The skull in homozygous beta-thalassaemia may present several abnormalities, such as osteopenia, widening of the diploic space, and a "hair-on-end" appearance. In some cases it presents also a particular stratified appearance caused by a variable number of osseous lamellae, parallel with the inner table. This "lamellated skull" was observed in 16 out of 150 patients affected by the disease (10.6%). Possible mechanisms are discussed. The lamellar osseous changes could be due to repeated periosteal osteoblastic reactions to the sinusoidal neovascularization associated with marrow hyperplasia in poorly transfused patients.
Solitary or multiple circumscribed osteolytic areas are described in seven patients, selected from 250 patients with homozygous beta-thalassaemia (Cooley's anaemia). On X-ray examinations, these areas appear as purely osteolytic lesions with well-defined margins not associated with sclerosis. The possible mechanisms are discussed.
This study was intended to evaluate whether unusual circadian patterns in blood cells exist in cancer patients. Ten patients (five men and five women) suffering from advanced malignancy were compared with a control group of apparently healthy volunteers, of comparable age and sex. After synchronization of daily activities, meals and rest of the two groups, blood samples were taken four times (at 8.00 a.m., 12.00 a.m., 4.00 p.m. and 8.00 p.m.) in a single day. The total red and white cell counts, haemoglobin, platelet count, and neutrophil, lymphocyte, eosinophil, monocyte and basophil differential white cell counts were analysed by both conventional (Student's t-test; multifactorial analysis of variance) and inferential statistics (single and mean cosinor). The average values for platelets (P = 0.04), white blood cells (P = 0.004) and lymphocytes (P << 0.001) showed significant changes with time, independently of disease state. Cosinor analysis indicated a circadian rhythmicity for haemoglobin (P = 0.02), eosinophils (P = 0.014), and lymphocytes (P = 0.001) in healthy subjects, and for eosinophils only (P = 0.024) in cancer patients.
The main lesions of the skull and hand, observed in a group of hypertransfused beta-thalassaemic patients, are compared with a control group of low-transfused patients. Bony abnormalities reflect the relationship between proliferating bone marrow and bone cortex, and hypertransfusion therapy will prevent development of lesions only if established early in life. If this is done, the diploë in the skull may become normal, overgrowth of facial bone is moderate, pneumatisation of the paranasal sinuses is not completely prevented, and the "hair-brush" pattern may disappear completely. A normal appearance of the hand in adequately treated patients differentiates between prepubertal patients and adults.
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