Pelioid-type hepatocellular carcinoma with numerous eosinophilic infiltrations in a patient with primary biliary cirrhosis.

A 65-year-old woman with liver injury was referred to our hospital in 1992. She was diagnosed with primary biliary cirrhosis (PBC) of Scheuer's histological classification stage IV. She was treated with 600 mg/day of ursodeoxycholic acid. A 1-cm mass in S7 was detected in August 1995. The serum alpha-fetoprotein (AFP) level increased to 1288 ng/mL in January 1996. Angiography showed a cotton wool-like appearance in the delayed phase. Because the size of the tumor appeared to be increasing and the serum AFP levels increased with high levels of L3 fraction, a pelioid-type hepatocellular carcinoma (HCC) was strongly suspected. Hepatic artery infusion with SMANCS and partial resection of S7 and S8 of the liver were performed in March 1996. The pathological diagnosis for theresected liver tumor was pelioid-type HCC. The serum AFP level decreased to 50 ng/mL after the operation, but relapsed HCC was detected in S6 and S7. Angiography in September 1996 revealed multiple hypervascular lesions, and hepatic artery infusion with SMANCS was again performed; however, we were unable to suppress the progression of the relapsed HCC. The patient died due to an intra-abdominal rupture of relapsed HCC and subsequent liver failure in December 1996. We report a rare case of pelioid-type HCC with numerous eosinophilic infiltrations arising from PBC.

Phase II study of chemotherapy and stem cell transplantation for adult acute lymphoblastic leukemia or lymphoblastic lymphoma: Japan Clinical Oncology Group Study 9004.

Granulocyte colony-stimulating factor (G-CSF)-supported, post-remission chemotherapy (Cx) for adult acute lymphoblastic leukemia (ALL) or lymphoblastic lymphoma (LBL) was evaluated. One hundred and forty-three eligible patients (median age, 41 years) including 126 ALL and 17 LBL receiving induction Cx (vincristine, cyclophosphamide, prednisolone [PSL], doxorubicin, L-asparaginase, intrathecal-methotrexate [IT-MTX]) were analyzed. For patients achieving complete response (CR), two courses of post-remission Cx (course A of daunorubicin, cytosine arabinoside, vindesine, PSL plus IT-MTX; course B of mitoxantrone, etoposide, vincristine, PSL plus IT-MTX) with the use of G-CSF were repeated alternately; thereafter, maintenance Cx including MTX and 6-mercaptopurine was given for 2 years. One hundred and nineteen (83%) patients achieved CR, while 14 (10%) died during induction. Among the 119 patients achieving CR, five died in remission, 76 relapsed, and the remaining 38 were alive without disease. The median survival time of the 143 eligible patients was 26 months (95% confidence interval, 19-34). At a median follow-up time of 9 years, the 5-year survival rate was 32% and the 5-year progression-free survival (PFS) rate was 26%. The 5-year survival rate of 36 patients who underwent autologous (n = 20) or allogeneic stem cell transplantation (SCT; n = 16) in the first CR group was 58%. Compared with the authors' previous trials, survival and PFS were markedly improved. In conclusion, G-CSF-supported, intensive post-remission Cx and subsequent SCT are worthy of further investigation for the treatment of adult ALL and LBL.

Sex differences in lipoprotein metabolism and dietary response: basis in hormonal differences and implications for cardiovascular disease.

The transport of fat in the blood stream is approximately twice as fast in women as men. Disease states such as obesity and diabetes are associated with greater lipoprotein abnormalities in women compared with men. A greater increment in cardiovascular disease risk in women is linked to these abnormalities. A greater change in triglyceride level and a lesser change in low-density lipoprotein are observed in women than men with high-carbohydrate or high-fat feeding. Most consistent are greater changes in high-density lipoprotein (HDL), HDL(2), and apolipoprotein A-I levels in women compared with men with high-carbohydrate or high-fat feeding. Dietary fat restriction in women appears to have a less beneficial lipoprotein effect than in men. Dietary fat restriction for heart disease prevention may be less ideal in women than in men.

Gender differences in lipoprotein metabolism and dietary response: basis in hormonal differences and implications for cardiovascular disease.

The transport of fat in the blood stream is approximately twice as fast in women as men. Disease states such as obesity and diabetes are associated with greater lipoprotein abnormalities in women compared with men. A greater increment in cardiovascular disease risk in women is linked to these abnormalities. A greater change in triglyceride level and a lesser change in low-density lipoprotein are observed in women than men with high-carbohydrate or high-fat feeding. Most consistent are greater changes in high-density lipoprotein (HDL), HDL2, and apolipoprotein A-I levels in women compared with men with high-carbohydrate or high-fat feeding. Dietary fat restriction in women appears to have a less beneficial lipoprotein effect than in men. Dietary fat restriction for heart disease prevention may be less ideal in women than in men.

Blastic natural killer cell lymphoma/leukemia (CD56-positive blastic tumor): prognostication and categorization according to anatomic sites of involvement.

BACKGROUND: Blastic natural killer (NK) cell lymphoma/leukemia (BNKL) is an immature CD56-positive neoplasm, which was recognized recently and characterized by systemic proliferation of tumor cells including skin, lymph node, and bone marrow. METHODS: The current study analyzed 47 patients with BNKL (27 had leukemias and 20 had lymphomas). Patient data were collected for the survey of the NK-Cell Tumor Study Group. RESULTS: There were 33 males and 14 females, with a median age of 53 years (range, 3 months to 89 years). There were few clinicopathologic differences between the leukemia and lymphoma types. Cutaneous involvement was noted at diagnosis in 28 patients, who presented a tendency for older age of onset (median: 56 vs. 46 years, P = 0.11) than patients with noncutaneous BNKL. Cutaneous BNKL showed less frequent mediastinal involvement (4% vs. 53%, P = 0.0002) and less severe thrombocytopenia (P =0 .03). Phenotypic characteristics were also different, with cutaneous BNKL favoring CD4 and HLA-DR expression, and noncutaneous BNKL favoring CD16 and CD34 expression. Both groups responded well to chemotherapy for lymphoid malignancies, but disease recurrence was frequent. The prognosis of patients with noncutaneous BNKL was significantly poorer than that of patients with cutaneous BNKL (median survival: 15 vs. 25 months, P = 0.02). Multivariate analysis confirmed that cutaneous involvement was a significant and independent prognostic factor for BNKL, as were age of onset and leukocyte count. CONCLUSIONS: These findings suggested that BNKL is a heterogeneous disease and contains at least two subtypes. Although further investigations are needed to settle a marker for distinction, the presence of cutaneous involvement is a useful prognostic factor.

Estrogen therapies, lipids, and the heart disease prevention controversy.

One of the most highly unexpected reports in recent medical literature was the lack of benefit of estrogen-progestin replacement therapy in cardiovascular disease prevention in postmenopausal women. The ensuing negative view of hormone replacement therapy has now extended to all forms of postmenopausal hormone treatment, including estrogen alone. Is this pessimism justified? A review of the effects of estrogens and progestins on the estrogen-sensitive systems of the body can help explain why combined oral estrogen and low-dose continuous medroxyprogesterone acetate administration may not be the paradigm for all other forms of postmenopausal hormone replacement. Some of these effects include the following: progestins are anti-estrogens, as evidenced in their divergent effects on plasma lipids; not all progestins are equal in their effect on lipids and other physiologic functions; administration of any hormone by mouth is not physiologic; giving estrogen 10 to 15 years postmenopausally may be too late to prevent atherosclerosis. On the other hand, high doses of oral estrogen/progestin in the presence of high cardiovascular risk appear to promote atherosclerosis risk. Given the current evidence the common sense answer to the question of the benefit of estrogen is "it depends." Until these and other points are formally addressed, the hypothesis that estrogen prevents heart disease remains open.

Effects of insulin resistance and obesity on lipoproteins and sensitivity to egg feeding.

OBJECTIVE: This study was undertaken to determine if insulin resistance without and with obesity influences LDL response to dietary cholesterol and saturated fat. METHODS AND RESULTS: We fed 0, 2, and 4 egg yolks per day to 197 healthy subjects in a 4-week, double-blind, randomized, crossover design. Subjects were dichotomized on body mass index (<27.5 and > or =27.5 kg/m2) and insulin sensitivity (insulin-sensitivity index > or =4.2x1.0(-4) and <4.2x1.0(-4) min(-1) microU/mL), yielding insulin-sensitive (IS, n=65), insulin-resistant (IR, n=75), and obese insulin-resistant (OIR, n=58) subjects. Mean fasting baseline LDL cholesterol (LDL-C) levels were higher in IR and OIR subjects (3.44+/-0.67 and 3.32+/-0.80 mol/L) than in IS subjects (2.84+/-0.75 mmol/L) (P<0.001). Progressive triglyceride elevations and HDL-C decreases were seen across the 3 groups. Ingesting 4 eggs daily yielded significant LDL-C increases of 7.8+/-13.7% (IS) and 3.3+/-13.2% (IR) (both P<0.05) compared with 2.4+/-12.6% for OIR (NS). HDL-C increases were 8.8+/-10.4%, 5.2+/-10.4%, and 3.6+/-9.4% in IS, IR, and OIR, respectively (all P<0.01). CONCLUSIONS: Insulin resistance without and with obesity is associated with elevated LDL-C as well as elevated triglyceride and low HDL-C. The elevated LDL-C cannot be explained by dietary sensitivity, because the LDL-C rise with egg feeding is less in IR persons regardless of obesity status, probably attributable to diminished cholesterol absorption. The results suggest that dietary management of insulin resistance and obesity can focus more on restricting calories and less on restricting dietary fat.

Management of patients with diabetic hyperlipidemia.

Hyperlipidemia is commonly observed in patients with type 2 diabetes and is also characteristic of the metabolic syndrome. We discuss the lipoprotein abnormalities in type 2 diabetes and the relation of triglyceride, low-density lipoprotein cholesterol, and high-density lipoprotein cholesterol to insulin resistance and diabetes. We also present a case study of a diabetic woman with hyperlipidemia and coronary artery disease.

The severity of functional mitral regurgitation depends on the shape of the mitral apparatus: a three-dimensional echo analysis.

BACKGROUND AND AIMS OF THE STUDY: The relationship between structural abnormalities of the mitral apparatus and severity of functional mitral regurgitation (MR) was examined in patients with non-ischemic dilated cardiomyopathy. METHODS: Three-dimensional (3D) echocardiography was performed in 13 cardiomyopathy patients with mild (n = 5) or moderate to severe (n = 8) MR, and in eight normal volunteers using freehand scanning. The size, shape and function of the left ventricle, and the dimensions of the mitral annulus, chordae tendineae and papillary muscles, were measured. RESULTS: Virtually all parameters differed significantly between normal subjects and cardiomyopathy patients. Annular size, the central angle between the anterior and posterior chordae, and dilatation of the anterior and anterolateral left ventricular (LV) wall were greater in patients with moderate to severe MR than in those with trace to mild MR. Anterior wall dilatation was associated with greater outward displacement of the anterior papillary muscle, and correlated with widening of the central chordal angle, apical displacement of the mitral leaflet coaptation point, and mitral annular dilatation. CONCLUSION: In non-ischemic dilated cardiomyopathy, functional MR is related to both annular dilatation and to dilatation of the anterior and anterolateral LV wall. The latter results in displacement of the anterior papillary muscle, narrowing of the angle of the anterior chorda to the mitral annulus, and widening of the central angle between the anterior and posterior chordae.

[An elderly non-Hodgkin lymphoma patient with a massive tumor of the heart].

We report on an elderly patient with a malignant lymphoma forming a huge mass in the heart. An 82-year-old woman became aware of general fatigue and a cough in August 1999. Her right supraclavicular, bilateral axillary, and right inguinal lymph nodes were swollen. A hypodermical mass in the right frontal chest was detected. Her left axillary lymph node was biopsied. She was diagnosed as having non-Hodgkin lymphoma, diffuse large cell type, B-cell type. Computed tomography scans showed a markedly thickened right ventricular wall of the heart, swollen lymph nodes of the mediastinum, bilateral pleural effusions, and a tumor in the spleen. Lymphoma cells were found in the pleural effusion, and the lymphoma was diagnosed as clinical stage IV. Hypofunction of the heart, ejection fraction (EF) 49%, was demonstrated with transthoracic echocardiography. EF increased to 70% after 3 courses of chemotherapy with CHOP regimen. All lesions disappeared after 6 courses of chemotherapy were completed. After consolidative radiotherapy with a total dose of 37 Gy to the mediastinum and heart, bilateral pleural effusions, elevation of the patient's lactate dehydrogenase level and soluble IL-2 receptor value were recognized, which suggested relapse of the lymphoma, although histopathological confirmation could not be realized.