Causes of death in symptomatic cryoglobulinemia: 30 years of observation in a Department of Internal Medicine.

UNLABELLED: Cryoglobulinemic Syn&come (CS) is a multi-systemic disease, and its fatal evolution can involve different organs. AIMS: To describe the most frequent causes of death in CS, by researching different evolutions between older cases and those of the last 15 years. PATIENTS: The data of 238 patients affected by symptomatic cryoglobulinemia followed by our Medicine Department in the last 30 years are recorded in a database. 54 are presently living and being followed, 70 (36F, 34M) have died. The type II/type III ratio is 5/4. We distinguish between two groups, the oldest, without any data on HCV, and the most recent who were tested for HCV. The follow-up ranges from 0.5 to 16 years. RESULTS: Liver diseases (25 cases, 9 with hepatic carcinomas), lymphomas and myeloproliferative diseases (12), and cardiovascular events (8) are the most reported causes of death. Sepsis, neurological syndromes, nephropathy, other malignancies and diffuse vasculitis are also reported. The median age at death was 67.2 years (58.4 in the oldest group, 71.9 in the other). Hepatic carcinomas are reported only after 1991. CONCLUSION: Cirrhosis complications are more frequent in patients affected by HCV. The increase in instrumental diagnostic ability and the improved survival of patients with cirrhosis account for the increase in patients with hepatic carcinomas. Improved treatment has resulted in a reduction of deaths from renal failure.

[Carbamazepine-induced agranulocytosis. Apropos of 2 cases]. / Agranulocitosi indotta da carbamazepina. A proposito di due casi.

Carbamazepine-induced agranulocytosis (CIA) is a rare event. We report on two cases, highlighting the wide variability of the bone marrow, which may show pseudohypercellularity with disappearance of neutrophils and excess of immature cells (myeloblasts and promyelocytes), thus mimicking the features of acute myeloid leukemia. Although its pathogenesis is still unclear, CIA must be considered as an idiosyncratic hemopathy and moreover it appears to be an unpredictable complication among patients taking the drug. It should be clearly distinguished from the benign neutropenia frequently associated with carbamazepine therapy and often self-limiting. Anyhow a careful clinical and hematological monitoring is the only mean to recognize promptly this life-threatening disease and to treat it with the withdrawal of the drug and the administration of an adequate anti-infectious therapy.

[2 cases of acute cholestasis caused by ticlopidine]. / Due casi di colestasi acuta da ticlopidina.

We report the case of two patients suffered from cholestatic jaundice occurred 3-4 weeks after starting ticlopidine therapy. In both cases the diagnosis was made by ruling out any other known cause of acute hepatitis or cholestasis. One patient underwent liver biopsy, which showed a typical intralobular cholestatic pattern and a slight lymphocytic infiltration of the portal tracts. The other patient, a 29 year-old woman, was taking ticlopidine as the sole drug, further to an ischemic stroke occurred while she was taking oral contraceptives; she presented a diffuse itchy dermatitis, fever and slight eosinophilia besides cholestasis. In both patients ticlopidine was discontinued and liver tests returned to normal values within 4-8 weeks; no rechallenge was attempted and ticlopidine was replaced with another antiplatelet drug. To the best of our knowledge 19 cases of ticlopidine-related cholestatic disease have been described so far in the literature. Its pathogenesis is still unknown, although some clinical findings and experimental results from patients with acute enteropathy or agranulocytosis induced by ticlopidine suggest that the drug may act through a toxic mechanism, perhaps mediated by prostaglandins.