Local control and complications after radiation therapy for primary orbital lymphoma: a case for low-dose treatment

Orbital involvement at the time of initial presentation is unusual in non-Hodgkin's lymphoma. In an effort to identify potential ways of improving the radiotherapeutic management of this disease, the records of 22 patients were reviewed retrospectively. All had biopsy-proven orbital non-Hodgkin's lymphoma, and the minimal, median, and maximal durations of follow-up in surviving patients were 4.8 years, 7.0 years, and 17.4 years, respectively. Permanent local control was achieved in 21 of the 22 patients (96%). Complications were scored according to a grading scheme in which grade 1 was the least significant complication and grade 4 was blindness as a result of radiation therapy. Of the 12 patients who received a radiation dose less than 35 Gy, 6 developed a grade 1 or grade 2 complication. Of the 10 patients treated with greater than or equal to 35 Gy, 6 experienced a complication, 1 of whom had a grade 4 complication resulting in blindness and another who developed a severe keratitis, which was scored as a grade 3 complication resulting in decreased visual acuity. At last follow-up, 10 patients were alive at 4.8 to 17.4 years after completion of radiation therapy, 4 had died of intercurrent disease at 3 months to 10.6 years, and 8 had died of disease at 3 months to 15.8 years. Actuarial survival for the entire group was 75% at 5 years and 48% at 10 years. Survival in patients with Stage I AE disease (lymphoma confined to orbit) at presentation was 87% at 5 years and 50% at 10 years, and survival in patients with Stage II A through Stage IV disease was 36% at 5 years and at 10 years. Primary orbital lymphoma is an indolent disease characterized by prolonged survival after radiation therapy. Excellent local control can be achieved with radiation doses of 20 Gy to 35 Gy. Higher doses may result in an increased risk of complications.     

Is homozygous α-thalassaemia a lethal condition in the 1990s?

Two cases of homozygous α-thalassaemia who received active treatment in accordance with parental wishes are reported. One infant survived and the other, although successfully weaned off mechanical respiratory support, unexpectedly developed portal vein thrombosis and died. Homozygous a-thalassaemia, a condition previously considered to be universally fatal, and an indication for therapeutic abortion, is now potentially curable with advances in diagnostic technology and treatment. However, active management of these cases raises serious ethical questions and has major financial implications on the health-care system. Invasive prenatal and intensive postnatal interventions should remain experimental and cannot be recommended as routine clinical practice until the questions of long-term neurodevelopmental outcome, and the morbidity and mortality associated with bone-marrow transplantation have been fully addressed. As a result of advances in information technology, more and more parents of affected foetuses are likely to request active treatment.     

Magnetic resonance imaging of prosthetic heart valves

To evaluate the safety of magnetic resonance (MR) imaging of prosthetic heart valves, nine different synthetic and tissue valves were studied ex vivo. Deflection was measured in 0.35-tesla (T) and 1.5-T superconducting magnets and at the edge of the bore of a 2.35-T electromagnet in field gradients of 5, 1.1, and 6.3 mT/cm, respectively. No valve deflected in the 0.35-T magnet; six synthetic valves deflected 0.25 degrees-3 degrees in the 1.5-T magnet; all valves deflected 1 degree-27 degrees at the edge of the 2.35-T magnet. Each valve was then submerged in a vial of water and the temperature was measured immediately before and after each of two spin-echo imaging sequences in the two superconducting magnets. No significant temperature rise followed exposure in either magnet. Image distortion varied from negligible to severe in both imagers; magnitude of distortion paralleled magnitude of deflection. These data suggest that patients with present-day prosthetic heart valves can be safely imaged in present-day MR imagers and that prosthesis-induced artifacts will not interfere with interpretation in most instances.     

Young adults with α1antitrypsin deficiency identified neonatally: their health, knowledge about and adaptation to the high-risk condition

The psychological and psychosocial consequences of screening for α₁-antitrypsin deficiency (α₁ ATD) were investigated when the subjects were 5–7 years old. The present study was conducted when the subjects were 18–20 years old, the foci of interest being their health, psychosomatic problems, knowledge about α₁ATD and the potential effect of that knowledge on their lives and future family planning. Samples of 61 PiZ and 61 demographically matched control subjects, 18–20 years old, were asked to participate. Written, structured questionnaires covered the following items: basic familial characteristics, psychosomatic symptoms, opinions on medical check-ups, information and views on future α₁ATD screening, whether the knowledge about α₁ATD had affected the life and family planning of α₁ATD individuals. Items concerning the “α₁ATD matter” were excluded in the questionnaires given to the controls. Questionnaire data were obtained from 50 α₁ ATD and 48 control individuals, 41 of each being matched α₁ATD-control pairs. No significant differences were found in demographic or educational backgrounds, psychosomatic complaints such as headache, sleep difficulties, stomach ache, tiredness or anxiety. Lung symptoms occurred more frequently in α₁ATD subjects (p= 0.05). Six per cent of the α₁ATD individuals planned working careers with a high risk of air pollution. The majority (86%) of the α₁ATD subjects perceived the contact with the medical services as positive; 14% as both positive and negative. The information concerning α₁ATD was assessed as satisfactory by 73%, as both good and bad by 17% and as unsatisfactory by 10%. All α₁ATD subjects advocated general screening for α₁ATD, the neonatal period being chosen as optimal by 94%. Half of the α₁ATD individuals thought that the knowledge of their high-risk condition had affected their lives, particularly their awareness of the dangers of smoking and environmental pollution. The majority, 88%, knew that they should avoid smoking to protect their lungs. In conclusion, no negative psychosocial consequences of the neonatal α₁AT-screening were found in early adulthood. The α₁ATD individuals were aware of the dangers of smoking and were of the opinion that α₁ AT-screening should be recommended.     

Cyclophosphamide in the treatment of orbital vasculitis

When vasculitis, an angiocentric and angiodestructive process, occurs in the orbit, the clinical presentation and radiographic findings resemble those of idiopathic inflammatory pseudotumor. Three patients, two men and a woman, 28 to 72 years old, initially thought to have "pseudotumor" failed to response to corticosteroid therapy. Orbital biopsy specimens in all patients disclosed vasculitis. There was no evidence of systemic vasculitis. High-dose prednisone effectively eliminated pain and reduced inflammation but did not adequately control fibrosis formation leading to ultimate loss of function. Each patient eventually lost an eye to this process. Therapy with cyclophosphamide, a B-cell cytotoxic drug, produced a prompt response in terms of eliminating pain, inflammation, and formation of fibrous tissue. Cyclophosphamide therapy has been instrumental in preserving sight in each patient's remaining eye. In such cases we believe the benefits of cyclophosphamide therapy outweight the known risks.