Increased Hemolysis after Intramuscular Iron Administration in Patients with Paroxysmal Nocturnal Hemoglobinuria: Report of Six Occurrences in Four Patients, and Speculations on a Possible Mechanism

Four patients with PNH were described who developed accelerated activityof their disease after the intramuscular administration of iron-dextran fortreatment of iron deficiency. This was considered to reflect a unique effect ofiron on PNH erythrocytes. It was postulated that this effect resulted from celldamage caused by iron catalyzed peroxidation of erythrocyte lipids, a reaction to which PNH erythrocytes could be unusually susceptible by virtue ofan increased content of unsaturated fatty acids or pro-oxidants or decreasedcontent of antioxidants.

Submitted on August 13, 1964 Accepted on November 11, 1964     

Recurrent lupus nephritis in a rejected renal allograft

SUMMARY: The case of a 48-year-old female patient who underwent renal transplantation because of an end-stage renal disease after membranous glomerulonephritis (WHO class V) in systemic lupus erythematosus (SLE) is presented. the patient lost one cadaveric allograft immediately after transplantation because of renal vein thrombosis, presumably caused by anti-Cardiolipin antibodies. A second cadaveric allograft showed a stable function for several years before slowly deteriorating. an abrupt increase of serum creatinine led to the suspicion of a final episode of acute rejection. A biopsy was performed, which showed an overlap of rejection and recurrent iupus nephritis in an advanced chronically damaged allograft. the lupus nephritis recurred as the same WHO class V as in the native kidney, but without significant predictive clinical or serological signs of SLE activity. the case presented and a review of the literature indicate that the frequency of recurrent lupus nephritis might be underestimated, and earlier surveillance biopsies in transplanted SLE patients should be considered.     

Desquamative gingivitis and balanitis — linear IgA disease or cicatricial pemphigoid?

A 38-year-old man presented with gingival inflammation together with erosions of the penis. Direct immunofluorescence demonstrated linear deposits of IgA at the basement membrane zone; indirect immunofluorescence and immunoblotting were negative. Linear IgA disease (LAD) was therefore suspected and treatment with dapsone initiated but this was changed to sulfamethoxypyridazine and systemic corticosteroids because of methaemoglobinaemia. During 1-year follow-up the lesions continued to wax and wane although they were never as extensive as before. Eighteen months after disease onset there was scarring of the penis together with suspected fibrosis of the inflamed gingival region. In addition the patient was HLA DQ7(3) positive, a haplotype thought to be increased in patients with cicatricial pemphigoid (CP); LAD with scarring or CP with solely linear IgA deposits are possible diagnoses of his condition.     

Effects of in Vivo Hyperoxia on Erythrocytes: V. Changes of RBC Glycolytic Intermediates in Mice after in Vivo Oxygen under High Pressure

During prehemolytic exposure of mice to 100 per cent oxygen, changes inglycolytic intermediates were observed. Red cell ATP and hexosemonophosphate compounds were increased and fructose 1-6 diphosphate, triose-phosphates, ADP and TPN were decreased. These changes were not affected bysplenectomy or tocopherol status of the animals.

Submitted on April 4, 1966 Accepted on August 6, 1966     

Recurrent Ecchymoses and Cutaneous Hyperreactivity to Hemoglobin: A Form of Autoerythrocyte Sensitization

A patient with recurrent painful ecchymoses was presented with featuressimilar to those described for patients with autoerythrocyte sensitization.This patient differed in that she was reactive to the red cell hemolysate andpurified hemoglobin and not to red cell lipid, red cell stroma, or leukocyteDNA. No evidence was found to support the hypothesis that the abnormalvascular reaction involved an immunologic mechanism. The patient was observed forcibly inducing bruises but not manipulating the skin tests. It wasconcluded that a cutaneous hyperreactivity to hemoglobin in association withpsychological disturbances was responsible for the painful ecchymoses in thispatient.

Submitted on August 16, 1966 Accepted on February 27, 1967     

Coexistent Paroxysmal Nocturnal and Cold Hemoglobinuria Preceded by Aplastic Anemia: A Case Report and Family Study

This report described a patient with coexistent paroxysmal cold and nocturnal hemoglobinuria. The hemolytic processes were preceded by a periodof marrow aplasia. He demonstrated two complications of prolonged hemolysis and hemoglobinuria, namely iron deficiency and probably "relative folatedeficiency." Forty of his family members were investigated for evidence ofPNH or PCH and none was found. The need for evaluation and follow upof patients with aplastic anemia in terms of PNH was emphasized.

Submitted on April 5, 1963 Accepted on February 10, 1964