1/157. Ultrastructure of the haemophilic synovial membrane and electron-probe X-ray analysis of haemosiderin. An ultrastructural study of the haemophilic synovial membrane revealed the presence of solitary siderosomes, compound siderosomes and a peppering of the cell cytoplasm with electron-dense particles. These changes were found in synovial intimal cells, subsynovial macrophages and fibroblasts. Electron-probe X-ray analysis of siderosomes revealed the presence of iron and traces of phosphorus. On the basis of previous and present studies it is postulated that haemosiderin is essentially a condensate of hydrated ferric oxide and that a variable amount of phospholipid material lies in company with it in the siderosome. ( info) |
2/157. The locked patella. An unusual complication of haemophilia. Mechanical derangements of the knee are an uncommon complication of chronic haemophiliac arthropathy. Two patients with locking of the patella were treated by manipulation. The mechanism of the injury was forced flexion of the knee joint beyond the limit of its restricted range. The injury is a serious one and may take six months to recover. ( info) |
3/157. An acutely painful elbow as a first presentation of von Willebrand's disease. A 26 year old woman presented to the accident and emergency department with a painful right elbow. There had been no history of trauma. Clinical examination suggested an effusion, which was confirmed on radiological examination. Her elbow was aspirated and revealed a haemarthrosis. Subsequent investigations revealed a diagnosis of von Willebrand's disease (vWD). A spontaneously occurring effusion of the elbow may be due to a haemarthrosis. Aspiration of blood in the absence of trauma may lead to a diagnosis of an occult coagulopathy in addition to relieving pain. The diagnosis and treatment of vWD is discussed. ( info) |
4/157. The importance of arthroscopy in diagnosing synovial haemangioma of the knee joint. In connection with one of their cases, authors call attention to the importance of differential diagnosis in synovial haemangioma of the knee joint, a rare condition. They also give a survey of its clinical, radiological and pathological features, review the literature on the topic, with special emphasis on the importance of arthroscopy in making the clinical diagnosis, and describe the treatment applied in their case. ( info) |
5/157. Dealing with a hemophilia-A patient undergoing cerebral aneurysm surgery. In this article anesthesiologic and hematologic aspects of a patient with Hemophilia-A, who underwent craniotomy for a right middle cerebral artery aneurysm, are discussed. ( info) |
6/157. Expanding portal haematomata as a complication of knee arthroscopies in persons with haemophilia. Recurrent haemarthroses stimulate the hypertrophy of synovial tissues that if left in situ will eventually cause joint destruction. Synovectomies have been the cornerstone of joint preservation and a number of different methods exist. We report two patients who suffered complications after an arthroscopic procedure. No previous complications of this nature have been reported in the literature. ( info) |
7/157. Potentiation of oral anticoagulation and hemarthrosis associated with nabumetone. Concomitant therapy with warfarin and nonsteroidal antiinflammatory drugs (NSAIDs) is of concern due to the potential for increased bleeding. Nonsteroidal antiinflammatory drugs may alter patient response to warfarin by pharmacodynamic or pharmacokinetic interactions. A man receiving long-term, stable warfarin therapy experienced a significant increase in international normalized ratio 1 week after nabumetone was added to his regimen. Despite prompt reduction of the warfarin dosage, he experienced hemarthrosis of his right knee. Previous reports suggested lack of interaction between nabumetone and warfarin. Caution and close monitoring are advisable when the two agents are administered concomitantly. ( info) |
| A 52-year-old, trans-femoral amputee with haemophilia was hospitalized because of ambulatory problems arising from the osteo-arthropathic involvement of other major articulations. Reduced function in the upper limbs, caused by the effects of recurrent haemarthroses, resulted in additional problems concerning the usage of auxiliary ambulatory aids. The advantages and disadvantages of traditional and experimental crutches highlight the functional problems of ambulation in persons with concomitant upper limb pathologies. ( info) |
9/157. Acquired delta-storage pool deficiency associated with idiopathic myelofibrosis. A 73-year-old woman complained of easy bruising, as a consequence of prolonged bleeding time despite normal platelet counts. platelet aggregation profile, mepacrine fluorescence test, flow cytometry and transmission electron microscopy studies led to the diagnosis of delta-storage pool deficiency (SPD) A few months later, she developed hyperleucocytosis with immature granulocytes and erythroblasts. The presence of bone marrow fibrosis and clonal cytogenetic abnormalities led to the diagnosis of idiopathic myelofibrosis (IM). association between SPD and IM has never been reported. The pathogenesis of this unusual association remains unclear and may involve proliferation of abnormal monoclonal stem cells with differentiation into activated megakaryocytes associated with impaired dense granule development and increased cytokines release which may be. involved in myelofibrosis. ( info) |
10/157. Recombinant VIIa concentrate in the management of bleeding following prothrombin complex concentrate-related myocardial infarction in patients with haemophilia and inhibitors. prothrombin complex concentrates (PCCs) and, more recently, activated prothrombin complex concentrates (APCCs), are widely used for the treatment of active bleeding in haemophiliacs with inhibitors. myocardial infarction (MI), associated with the use of these concentrates, is a well-recognized, but uncommon, complication. We review the 14 previous cases published in the literature and describe two additional patients. MI related to the use of activated and non-activated PCCs predominantly affects young patients who often have no preceding history of, or risk factors for, MI and tends to be associated with large cumulative doses of concentrate. The most frequent pathological finding is myocardial haemorrhage, with no evidence of coronary artery atheroma or thrombosis. The management of further bleeding in these patients is difficult. We have safely used recombinant factor viia to treat bleeding in the immediate and long-term period following PCC-related MI. ( info) |