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E S Zafrani

Publications and source records attributed to E S Zafrani.

At least 19 recordsLinked to original sources
PubMed1992

Multiple nuclear dots antinuclear antibodies are not specific for primary biliary cirrhosis.

Multiple nuclear dots antinuclear antibodies display a specific immunofluorescence pattern on HEp-2 cells. They have been reported to be strongly associated with primary biliary cirrhosis, especially when sicca syndrome was present. To determine whether multiple nuclear dots antinuclear antibodies are specific for primary biliary cirrhosis, we studied the clinical, biochemical, immunological and morphological features of 38 patients between December 1983 and September 1990 who had serum multiple nuclear dots antinuclear antibodies detected in an immunology laboratory of a large medical center. Sufficient information was reliable in 36 patients; the group included 31 women and 5 men (mean age = 57.6 +/- 14.5, range = 30 to 87). Fifteen patients (42%) had primary biliary cirrhosis, 5 patients (14%) had type 1 autoimmune chronic active hepatitis, 4 patients (11%) had liver disease of unknown cause and 12 patients (33%) had various immunological disorders but no liver disease. Two of the patients with primary biliary cirrhosis (13%) had clinical sicca syndrome. Our study demonstrates the following: (a) serum multiple nuclear dots antinuclear antibodies are not specific for liver disease because they can be observed in one third of patients with various immunological disorders without liver involvement, and (b) serum multiple nuclear dots antinuclear antibodies are not specific for PBC because they can also be observed in type 1 autoimmune chronic active hepatitis. Our results also suggest that patent sicca syndrome is abnormally present in patients with primary biliary cirrhosis and multiple nuclear dots antinuclear antibodies.

Adult↗
PubMed1992

Cutaneous lymphadenoma with focal mucinosis.

Cutaneous lymphadenoma of the eyebrow occurred in a 72-year-old man. The nodular tumor was well circumscribed but non encapsulated and invaded the reticular dermis and superficial hypodermis without any connection with the epidermis. Tumor cells had a basal-like appearance and were arranged in small lobules and islands with peripheral palisading and areas of central keratinization. They were often dissociated by an extracellular mucinous material. The stroma was fibroblastic and contained a marked lymphoid infiltrate that surrounded and penetrated the neoplastic lobules. Occasional germ-like hair follicle structures were noted. The immunohistochemical pattern was similar to that of hair follicles, and the leucocytic component was predominantly composed of T-lymphocytes and Langerhans cells, which were specially numerous within the neoplastic lobules. We believe that the tumor, in the present case, has morphologic features consistent with epithelial and stromal attributes observed in hair follicles.

Aged↗
PubMed1991

Bone marrow histologic and immunohistochemical findings in peripheral. T-cell lymphoma: A study of 38 cases.

The histologic and immunohistochemical findings in bone marrow (BM) biopsies from 38 patients with peripheral T-cell lymphoma (PTCL) are reported. Routine light microscopy showed that BM involvement was unequivocal in 12 cases and questionable in 14 cases. There was no histologic evidence of lymphoma in the remaining 12 cases. Immunohistochemistry performed on BM frozen sections demonstrated the T-cell origin of the infiltrating lymphoid cells in 24 of the 26 patients with unequivocal or questionable involvement. The malignant nature of these cells was suggested by demonstration of an aberrant T-cell phenotype identical to that observed in the other sites of involvement. In addition, in four of the 12 cases with apparently normal BM at routine light microscopy, immunohistochemistry revealed a minimal but phenotypically abnormal T-cell population, suggesting mild infiltration by lymphoma. These combined histologic and immunohistochemical data documented a high incidence (73%) of BM involvement by PTCL. In addition, a very peculiar sinusal pattern of BM involvement was found in five patients who presented an unusual type of hepatosplenic T-cell lymphoma expressing the gamma delta T-cell receptor. The present study demonstrates the high incidence of BM involvement by PTCL and emphasizes the value of frozen section immunohistochemistry to establish this diagnosis, especially when routine light microscopy findings are questionable.

Adolescent↗
PubMed1991

[Nodular regenerative hyperplasia of the liver associated with azathioprine therapy].

Three cases of nodular regenerative hyperplasia of the liver associated with azathioprine therapy are reported. The indication of azathioprine differed in each of the 3 cases including kidney transplantation, graft-versus-host disease following bone marrow transplantation, and suspicion of bowel inflammatory disease, respectively. In all three patients, nodular regenerative hyperplasia was discovered after a prolonged administration of azathioprine (24 to 40 months), with a cumulative dose of 52 to 120 g. Under light microscopy, vascular lesions were associated with nodular regenerative hyperplasia in the 3 cases and consisted of sinusoidal dilatation (2 cases), perisinusoidal fibrosis (2 cases), and peliosis (1 case). In two patients, nodular regenerative hyperplasia was responsible for severe portal hypertension which was treated by portacaval shunt. These findings are strongly suggestive of a role of azathioprine in the occurrence of nodular regenerative hyperplasia. The mechanism of azathioprine-induced nodular regenerative hyperplasia could be related to sinusoidal lesions caused by azathioprine and responsible for liver hypoperfusion, with regenerative hyperplasia in the areas remaining normally perfused. Patients receiving long-term therapy with azathioprine should be followed-up regularly and liver biopsy should be performed when clinical or biochemical liver abnormalities are observed.

Adult↗
PubMed1991

[Hepatic cholesterol ester storage disease. Two new cases diagnosed in adults].

Cholesterol ester storage disease is a rare disorder characterized by an hereditary deficiency of lysosomal acid lipase that induces an accumulation of cholesterol ester in most tissues of the body, particularly in liver. The diagnosis is usually made during childhood. The aim of this article is to report two new cases diagnosed in adult age. Two patients, 25 and 20 years old, respectively, presented with hepatomegaly, a slight to moderate increase in serum transaminases, and esophageal varices. In both cases, diagnosis was based on the presence of hypercholesterolemia, fatty infiltration of the liver with lipid droplets in hepatic parenchymal cells, foamy macrophages, hepatic storage of cholesterol esters, and low activity of lysosomal acid lipase. Histological abnormalities were associated with portal and periportal fibrosis in one patient and a micronodular cirrhosis in the other; these lesions were probably the cause of portal hypertension. Fibrosis of varied degrees has been previously reported in cholesterol ester storage disease. Its mechanism remains unclear.

Adult↗
PubMed1990

Mechanism of gamma-glutamyl transpeptidase release in serum during intrahepatic and extrahepatic cholestasis in the rat: a histochemical, biochemical and molecular approach.

The mechanism of the elevation of serum gamma-glutamyl transpeptidase activity in cholestasis is not clear. We therefore analyzed rat gamma-glutamyl transpeptidase activities in liver, bile and serum during intrahepatic cholestasis induced by a single dose of alpha-naphthyl isothiocyanate (20 mg/100 gm body weight) and during extrahepatic cholestasis after bile duct ligation. At days 1 and 2 after alpha-naphthyl isothiocyanate ingestion, we saw a fivefold and a 60-fold increase in serum and bile gamma-glutamyl transpeptidase activities, respectively. These increases were associated with a decrease in hepatic gamma-glutamyl transpeptidase activity and of corresponding mRNA. Simultaneously, necrosis of the biliary epithelium appeared in portal tracts. From day 2 to day 14, gamma-glutamyl transpeptidase activity in bile and serum progressively returned to basal levels; in the liver, cholangiolar proliferation was mild and was associated with moderate elevation of the gamma-glutamyl transpeptidase activity and of its corresponding mRNA. In extrahepatic cholestasis, a 10-fold increase in serum gamma-glutamyl transpeptidase activity was detected between day 0 and day 14. This increase was associated with major cholangiolar proliferation and with a progressive rise in hepatic gamma-glutamyl transpeptidase activity and in specific mRNA; in bile, gamma-glutamyl transpeptidase activity was slightly elevated. In these two models of cholestasis, histochemically detected gamma-glutamyl transpeptidase activity was largely predominant in biliary cells. We found no significant induction of gamma-glutamyl transpeptidase activity in hepatocytes. These results suggest that in these two models of cholestasis, the increase in serum gamma-glutamyl transpeptidase activity is of biliary cell origin and does not originate from hepatocytes.(ABSTRACT TRUNCATED AT 250 WORDS)

1-Naphthylisothiocyanate↗
PubMed1990

Idiopathic biliary ductopenia in adults: a report of five cases.

The clinical and pathological findings of five adult cases of idiopathic nonsyndromatic paucity of interlobular bile ducts are reported. Patients were 18-32 years old at the onset of the disease; four presented with pruritus and/or jaundice and one with bleeding of the esophageal varices. Two patients were siblings. Serum alkaline phosphatase counts ranged from 1 to 16 times the upper normal value, and total bilirubin counts ranged from 0.6 to 8.8 mg/dL (10 to 150 mumol/L). Initial liver biopsy showed portal and periportal fibrosis with cholangiolar proliferation and reduction in the number of interlobular bile ducts. Antimitochondrial antibodies were absent, and bile ducts were normal after opacification. The patients were observed for 3-11 years. Repeated liver biopsies in the five patients showed progression of the lesions, with development of biliary type cirrhosis in four. Two of the four patients with cirrhosis died of hepatic failure 3 and 11 years after onset of the disease. In the two other cases, liver transplantation was performed successfully. These cases suggest that chronic cholestasis with marked ductopenia resembling the nonsyndromatic paucity described in infancy and childhood may reveal itself at an adult age. This disorder, possibly familial, may rapidly progress to severe and even fatal liver disease and could be a new indication for liver transplantation.

Adolescent↗
PubMed1990

Cystic dilatation of intrahepatic bile ducts in primary sclerosing cholangitis.

A case of primary sclerosing cholangitis associated with cystic dilatations of intrahepatic bile ducts simulating Caroli's disease is described. The diagnosis of primary sclerosing cholangitis was based upon cholangiogram features, liver histologic examination and the association with chronic ulcerative colitis. It may be suggested that the cystic dilatation of intrahepatic bile duct represents an extreme form of the usual mild dilatations (cholangiectases) described in primary sclerosing cholangitis. We suggest that cystic dilatation of intrahepatic bile ducts could be included among the radiologic features of this disease.

Bile Duct Diseases↗
PubMed1990

Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas.

Fifty-seven cases of peripheral T-cell lymphoma were studied for cell expression of the T-cell receptor (TCR) chains, using monoclonal antibodies specific for the beta chain (beta F1) of the alpha/beta TCR, and for the delta chain (anti-TCR delta-1) of the gamma/delta TCR. Three different patterns were demonstrated: in 39 cases (69%), the phenotype (CD3+beta F1+TCR delta-1-) was that of most normal T cells. A second pattern was found on six cases (10%), which were of CD3+beta F1-TCR delta-1+ phenotype, and in which DNA analysis showed a clonal rearrangement of the delta locus in the five cases studied. It is suggested that these cases are the neoplastic counterpart of the small subpopulation of normal T cells that express gamma delta receptor. It is of considerable interest that these gamma delta lymphomas had unusual clinicopathologic presentations, as one case corresponded to a lethal midline granuloma and the five others to hepatosplenic lymphomas with a sinusal/sinusoidal infiltration in spleen, marrow, and liver. The fact that the distribution of the neoplastic gamma delta cells in the splenic red pulp resembles that of normal gamma delta cells reinforces the concept of a preferential homing of gamma delta T cells to this tissue. A third pattern (CD3 +/- beta F1-TCR delta-1-) was seen in 12 cases (21%), in which, by contrast to normal post-thymic T cells, no evidence of either alpha beta or gamma delta T cell receptor was found.

Antibodies, Monoclonal↗
PubMed1989

Association of focal nodular hyperplasia and hepatic hemangioma.

The association between hepatic hemangioma and focal nodular hyperplasia has been occasionally reported. To evaluate the reality of this association, the prevalence of hemangioma was studied in 26 patients with focal nodular hyperplasia by comparison with 27 patients with hepatocellular adenoma. All of these patients underwent surgery, which provided the histologic confirmation of the type of tumor. The presence of hemangioma was assessed by preoperative ultrasonography, dynamic computed tomography, and pathological examination of resected liver specimens. No hemangioma was found in patients with hepatocellular adenoma, but 6 of the 26 patients with focal nodular hyperplasia (23%) had one or two associated hemangiomas that varied in size from 1.5 to 4 cm. All 6 patients having focal nodular hyperplasia and hemangioma were women who had previously used oral contraceptives. None of the patients who had not used oral contraceptives had this association. Moreover, oral contraceptives were taken for a significantly longer period by the women with focal nodular hyperplasia and hemangioma than by those without this association. It is concluded that (a) the association of hemangioma with focal nodular hyperplasia is frequent (23%) and not fortuitous, and (b) prolonged administration of oral contraceptives may facilitate the recognition of this association, possibly by affecting the growth of these tumors.

Adult↗
PubMed1989

Peripheral neuropathy, coagulopathy and nodular regenerative hyperplasia of the liver in a patient with multiple serologic auto-antibody activities and IgM B-cell lymphoma.

A patient with IgM-secreting B-cell lymphoma developed demyelinating peripheral neuropathy, nodular regenerative hyperplasia of the liver, coagulopathy, and terminal miliary lymphomatous spreading. Serologic auto-antibody activities directed against myelin associated glycoprotein, prothrombinase complex (i.e. lupus anticoagulant) and smooth muscle cells were detected. It is suggested that there is a link between the IgM gammopathy, the immunological abnormalities and the various clinico-pathological conditions.

Autoantibodies↗