Hereditary hemolytic anemia glucose-6-phosphate dehydrogenase deficiency

Hemolytic anemia results from decreased RBC survival time due to destruction in the spleen or circulation. The most common etiologies are RBC membrane defects (e.g., hereditary spherocytosis), altered Hb solubility or stability (e.g., sickle cell anemia [see Chap. 34] and thalassemias), and changes in intracellular metabolism (e.g., glucose-6-phosphate dehydrogenase deficiency). Some drugs cause direct oxidative damage to RBCs (see Appendix 3). 

Deficiencies of any of these red cell enzymes may result in impaired ATP generation and consequently loss of function of the erythrocyte. By far the majority of these disorders are hereditary in nature, although acquired deficiencies have been described, mainly in malignant disorders involving the bone marrow. Hereditary enzymatic defects in these pathways disturb the erythrocyte s integrity, shorten its cellular survival, and produce chronic nonspherocytic hemolytic anemia (CNSHA). Deficiencies of some enzymes, however, do not lead to chronic hemolytic anemia, but to acute episodes of severe hemolysis when there is increased oxidative stress on the red cell (as in some types of glucose-6-phosphate dehydrogenase deficiency). 

In children with glucose-6-phosphate dehydrogenase (G6PD) deficiency, a hereditary disease also known as favism, hemolytic anemia and hyperbilirubinemia have been reported after topical application of henna (Devecioglu et al. 2001 Kandil et al. 1996 Kok et al. 2004 Raupp et al. 2001 Zinkham and Oski 1996). In some cases, the patients also developed acute renal failure, sometimes fatal (Devecioglu et al. 2001 Kok et al. 2004). No case reports for such adverse effects in adults were identified after topical application. Relative thickness of skin and higher body mass to skin surface area ratio may account for a lack of case reports in adults. 

---