Marion E. Erlandson

Late Cardiac Complications of Chronic, Severe, Refractory Anemia with Hemochromatosis

Four fifths of 49 patients with chronic, severe anemia from birth have manifested, usually during the second decade, signs of hemochromatosis with cardiac involvement. Nineteen patients had 33 attacks of pericarditis. Congestive cardiac failure with arrhythmias developed in 26 patients, and all but one died with this complication. Four others died of sepsis before heart failure was evidenced. Postmortem examination showed hemochromatosis with severe myocardial involvement.

Hazard of severe infections in splenectomized infants and children

Abstract A series of nineteen cases of severe and often fulminating infection, six terminating fatally, in children following splenectomy is presented. Except for two cases of traumatic rupture of the spleen, the major indication for splenectomy was based on the needs arising from an established blood disorder. The infections fell into several well defined categories: meningitis, acute benign pericarditis (in patients with Cooleys anemia), acute endocarditis and sepsis. In the cases in which bacterial diagnosis was possible pneumococcus was the most frequent offender and of the clinical types, meningitis was most common. Pneumococcic meningitis predominated in this and other reports. In the majority of cases the interval between splenectomy and infection was two years or less, with a range from one day to sixteen years. The age at splenectomy extended from thirteen months to seventeen years. There was no diminution in the concentration of gamma globulin in these patients. The cases described in this paper, together with current reports of a similar nature, suggest more than a random association between splenectomy and susceptibility to infection. An extensive experimental background implicates the spleen in fundamental processes relating to resistance to infection. The sample reported in this paper is avowedly small in comparison with the ever increasing number of splenectomies. Nevertheless, while the benefits accruing from splenectomy are substantial and well documented, the potential hazards demand that exact criteria be established in selecting patients for the operation. In the light of the experience cited in this paper, the young splenectomized patient requires close supervision for several years postoperatively so that immediate and energetic treatment may be instituted in the event of sudden and severe illness. Specific prophylaxis presents manifold problems which await further study

Hemolytic disease in the neonatal period and early infancy.

Summary A basic understanding of the consequencesof hemolysis and of their intensification in infancy together with an awareness of the etiology and ultimate prognosis of each particular disorder is necessary to optimal management of infants in whom hemolytic disease occurs.

Chromium51 Elution from Hemoglobin and Intact Erythrocytes of Adults, Infants and Patients with Cooley's Anemia.∗:

Summary Accelerated rates of chromium51 elution from hemoglobin solutions containing fetal hemoglobin have been shown to be present in specimens obtained from an infant and from patients with Cooleys anemia. This acceleration has been shown to be directly related to the percent fetal hemoglobin present. Rapid rates of chromium51 elution could not be demonstrated to occur from intact labeled erythrocytes containing large amounts of fetal hemoglobin studied by the technic of dialysis.

The coagulation mechanism in patients with thalassemia major

A study of the coagulation mechanism in patients with thalassemia major revealed no evidence of a severe bleeding diathesis. Seven patients demonstrated 1 or 2 minimal coagulation defects; 19, mild-to-moderate first phase defects; 1, thrombocytopenia; and 14, mild-to-moderate prothrombin complex defects. Mild degrees of impairment appeared between 7 to 10 years of age and progressed with age to the moderate degree found in older patients. No correlation was found between epistaxis and the abnormalities of the clotting mechanism. A general correlation was found between the coagulation status and hepatic function as assessed by other measurements.

Maturation in chronic anemias

(6 months) . While the exact mechanism of runt (homologous) disease is not completely understood, it nonetheless is an established experimental entity in certain laboratory animals and a theoretical hazard of all human pregnancies. Transplantation of foreign immunologically competent and self-replicating cells into the fetus is the accepted basis for the classic type of runt disease. The theoretical and practical implications of the transplantation and then slow rejection of foreign cells in this patient will be discussed. I t appears that homologous disease even under such clear-cut experimental conditions remains only a theoretical hazard in humans.