A 50-year-old woman presented with elevated transaminase levels (AST 133 IU/l and ALT 337 IU/l), GGT 1624 IU/l and AF 1,123 IU/l.
Coagulation studies and unaltered blood count, ESR 68 mm and polyclonal hypergammaglobulinemia due to IgM deficiency (1470 mg/dl) and IgG (2260 mg/dl).
Symptoms were dry oropharynx mucosa and occasional pruritus.
The physical examination showed no hepatomegaly or enlarged spleen.
Antimitochondrial antibodies (AMA) 1/1,280 and antinuclear antibodies (ANA) 1/640, with rheumatoid factor, thyroid antibodies, anti-MS, anti-SCM-70 negative, anti-SCMSB.
Normal thyroid hormones
A Schirmer test was compatible with the diagnosis of "sicca" syndrome.
Abdominal ultrasound was normal.
When PBC was suspected, a liver biopsy confirmed the diagnosis in Scheuer stage I/II.
Treatment was initiated with ursodeoxycholic acid (AUDC) 15 mg/kg/day with progressive improvement of laboratory abnormalities, presenting in subsequent controls ALT 71 IU/l, AST 44 IU/l, FAGT 200
Five months later she came to the outpatient clinic complaining of progressive asthenia, tinnitus and dark urine without fever of 10 days duration.
There was no history of drug intake.
In the exploration, severe mental weakness with sub-chronic cough and polysystolic murmur grade IV/VI.
Laboratory tests showed hemoglobin 7.1 g/dl, MCV 118, 10.900 leukocytes/mm3 and normal leukocyte formula.
Reticulocytes 1
The morphology was polychromatophilia, spherocytosis, anisopoycylocytosis, basophilic punctate and isolated dacrocytes without schistocytes.
Direct testing of Coombs +4 for IgG and C3d and indirect positive study I, II with immunoassay compatible with the presence of autoantibody with possible anti-e specificity.
Hyperbilirubinaemia 56.1 mmol/l with indirect predominance (BD 49.3 mmol/dl), LDH 1.164 IU/l, ALT 51 IU/l, AST 42 IU/L, GGT 135 IU/l).
ESR: 129 mm. Serological study for HIV, HCV, HBV, EBV, CMV, VHZ, ESR, Mycoplasma pneumoniae and syphilis negative.
Tumor markers CEA, Ca 12.5, Ca 19.9, alpha fetoprotein, Ca 15.3 and TPS in normal ranges.
Total computerized tomography with i.v. contrast and oral contrast showed moderate homogeneous enlargement of the body, with no adenomegaly or portal hypertension data.
Bone densitometry was normal.
With the diagnosis of severe AHAI associated with PBC, steroid treatment was initiated with prednisone 1 mg/kg/day, calcium 1 g/day, and ferritin 16,000 IU twice a week.
The administration of UDCA was maintained.
Given the high risk associated with AIHA, prophylaxis with low molecular weight heparin has been administered.
After three weeks of treatment there was a progressive improvement in hemoglobin levels and other hemolysis data, which were sent home.
After two months of outpatient follow-up she has 11.3 g/dl of Hb, maintaining steroid treatment at initial doses.
