We report the case of a 40-yr-old man presenting with symptoms of small bowel obstruction. Small bowel x-rays revealed a stricture of the mid-jejunum. Push enteroscopy found a polypoid mass at 1 ...meter of the ligament of Treitz. Histopathological examination of the biopsy and surgical specimens showed a diffuse infiltrate of the mucosa made of medium to large cells, which were stained on immunohistochemistry by the leucocyte marker CD45 and the histiocyte/monocyte marker CD68 but were negative for the B and T cell markers. Cytological examination of the ascitic fluid revealed many myelobasts with cytoplasmic Auer rods and positive myeloperoxidase staining. There was no evidence of blood or bone marrow involvement suggestive of acute leukemia or myeloproliferative disorders. These findings were consistent with the diagnosis of preleukemic granulocytic sarcoma (or chloroma). Chemotherapy led to complete remission, but 21 months later the patient developed an acute myeloid leukemia. He died from aspergillus pneumonitis, 10 months after bone marrow allograft. Preleukemic granulocytic sarcoma of the small bowel is a rare condition and its diagnosis is usually not easy, requiring histochemical or immunohistochemical studies. Most cases have progressed to acute myeloid leukemia.
Primary biliary cholangitis Soret, Pierre-Antoine; Chazouillères, Olivier; Corpechot, Christophe
La Revue du praticien
71, Issue:
8
Magazine Article
PRIMARY BILIARY CHOLANGITIS Primary biliary cholangitis (formally primary biliary cirrhosis, PBC) is the most common chronic cholestatic liver disease in humans. It is a presumed autoimmune liver ...disease, characterized by inflammation and progressive destruction of small bile ducts. Without treatment, PBC progresses towards liver fibrosis and, ultimately, cirrhosis. Women older than 40 are more likely affected. Pruritus and tiredness are the most common symptoms but they are frequently lacking. Diagnosis is made by the association of chronic biochemical features of cholestasis (parallel increase in ALP and GGT) and presence of specific auto-antibodies (particularly M2 anti-mitochondrial antibodies). Ursodeoxycholic acid (UDCA), which is the standard of care treatment for PBC, has dramatically improved the prognosis of the disease. However, 30 % to 40 % of patients have an inadequate biochemical response to UDCA and continue to be at high-risk of complications. In this situation, second-line treatments, including obeticholic acid or fibrates, should be considered in association with UDCA.
Background and Aims
Autoimmune hepatitis (AIH) is a rare indication (<5%) for liver transplantation (LT). The aim of this study was to describe the early outcome after LT for AIH.
Methods
A ...multicenter retrospective nationwide study including all patients aged ≥16 transplanted for AIH in France was conducted. Occurrences of biliary and vascular complications, rejection, sepsis, retransplantation and death were collected during the first year after LT.
Results
A total of 344 patients (78.8% of women, 17.0% of (sub)fulminant hepatitis and 19.2% of chronic liver diseases transplanted in the context of acute‐on‐chronic liver failure ACLF) were included, with a median age at LT of 43.6 years. Acute rejection, sepsis, biliary and vascular complications occurred in respectively 23.5%, 44.2%, 25.3% and 17.4% of patients during the first year after LT. One‐year graft and patient survivals were 84.3% and 88.0% respectively. The main cause of early death was sepsis. Pre‐LT immunosuppression was not associated with an increased risk for early infections or surgical complications. Significant risk factors for septic events were LT in the context of (sub)fulminant hepatitis or ACLF, acute kidney injury at the time of LT (AKI) and occurrence of biliary complications after LT. AKI was the only independent factor associated with graft (HR = 2.5; 95% CI: 1.1–5.4; p = .02) and patient survivals (HR = 2.6; 95% CI: 1.0–6.5; p = .04).
Conclusion
Early prognosis is good after LT for AIH and is not impacted by pre‐LT immunosuppression but by the presence of AKI at the time of LT.
