Two anhaptoglobinemic patients showing anaphylactic transfusion reactions by antihaptoglobin antibody were found. Southern blot analysis indicated that 2 patients were homozygous for the deleted allele of the haptoglobin gene (Hpdel) as reported previously. We have identified the junction region of the deletion from genomic DNA of 1 patient using cassette-mediated polymerase chain reaction (PCR). Then, the deleted region from the 5′ breakpoint to the promoter region of the Hpwas amplified from genomic DNA of a control individual using PCR. DNA sequence analysis of these regions indicated that the 5′ breakpoint of the Hpdel allele was located 5.2 kilobase (kb) upstream of exon 1 of the Hp and the 3′ breakpoint was positioned between 52 and 53 base pair (bp) upstream of exon 5 of the haptoglobin-related gene. There was no significant homology between the DNA sequences flanking the 5′ and 3′ breakpoints, except for a 2-bp (TG) identity. To examine the gene frequency, we have developed a simple PCR method to detect the gene deletion. We found 8, 16, and 17 Hpdelalleles in 157 Koreans, 523 Japanese, and in 284 Chinese, respectively, but did not find the Hpdel in 101 Africans or in 100 European-Africans. The incidence of individuals homozygous for the Hpdel allele was therefore expected to be 1/4000 in Japanese, 1/1500 in Koreans, and 1/1000 in Chinese. This incidence is higher than that of IgA deficiency in Japanese. More attention should be paid on haptoglobin deficiency and antihaptoglobin antibody as the cause of transfusion-related anaphylactic reactions in Asian populations.

Anaphylactic NHTRs in these patients with haptoglobin deficiency associated with serum haptoglobin antibodies were suggested to be prevalent in Japan. In addition to IgG antibodies, IgE haptoglobin antibodies detected in the sera of such patients were suggested to play a role in the occurrence of the reactions.

Simply by increasing FA to 90 degrees in 3D-DESS imaging, the contrast between cartilage and synovial fluid increased substantially. Subtle cartilage lesions may thus be detectable using 3D-DESS sequences.

Experimental hepatic fibrosis was produced in the guinea pig. We produced hepatic necrosis associated with inflammatory cell infiltration in guinea pigs immunized with acetaldehyde adducts and fed ethanol for 40 days. Extending the period of these treatments to 90 days resulted in producing hepatic fibrosis developing around individual hepatocytes in the terminal hepatic venule areas and portal areas, accompanied by an increase in hepatic hydroxyproline content. In contrast, no fibrosis was observed in the livers of the control groups that had been exposed to nothing, ethanol alone, or a combination of ethanol and immunization with unmodified human hemoglobin. Minimal fibrotic changes were observed in animals immunized with human hemoglobin acetaldehyde adducts but not fed ethanol. These results indicate that the formation of acetaldehyde adducts and the acquisition of immunity against them can produce hepatic fibrosis. Immune mechanisms against acetaldehyde adducts may, in part, be involved in the pathogenesis of hepatic fibrosis seen in alcoholics.