[Hemolysis and schizocytosis, malabsorption and the "folate trap": unusual semiological peculiarities associated with vitamin B12 deficiency]. / Hémolyse et schizocytose, malabsorption et "piège à folates": à propos de particularités sémiologiques mal connues des carences en vitamine B12.

INTRODUCTION: Hemolysis and red cell fragmentation accompanying vitamin B12 deficiency may misdirect the diagnosis. Signs of malabsorption and abnormalities related to folic acid metabolism characterized by discrepancies between folic acid normal serum levels and erythrocytic folic acid levels may also exist. EXEGESIS: We report the occurrence of hemolysis and red cell fragmentation mimicking microangiopathic hemolytic anemia, malabsorption and folic acid deficiency in the course of vitamin B12 deficiency. Appropriate replacement therapy corrected all abnormalities. CONCLUSION: An association between hemolysis, malabsorption and folic acid deficiency should lead physicians to search for signs of vitamin B12 deficiency.

[Autoimmune hepatitis and hepatitis C]. / Hépatites auto-immunes et infection par le virus de l'hépatite C.

From autoimmune hepatitis (AIH) classification which recognizes three types of AIH, we discuss the main relations between hepatitis C virus (HCV) infection and AIH. Type I AIH is associated with antinuclear and antismooth muscle antibodies, and with other autoimmune diseases. There is no relation between type I AIH and HCV. Type I anti-liver kidney microsome and anti-liver cytosol I antibodies represent the hallmark of type II AIH. Among type II AIH, two subgroups emerged: type IIa AIH (10-40%) are true AIH (sensitive to steroids but worsens with interferon alpha), whereas type IIb AIH (60-90%) appear as a particular form of HCV hepatitis. Type IIb AIH have a moderate activity, a low titer of autoantibodies, anti-GOR antibodies but never anti-liver cytosol I, no sensitivity to steroids but are sensitive to interferon alpha. The hallmark of type III AIH are anti-cytosol antibodies, but these AIH have the same characteristics as type I AIH. The classification between true AIH (I, IIa, III) or "pseudo-AIH" due to HCV infection has major therapeutic implications. Steroids or immunosuppressive treatments are effective in type I, IIa and III AIH but have no efficacy in type IIb AIH. Alpha interferon has an efficacy in type IIb AIH, but it has no efficacy and may even worsen hepatitis in type I, IIa and III AIH.

[Value of the protein profile in the diagnosis of infectious endocarditis when hemolysis is present]. / Apport du profil protéique pour le diagnostic d'endocardite infectieuse quand il révèle une hémolyse.

PURPOSE: Haptoglobin (H) and orosomucoid (O) are acute phase proteins that increase in a parallel manner. When hemolysis and inflammation are both present, study of the O-H couple on the protein profile may reveal an unknown hemolysis. METHODS: To determine if hemolysis is more frequent during infectious endocarditis than during septicemia without valvulopathy or during valvulopathy without septicemia. Study of three groups of patients: 26 patients with infectious endocarditis, 13 patients with septicemia and 36 patients with valvulopathy without septicemia. Studied parameters were the O-H couple, hemoglobin and rate of O. RESULTS: Hemolysis is clear in patients with endocarditis. The difference O-H is significantly more important during endocarditis than during septicemia without valvulopathy (P < 0.001) and during valvulopathy without sepsis (P < 0.001). CONCLUSION: Study of the O-H couple may be useful for the diagnosis of endocarditis showing a difficult-to-diagnose hemolysis.

Zidovudine-induced mitochondrial disorder with massive liver steatosis, myopathy, lactic acidosis, and mitochondrial DNA depletion.

Zidovudine is known to be responsible for a mitochondrial myopathy with ragged-red fibres and mitochondrial DNA depletion in muscle. Lactic acidosis alone or associated with hepatic abnormalities has also been reported. A single report mentioned the concomitant occurrence of muscular and hepatic disturbances and lactic acidosis in a patient receiving zidovudine, but muscle and liver tissues were not studied. A 57-year-old man with AIDS, who had been treated with zidovudine for 3 years, developed fatigue and weight loss. Serum creatine kinase and hepatic enzyme levels were high. Lactic acidosis was present. Liver biopsy showed diffuse macrovacuolar and microvacuolar steatosis. After withdrawal of zidovudine, creatine kinase, aspartate aminotransferase, and alanine aminotransferase levels normalised within 5 days, and lactacidaemia decreased. Acidosis persisted. The patient became confused and febrile and died 8 days after detection of high blood lactic acid. A muscle sample obtained at autopsy showed mitochondrial abnormalities with ragged-red fibres and lipid droplet accumulation. Southern blot analysis showed depletion of mitochondrial DNA, affecting skeletal muscle and liver tissue. No depletion was found in myocardium and kidney. This case emphasises that zidovudine treatment can induce mitochondrial multisystem disease, as revealed in our case by myopathy, liver steatosis and lactic acidosis.