Haptoglobin therapy for acute favism: a Japanese boy with glucose‐6‐phosphate dehydrogenase Guadalajara

Abstract

We report the case of a 2-year-old Japanese boy with acute favism who was treated with human haptoglobin products. He had been exhibiting chronic nonspherocytic haemolytic anaemia until the diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency when 14 months old. He suffered a favic crisis at 24 months of age, when the administration of haptoglobin was effective for relieving bilirubinaemia and haemoglobinuria. Serum-free Hb rapidly decreased to normal levels despite the sustained level of serum lactate dehydrogenase. His G6PD gene was G6PD Guadalajara. This is the first application of haptoglobin therapy for acute favism and the first reported case of Japanese G6PD deficiency with typical favic crisis. Haptoglobin treatment might be helpful for managing the haemolytic crisis in the disease.

New perspectives on the thrombotic complications of haemolysis

“…However, haptoglobin use in massive trauma and resuscitation in humans was studied retrospectively; unfortunately, differences in baseline characteristics between the haptoglobin-treated and untreated groups limited the conclusions of this study (Gando & Tedo, 1994). Nonetheless, haptoglobin administration seemed to be beneficial in case reports of severe ABO-incompatible haemolytic transfusion reactions (Homann et al, 1977), ABO-incompatible bone marrow transplantation (Ito et al, (Ohga et al, 1995) and burn injury with haemolysis (Imaizumi et al, 1994). However, the effect of haptoglobin on thrombotic risk in various haemolytic conditions in humans remains to be determined.…”

Haptoglobin and Free Haemoglobin during Cardiac Surgery—is there a Link to Acute Kidney Injury?

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New Therapeutic Options for the Treatment of Sickle Cell Disease

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