Haptoglobin Level in Some Diseases of Infancy and Childhood

Abstract

Summary The level of serum haptoglobin was estimated in favism, thalassemia major, acute rheumatic fever, acute glomerulonephritis, nephrotic syndrome and virus hepatitis. In favism, it was totally absent during the haemolytic episode and then started to rise till it reached normal values after 6‐8 days. Ahaptoglobinaemia was persistent in patients with Thalassemia Major before and after treatment with blood transfusion, dexamethasone administration and splenectomy. In acute rheumatic fever, acute glomerulonephritis and nephrotic syndrome, there was elevation of the haptoglobin level. This could therefore be taken as one of the criteria to evaluate activity of the disease process. The level was low in virus hepatitis and returned to normal with cure. There was a good correlation between the chronicity of the case and the diminution in haptoglobin level.

Serum Concentrations of Haptoglobin and Hemopexin in Favism and Thalassemia

“…In any case, it seems that hemolysis, when present, is so moderate in thalassemia minor, that it cannot significantly affect the Hp turnover. The fall in Hp serum level in thalassemia major [1, 3, 5-7, 9, 10] and favism [1,2] is consistent with the hemolytic state and it could be predicted that Hp depletion in the latter should be more pronounced. There is indeed no doubt that the hyperacute hemolysis which occurs in favism represents a singular condition which during the course of 24-36 h affects about 75% or more of the total erythrocyte mass.…”

Haptoglobin in Thalassemia

“…When the rate of Hp utilization exceeds that of production or release of this protein, the Hp concentration falls to levels too low to be measured. Because this glucoprotein was shown in one patient of Badr-El-Din et al [9] following infection, in 2 after milk injec tion and in our 9 cases, it is strongly suggested that ahaptoglobinemia in thalassemia is not due to the inability of the synthesis in general, but to increased consumption, as is usual in hemolytic anemia.…”

“…In one case Hp was determined only after splenectomy and in the rest only before splenectomy. The 2 patients of Badr-El-Din et al [9] were splenectomized without any rise in the Hp level due to this procedure, although it was stated that the Hb level was significantly raised in both. Since the hemolytic state per sists after splenectomy in thalassemia major, this finding is not at all surprising.…”

“…Its level is found to be low in the newborn period [18,19] and in liver diseases [17,20] and to have the effect of estrogens [21]. Although genetically determined absence of this protein is well known [22,23], in general, decrease or absence of serum Hp has been observed in chronic or acquired types of hemolytic anemias in which the Hb turnover may be more than doubled, which indicates the severity of hemolysis [1,7], Searches for Hp were made by J unnarkar [8], Badr-El-Din et al [9] and M üller-Eberhard et al [10] in 5, 7 and 3 patients with thalassemia major respectively, but none could be shown in any of them. Neither did we find Hp in the serum of 74% of our 35 thalas semia major cases.…”

“…If intra-vascular hemolysis increases, plasma Hp concentration decreases [2,3]. By starch-gel electropho resis, different kinds of Hp have been shown in the a2-fraction of the serum which are inherited genetically [4], and there is substantial evidence that its type is somewhat related to D chromosomes [5].The absence of this protein is well documented in sickle-cell anemia [6], but Hp in thalassemia has been determined in only a few cases [8][9][10]. For this reason, we looked for Hp in thalassemia major and minor.…”