The effectiveness of antiplatelet therapy as primary prophylaxis for thrombosis in low-risk essential thrombocythemia (ET) is not proven. In this study, the incidence rates of arterial and venous thrombosis were retrospectively analyzed in 300 lowrisk patients with ET treated with antiplatelet drugs as monotherapy (n ‫؍‬ 198) or followed with careful observation (n ‫؍‬ 102).

The frequency of vascular events and evolution to myelofibrosis (MF) in young individuals with essential thrombocythemia (ET) is not well known. The incidence and predisposing factors to such complications was studied in 126 subjects diagnosed with ET at a median age of 31 years (range: 5-40). Overall survival and probability of survival free of thrombosis, bleeding and MF were analyzed by the Kaplan-Meier method and the presence of the Janus Kinase 2 (JAK2) V617F mutation correlated with the appearance of such complications. The JAK2 mutation (present in 43% of patients) was associated with higher hemoglobin (Hb) (Po0.001) and lower platelets at diagnosis. With a median follow-up of 10 years (range: 4-25), 31 thrombotic events were registered (incidence rate: 2.2 thromboses/100 patients/year). When compared with the general population, young ET patients showed a significant increase in stroke (odds ratio 50, 95% CI: 21.5-115) and venous thromboses (odds ratio 5.3, 95% CI: 3.9-10.6). Thrombosis-free survival was 84% at 10 years, with tobacco use being associated with higher risk of thrombosis. Actuarial freedom from evolution to MF was 97% at 10 years. In conclusion, young ET patients have thrombotic events, especially stroke and venous thrombosis, more frequently than generally considered, whereas they rarely transform to MF.

A 19-year-old man with paroxysmal nocturnal hemoglobinuria treated for 3 years with Methandienone was admitted to the hospital with hemoperitoneum due to the rupture of an hepatic tumor. Histology revealed that it was a benign liver cell adenoma, with a pathologic appearance and mode of clinical presentation closely resembling those of cases observed to develop in association to contraceptive steroids. " Doubt has been raised about the histologic diagnosis of the latter, for the data presented are not always convincing as to the malignant nature of these growths.' Recently, a patient with a hepatic adenoma after prolonged treatment with oxymetholone was described. 2 ' We report a patient with a benign liver cell adenoma associated with Methandienone treatment, who seems to be the second recorded case of adenoma associated with androgenicanabolic steroids. CASE REPORTA 19-year-old man, treated for paroxysmal nocturnal hemoglobinuria (PNH) with Methandienone for 3 years, was admitted to hospital in May, 1975 patient was included in a cooperative therapeutic trial of the effect of androgenic-anabolic steroids on bone marrow insufficiency* and administered Methandienone in a dose of 65 mg daily. After 20 months of treatment a slight improvement of the pancytopenia was noted. In January, 1975, he had the first crisis of PNH, which reappeared in April, in spite of the treatment. In May, 1975, he was readmitted to the hospital complaining of left hypocondrial pain. Physical examination showed signs of peritonitis and an abdominal tap revealed the presence of blood. At laparotomy two small tumors were found in the left lobe of the liver. One of the tumors was ruptured and was responsible for the hemoperitoneum. Resection of the affected liver was done and the postoperative course was uneventful. Serum alpha-1-fetoprotein was negative in blood taken immediately after operation. Histological ExaminationAt microscopic examination both tumors, 10 X 60 X 30 cm and 80 X 45 X 25 mm in size, were pale and capsulated. The larger one had an extensive area of hemorrhagic necrosis at the surface.Microscopic examination showed a well-differentiated lesion, composed of liver cells resembling normal hepatocytes, arranged in plates thicker than in normal liver. Within the tumor there were no portal tracts, or biliary structures. Many dilated sinusoids and large spaces containing blood were seen, some of them with endothelium layer and other with no wall, as well as large areas of hemorrhagic necrosis (Fig.

Vascular events and evolution to either myelofibrosis (MF) and acute leukemia (AL) are the main causes of morbidity and mortality in individuals with essential thrombocythemia (ET). However, the frequency of these complications in young ET patients is not well known. The objective of the present study was to assess the frequency of vascular events and the incidence of MF and AL in young patients with ET and to identify the factors associated with the development of such complications. In 126 subjects diagnosed with ET at a median age of 31 years (range: 5–40), overall survival and probability of survival free of either thrombosis, bleeding, MF, AL, and polycythemia vera (PV) were analyzed by the Kaplan-Meier method, followed by the log-rank test. With a median follow-up of eleven years (range: 4–25) three patients have died, being the probability of survival 98% at ten years. A total of 31 thrombotic events were registered in 25 patients; thrombosis-free survival (TFS) was 84% at ten years. Tobacco use was the only factor associated with an increased thrombotic risk, since TFS at 10 years was 72% in smokers versus 90% in non-smokers (p=0.03). Severe hemorrhagic complications were observed in 11 patients, and the estimated probability of bleeding-free survival was 92% at ten years. Evolution to MF was seen in 6 patients, four of whom had never received treatment for ET. MF-free survival was 97% at 10 years, with the risk being higher in patients showing an increased reticulin network in the bone marrow biopsy performed at diagnosis of ET (p=0.005). Transformation to AL was registered in one patient. JAK2 was mutated in 33 out of the 87 assessable patients (38%) and the mutation was associated with higher Hb values at diagnosis (p = 0.001). ET evolved into PV in five patients, being the probability of evolution into PV of 15% in JAK2 V617F positive patients versus 0% in JAK2 V617F negative patients (p=0.01). In conclusion, severe vascular complications are not infrequent in young subjects with ET, whereas transformation to MF or AL is a rare event.

Bone-marrow biopsy has been performed in 63 cases of chronic lymphocytic leukaemia (CLL). Four different histological patterns were observed: (a) interstitial (lymphoid infiltration without displacement of fat cells) in 12 cases; (b) nodular (abnormal lymphoid nodules without interstitial infiltration) in 10 cases; (c) mixed (combination of the first two patterns) in 21 cases; and (d) diffuse (replacement of both haemopoietic and fat cells by lymphoid infiltration) in 20 cases. Statistical analysis of actuarial curves showed a significant difference of survival probability according to the bone marrow infiltration patterns. Thus, in patients with interstitial or nodular patterns the life expectancy is significantly longer than in those with mixed or diffuse patterns. furthermore, a significant degree of correlation between bone marrow infiltration patterns and different methods of clinical staging in CLL was apparent. The different bone marrow infiltration patterns in CLL probably reflects variations in the amount of lymphoid accumulation during the natural course of this disease. Because of its prognostic significance, bone marrow biopsy should have a place in CLL evaluation and staging.