Risk of secondary osteoporosis due to lobular cholestasis in non-cirrhotic primary biliary cholangitis.

BACKGROUND AND AIM: It remains unclear whether primary biliary cholangitis (PBC) represents a risk factor for secondary osteoporosis. METHODS: A case-control study was conducted to examine bone mineral density and bone turnover markers in middle-aged postmenopausal PBC patients without liver cirrhosis. We compared the incidence of low bone mineral density between propensity-score matched subgroups of PBC patients and healthy controls and investigated the mechanisms underlying unbalanced bone turnover in terms of the associations between bone turnover markers and PBC-specific histological findings. RESULT: Our analysis included 128 consecutive PBC patients, all postmenopausal women aged in their 50s or 60s, without liver cirrhosis or fragility fracture at the time of PBC diagnosis. The prevalence of osteoporosis was significantly higher in the PBC group than in the control group (26% vs 10%, P = 0.015, the Fisher exact probability test). In most PBC patients (95%), the level of bone-specific alkaline phosphatase was above the normal range, indicating increased bone formation. On the other hand, the urine type I collagen-cross-linked N-telopeptide showed variable levels among our PBC patients, indicating unbalanced bone resorption. Advanced fibrosis was associated with low bone turnover. Lobular cholestasis, evaluated as aberrant keratin 7 expression in hepatocytes, showed significant negative correlations with bone formation and resorption, indicating low bone turnover. CONCLUSION: Our results show that, compared with healthy controls, even non-cirrhotic PBC patients have significantly higher risk of osteoporosis. Moreover, lobular cholestasis was associated with low bone turnover, suggesting this feature of PBC may itself cause secondary osteoporosis in PBC patients.

Primary Duodenal Follicular Lymphoma Treated With Rituximab Monotherapy and Followed-up for 15 Years.

A 41-year-old woman was diagnosed with duodenal follicular lymphoma. She had no other lesions and was assigned to a "watch and wait" policy. Swelling of the inguinal lymph nodes appeared 45 months later, and rituximab monotherapy resulted in complete remission. However, follicular lymphoma recurred in the stomach, rectum and mesenteric and external iliac lymph nodes 81 months after the therapy. The patient received rituximab monotherapy again and has remained in complete remission in the fifteenth year after the initial diagnosis. This case suggests the usefulness of rituximab monotherapy in the long-term management of intestinal follicular lymphoma.

[A case of primary micropapillary carcinoma of the jejunum].

Micropapillary carcinoma (MPC) has been recently reported as a variant of invasive breast carcinoma. MPC is also known to be an aggressive variant of adenocarcinoma, and it is associated with poor prognosis and a high propensity for lymphovascular invasion and lymph node metastases. MPC of the breast, urinary bladder, and lung has been reported relatively frequently; however, there have been few reports on gastrointestinal MPC. Furthermore, MPC of the small bowel has not been reported yet. Here we report a case of MPC of the jejunum. A female septuagenarian was admitted because of anorexia and weight loss. We established a diagnosis of primary jejunal cancer and resected the duodenum and a part of the jejunum. Histologically, the tumor consisted of adenocarcinoma with moderate to poor differentiation. Among the carcinoma cells, approximately 10% formed small papillary neoplastic cell clusters surrounded by clear spaces, suggesting a diagnosis of MPC. We present the details of the case along with a review of relevant literature.