Stergios Tassiopoulos

Raynaud's phenomenon is correlated with elevated systolic pulmonary arterial pressure in patients with systemic lupus erythematosus:

In patients with Systemic lupus erythematosus (SLE), Raynaud phenomenon (RP) is frequently present and associated with pulmonary hypertension (PHT). Elevated pulmonary artery systolic pressure (PASP) is an indicator of PHT and can be estimated noninvasively. We attempt to explore the significance of RP in SLE and to correlate it with clinical and serological parameters of the disease. The study population consisted of 34 patients (age, sex and disease duration matched) who fulfilled the revised SLE criteria of the American College of Rheumatology, and were categorized into two groups: Group 1 had patients having SLE and RP (2 males/15 females, mean age 45 ± 18 years) and group 2 had patients with SLE but without RP (3 males/14 females, mean age 40 ± 14 years. Detailed cardiac ultrasound was performed including measurement of PASP, while clinical and serological features of both groups were collected and correlated. Significant differences were shown in the presence of arterial hypertension (P < 0.05), arthralgias (P < 0.005), arthritis (P < 0.05), myalgias (P < 0.05), alopecia (P < 0.05) and PASP (P < 0.0001). No difference was observed among the cardiac ultrasound indices and the ejection fraction between the two groups. PASP was significantly correlated with RP, while no correlation was observed regarding the disease duration. In patients with SLE, the presence of RP was associated with elevation in PASP. Further investigation is needed to clarify the significance of this relation. Lupus (2007) 16, 505—508.

Does Heterozygous β-Thalassemia Confer a Protection against Coronary Artery Disease?

Abstract: Six hundred and thirty‐eight patients who presented with clinical symptoms and/or electrocardiographic findings suggestive of stable angina pectoris were studied; they were also investigated by coronary arteriography. Hemoglobin electrophoresis was performed on all patients to detect the presence of the β‐thalassemia trait. Results were analyzed by logistic regression analysis to determine whether the latter confers any protective effect against advanced coronary artery disease (aCAD; defined as the presence of atheromas in coronary arteries, resulting in stenosis at least 70%). WeThe role of the currently accepted risk factors (smoking, hypertension, hypercholesterolemia, and diabetes) in developing aCAD were reconfirmed, while at the same time it was found that β‐thalassemia heterozygosity is associated with a reduced risk against aCAD (odds ratio 0.39, 95% confidence interval 0.16‐0.98). The lipoprotein and blood rheology profile of these individuals may be the underlying causes of this protective effect.

Hemothorax due to extramedullary erythropoietic masses

We describe a 27-year-old male patient suffering from beta-thalassemia intermedia who presented with a nontraumatic spontaneous hemothorax due to extramedullary hemopoietic foci. In reviewing the literature, four similar reports were found. The details of this unusual entity are discussed.

Unusual radiological findings in a case of myelofibrosis secondary to polycythemia vera

Dear Sir, The participation of bone tissue is an innate element in the pathogenesis of myelofibrosis, both idiopathic and secondary. Consequently, bone lesions are commonly encountered in patients with myelofibrosis and their röntgenomorphologic expressions have been precisely studied and defined [1–4]. The most common bone disorder, occurring in 40–70% of patients with myelofibrosis, is osteosclerosis [4, 5]. The osteosclerotic areas are caused by irregular thickening of bone trabeculae and formation of new ones without, in most cases, being accompanied by subperiosteal osteogenesis and increase of bone volume [6]. These changes usually involve bones with active bone marrow (vertebrae, ribs, and pelvis), less often the proximal metaphyseal ends of the femora and humeri and only occasionally the radius, tibia, and feet. Diffuse osteoporosis is the second in frequency radiographic finding in myelofibrosis, while the coexistence of osteosclerosis and osteoporosis, with the characteristic “moth eaten” appearance, may also occur [2]. Osteolytic lesions are uncommon and when present are often mixed with osteosclerosis [7]. In some cases, the above radiological picture is associated with extramedullary hemopoietic foci which on X-ray appear as homogenous, welldefined, multilobulate shadows, usually located in the paravertebral cleavages, in the posterior mediastinum and also in many other organs and tissues [3, 8]. These features may develop at any phase of myelofibrosis suggesting a benign process. We report the unusual case of a 72-year-old male with myelofibrosis secondary to polycythemia vera who during the last year of his disease presented multiple osteolytic lesions. The patient was admitted to our hospital for investigation of lower back pain shooting down to his right foot. The physical examination revealed severe localized pain and local tenderness on palpation of several areas around the spine, particularly in the third lumbar vertebra and the sacral bone. There were also positive sciatic stretch test, muscle atrophy of the right quadriceps with a decrease in muscle strength, symmetric weakness of reflexes, and sensory loss of the anterior right thigh as well as the outer and posterior side of the right calf. The patient’s laboratory data were normal except for the following: Ht 31.8%, Hb 10 g/dl, RBC 3.95×10/l, serum iron 18 μg/dl, and ferritin 637 ng/dl. Erythropoietin activity was 19.2 mu/ml (normal <19). The morphology of the peripheral blood smear revealed mild poikilocytosis (elliptocytes, teardrop-like cells, acanthocytes, and schistocytes) and moderate hypochromia, anisocytosis, and microcytosis. Skeletal imaging survey (X-ray, CT, and MRI) revealed multiple osteolytic lesions localized in almost all adult myeloproductive bones, including ribs, sternum, vertebrae, pelvis, and calcaneus. In particular, severe and extensive osteolytic lesions were seen in the vertebral bodies, some of which were completely compressed. However, the intervertebral disks were intact. Around most of the osteolytic lesions, soft tissue masses originated from the bone lesions and infiltrated the adjacent tissues (Fig. 1). Needle biopsy of a paraspinal mass showed myeloid metaplasia with predominantly granulocyte precursors, megakaryocytes, and a scarcity of mature cells. CD34 positive cells were not identified (Fig. 2). P. Sideris . S. Tassiopoulos . N. Sakellaropoulos . E. Variami . H. Gogas . M. Kanakis . G. Vaiopoulos (*) First Department of Internal Medicine, School of Medicine, University of Athens, “Laikon” Hospital, 17 Agiou Thoma Street, Athens 11527, Greece e-mail: e.rissaki@laiko.gr Tel.: +30-21-07771161 Fax: +30-21-07788830