mentally retarded patients.
On 2 separate occasions intravenous nitrogen mustard administration to a patient with Hodgkin's disease resulted in cerebral toxicity characterized by hyperpyrexia and coma 7 days after initiation of therapy. In the first instance, operative findings and clinical response to ventricular decompression indicated an acute internal hydrocephalus. During the second episode, treatment with osmotic diuresis, hypothermia, and corticosteroids resulted in complete resolution of all neurologic signs and symptoms. These findings suggest that acute cerebral toxicity following conventional doses of nitrogen mustard, although rare, may occur.
Elevated titers to the Epstein-Barr virus have previously been reported in a number of lymphoproliferative diseases, including Burkitt’s lymphoma, infectious mononucleosis, and Hodgkin’s disease. This study also demonstrates a significantly higher titer against EBV in a group of patients with chronic lymphoproliferative disease (CLL) than in a group of patients with chronic myeloproliferative disease (CML) or normal individuals. No significant antibody changes were detected in the 20 CML patients or 23 of the 24 CLL patients who were followed for a period of time up to 5 yr. It appears that the elevated EBV titers seen in patients with CLL reflect an event or process occurring prior to the onset of disease or in the very early stages rather than a nonspecific rise paralleling the increase in total-body lymphocytes.
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