Iliopsoas hemophilic pseudotumor with bowel fistulization.

Haemophiliac pseudotumors are usually observed in the diaphysis of long bones. Pseudotumors due to psoas muscle hematoma are rare and surgical management is difficult. Surgical treatment of these lesions is usually associated with high morbidity and mortality rate. Here, we present a case with iliopsoas haemophiliac pseudotumors with bowel fistulization who underwent three abdominal operations and survived. Based on our experiences in this patient, we recommend to wait for the intraabdominal hematoma and adhesions to resolve and organise, so that the dissection can be kept to a minimum, which decreases the chances of iatrogenic injury and surgical bleeding (Fig. 3, Ref. 15). Full Text (Free, PDF) www.bmj.sk.

Unchanged global fibrinolytic capacity during the course of hematopoietic stem cell transplantation.

Hemostatic changes due to vascular endothelial damage are seen during the course of hematopoietic stem cell transplantation (HSCT). The fibrinolytic response to ongoing hemostatic activation in HSCT remains to be elucidated. Global fibrinolytic capacity (GFC) is a novel method, which reflects the amount of generated D-dimer when fibrinolysis of a freeze-dried fibrin clot is stopped by introducing aprotinin. GFC is sensitive to all the factors involved in the process of fibrinolysis. The aim of this study was to serially assess GFC at certain critical time points (days -1, +7, +14, +21 prior to and following stem cell infusion) during the course of HSCT. The study group comprised 16 patients with hematological malignancies (11 women, five men; median age 32+/-9 years) in whom HSCT had been performed. Thirty healthy adults (21 women, nine men; median age 31+/-7 years) served as controls. In this study, global fibrinolytic response, as reflected by GFC, was unchanged despite ongoing hemostatic activation, as indicated by D-dimer, moreover GFC remained stable, despite the development of thrombocytopenia associated with HSCT prior to platelet engraftment. Our results indicate that a global fibrinolytic response was impaired as a compensatory response to endothelial activation and to other hemostatic changes seen in HSCT. Further studies in larger HSCT populations are warranted to better understand the implications of these findings.

Massive pericardial effusion and dilated cardiomyopathy in a patient with familial multiple myeloma.

An 80-years-old-man with adult polycystic kidney disease and familial multiple myeloma that is complicated with massive pericardial effusion and dilated cardiomyopathy during the course of the disease is presented. Although no definite single genetic disorder is described, multiple myeloma cases may be seen in certain families. Environmental factors are also blamed in the etiology. Multiple myeloma may be complicated by myocardial and pericardial involvement, diagnoses of which are possible only during postmortem examination in some cases.

Circulating thrombopoietin in clonal versus reactive thrombocytosis.

INTRODUCTION: The aim of this study is to assess circulating thrombopoietin concentrations in patients with both clonal and reactive thrombocytosis (RT), which are two distinct categories of extreme platelet production circumstances. Investigation of the thrombopoietin levels in clonal versus reactive thrombocytosis may help us to understand the interactions of this key regulatory cytokine and the conditions in which abnormally increased platelet formation exist. MATERIALS AND METHODS: Thrombopoietin levels were measured in patients with platelet counts greater than 500 x1 0(3) microl(-1). The study population consisted of 21 patients with RT (13 with iron deficiency anemia, and 8 with rheumatoid arthritis), 24 patients with clonal thrombocytosis (six with essential thrombocytosis, three with myelofibrosis, eight with chronic myelogenous leukemia, and seven with polycythemia vera (PV)) and 16 healthy subjects were used as controls. RESULTS: The median plasma thrombopoietin concentration was 100.5 pg ml(-1) in patients with RT, 467 pg ml(-1) in patients with clonal thrombocytosis and 62.65 pg ml(-1) in the control group. The thrombopoietin concentration was found to be higher in the patients with primary thrombocytosis when compared to the control group (p=0.001), as well as in patients with RT (p=0.002). However, there was no statistically significant difference between the patients with RT and the control group (p=0.14). There was no correlation between thrombopoietin levels and the platelet counts in patients with clonal thrombocytosis, including essential thrombocythemia (ET). CONCLUSIION: Increased levels of thrombopoietin were found in patients with clonal thrombocytosis versus patients with RT and control subjects as well. Defective clearance of thrombopoietin by megakaryocytes and platelets due to a reduced number of thrombopoietin receptors may be the causative mechanism behind this. These results indicate that plasma thrombopoietin levels may be helpful in distinguishing between clonal and reactive thrombocytosis.

Piebaldism associated with congenital dyserythropoietic anemia type II (HEMPAS).

Congenital dyserythropoietic anemias (CDAs) are a group of relatively rare inherited anemias. They are characterized by ineffective erythropoiesis and classified as three major groups and a number of variants. CDA type II, also known as hereditary erythroblastic multinuclearity with a positive acidified serum test (HEMPAS), is the most frequent one. A number of associations with CDA II have been reported, although each described only one or a few patients. Here we presented a piebald woman with vaginal atresia who was tested for anemia and diagnosed as CDA type II. Piebaldism and anemia association were previously described in the mouse. Our case was the first that shows the features of both piebaldism and CDA in the same patient. This association may suggest a stem cell defect to cause both hematopoietic and cutaneous manifestations.

Unchanged global fibrinolytic capacity despite increased factor VIIa activity in Behçet's disease: evidence of a prethrombotic state.

The aim of this study was to evaluate coagulation and fibrinolytic systems in Behçet's disease (BD). Accordingly, various parameters of the coagulation and fibrinolytic systems were investigated in 39 patients with BD and 31 age- and sex-matched healthy volunteers as the control group. Seven of these patients with BD had histories of thrombotic complication. Three were found to have decreased protein S activity, and one patient had diminished protein C activity. Each of those patients had experienced a thromboembolic event. Activated protein C resistance was present in two patients, one of whom had had a thromboembolic episode. Activated FVII (FVIIa), fibrinogen, and cholesterol levels were significantly higher in patients with BD than in the control group. In the patient group, plasma FVIIa level was inversely correlated with age. Plasma global fibrinolytic capacity (GFC) did not differ between the patients and control group. No statistically significant difference was found in the GFC and FVIIa levels between patients with and without histories of thrombosis. Although the coagulation system was activated in vivo in patients with BD, there was no reactive activation in the fibrinolytic system to counteract the activated coagulation system. These findings suggest a relative hypofibrinolytic state in BD.