[Laparoscopic splenectomy for sclerosing angiomatoid nodular transformation]. / Laparoskopische Splenektomie bei sklerosierender angiomatöser nodulärer Transformation.

Sclerosing angiomatoid nodular transformation (SANT) is a benign lesion of the spleen which can be cured by splenectomy. In the literature about 45 cases have been reviewed. Although it is defined by the morphological details, data regarding surgical therapy are scarce. To the best of our knowledge, a laparoscopic approach has not been published before. We investigated in one case of SANT the feasibility of a laparoscopic approach. Histological investigations confirmed the diagnosis of a SANT which was resected in toto. This report shows that the laparoscopic splenectomy is a feasible, safe and effective method for treatment of SANT.

[An unusual complication of chronic pancreatitis]. / Ungewöhnliche Komplikation eines akuten Schubs bei chronischer Pankreatitis.

Atraumatic rupture of the spleen is a rare, but life-threatening complication of pancreatitis. We report a case of an atraumatic spleenic rupture in chronic pancreatitis. A 41 year old man presented in the emergency room with abdominal pain and typical signs of acute pancreatitis. His medical history showed a chronic pancreatitis due to alcoholism with recurrent acute pancreatitic episodes. He denied any trauma in the recent past. In the next few hours he showed clinical signs of a severe hemorrhagic shock. The haemoglobin level fell from 9.4 to 3.0 g/dl. Abdominal ultrasound and abdominal CT scan showed free fluid. In the following laparotomy a splenectomy was performed due to splenic rupture. A histological examination of the spleen revealed no reason, that could explain the splenic rupture. Hence we assumed a spontaneous rupture. The reported case demonstrates that in acute pancreatitis and signs of shock it is necessary to rule out rupture of the spleen e.g. via ultrasound and abdominal CT scan. If there are signs of spleenic rupture, the only therapy of this life-threatening complication is instant operation to save patient's life.

PRV-1 mRNA expression discriminates two types of essential thrombocythemia.

Essential thrombocythemia (ET) is a heterogeneous disorder. For example, the growth of erythropoietin-independent erythroid colonies, termed "endogenous erythroid colonies (EECs)", has previously been observed in only 50% of ET patients. We have recently described the overexpression of a hematopoietic receptor, PRV-1 (polycythemia rubra vera-1), in patients with polycythemia vera (PV). Here, we compare PRV-1 expression and EEC formation in a cohort of 30 patients with ET; 50% of the ET patients in our cohort displayed EEC growth. Likewise, 50% of the ET patients overexpressed PRV-1. Remarkably, only the 15 ET patients displaying EEC growth showed elevated PRV-1 expression, while the 15 EEC-negative ET patients expressed normal PRV-1 levels. It has previously been reported that EEC-positive ET patients develop PV during long-term follow-up. Here, we show that 40% of the PRV-1-positive patients develop symptoms of PV during the course of their disease. In contrast, none of the 15 PRV-1-negative patients displayed such symptoms (p=0.017). Moreover, PRV-1-positive patients had a significantly higher number of thromboembolic or microcirculatory events (p=0.003). We propose that PRV-1-positive ET comprise a pathophysiologically distinct subgroup of patients, one that is at risk for the development of complications and for the emergence of PV.

Cloning of PRV-1, a novel member of the uPAR receptor superfamily, which is overexpressed in polycythemia rubra vera.

Polycythemia vera (PV) is a clonal stem cell disorder characterized by hyperproliferation of the erythroid, myeloid, and megakaryocytic lineages. Although it has been shown that progenitor cells of patients with PV are hypersensitive to several growth factors, the molecular pathogenesis of this disease remains unknown. To investigate the molecular defects underlying PV, we used subtractive hybridization to isolate complementary DNAs (cDNAs) differentially expressed in patients with PV versus normal controls. We isolated a novel gene, subsequently named PRV-1, which is highly expressed in granulocytes from patients with PV (n = 19), but not detectable in normal control granulocytes (n = 21). Moreover, PRV-1 is not expressed in mononuclear cells from patients with chronic myelogenous leukemia (n = 4) or acute myelogenous leukemia (n = 5) or in granulocytes from patients with essential thrombocythemia (n = 4) or secondary erythrocytosis (n = 4). Northern blot analysis showed that PRV-1 is highly expressed in normal human bone marrow and to a much lesser degree in fetal liver. It is not expressed in a variety of other tissues tested. Although PRV-1 is not expressed in resting granulocytes from normal controls, stimulation of these cells with granulocyte colony-stimulating factor induces PRV-1 expression. The PRV-1 cDNA encodes an open reading frame of 437 amino acids, which contains a signal peptide at the N-terminus and a hydrophobic segment at the C-terminus. In addition, PRV-1 contains 2 cysteine-rich domains homologous to those found in the uPAR/Ly6/CD59/snake toxin-receptor superfamily. We therefore propose that PRV-1 represents a novel hematopoietic receptor. (Blood. 2000;95:2569-2576)