Polycythemia vera in patients of beta-thalassemia trait and stress erythropoiesis.

A 70-year-old male with a known case of beta-thalassemia trait and was on yearly follow-up was found to have a hemoglobin of 14.8 g/dL, hematocrit of 47.7%, and RBC count of 6.0 × 1012/L. Total leukocyte count (TLC) was 5 × 109/L and platelet count was 4 × 109/L. Secondary causes of polycythemia were ruled out (e.g., renal or cardiac disease and smoking). He did not have symptoms of hyperviscosity syndrome. The abdominal ultrasound showed no abnormalities. On further investigation, a JAK-2 (Exon 14) mutation was detected in this patient confirming the diagnosis of polycythemia vera (PV).

Hepatosplenic T-cell lymphoma with coexistent hepatitis B infection: a rare clinicopathologic entity.

BACKGROUND: Hepatosplenic T-cell lymphoma (HSTL) is a rare extranodal and systemic lymphoma derived from cytotoxic T cells usually of γδ T cell receptor type. It is characterized by primary extranodal disease with typical sinusoidal infiltration of liver, spleen, and bone marrow by medium-sized lymphoid cells. CASE REPORT: A 29-year-old man, with no significant prior medical history, presented with fever and massive splenomegaly. A diagnosis of HSTL was established by histologic examination and immunohistochemistry. Staging workup demonstrated bone marrow involvement by lymphoma. In addition, the patient was found to have hepatitis B infection. The association of these 2 entities has been described rarely. CONCLUSIONS: Hepatosplenic T-cell lymphoma is a distinct T cell lymphoma associated with an aggressive clinical course, a poor response to conventional treatment, and an exceedingly high mortality rate. An association of HSTL with hepatitis B as seen in the present case is exceedingly rare, with few cases reported in the literature.

After proper optimization of carvedilol dose, do different child classes of liver disease differ in terms of dose tolerance and response on a chronic basis?

BACKGROUND/AIMS: Literature regarding safe doses of carvedilol is limited, and safe doses across different Child classes of chronic liver disease are not clear. PATIENTS AND METHODS: A total of 102 consecutive cirrhotic patients with significant portal hypertension were included in this study. Hepatic venous pressure gradient was measured at baseline and 3 months after dose optimization. RESULTS: A total of 102 patients (63 males, 39 females) with a mean age of 58.3 ± 6.6 years were included. Among these patients, 42.2% had Child Class A, 31.9% had Class B, and 26.6% had Child Class C liver disease. The mean baseline hepatic venous pressure gradient was 16.75 ± 2.12 mmHg, and after dose optimization and reassessment of hepatic venous pressure gradient at 3 months, the mean reduction in the hepatic venous pressure gradient was 5.5 ± 1.7 mmHg and 2.8 ± 1.6 mmHg among responders and nonresponders respectively. The mean dose of carvedilol was higher in nonresponders (19.2 ± 5.7 mg) than responders (18.75 ± 5.1 mg). However, this difference was not statistically significant (P > 0.05). The univariate analysis determined that the absence of adverse events, the absence of ascites, and low baseline cardiac output were significantly associated with chronic response, whereas, the etiology, Child class, variceal size (large vs small), and gender were not. On multivariate analysis, the absence of any adverse event was determined to be an independent predictor of chronic response (OR 11.3, 95% CI; 1.9-67.8). CONCLUSION: The proper optimization of the dose of carvedilol, when administered chronically, may enable carvedilol treatment to achieve a greater response with minimum side effects among different Child classes of liver disease.