Thrombotic thrombocytopenic purpura associated with multiple myeloma

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic disease. Associations with collagen vascular diseases, pregnancy, some drugs, surgical intervention, and infections are documented (known). Malignancy is also one of the known factors associated with thrombotic thrombocytopenic purpura. These malignancies are usually the disseminated solid organ tumors. Hematological malignancies constitute a rare association. Here, we present a patient with thrombotic thrombocytopenic purpura associated with multiple myeloma and discuss the pathogenesis. To our knowledge, this is the first case report of thrombotic thrombocytopenic purpura associated with multiple myeloma.     

Effect of platelet count on secretion capacity: formulization and use of the formulae for evaluation of platelet secretion in thrombocytopenic patients

The correlation between platelet count and bleeding time is nonlinear. The bleeding time prolongs more prominently as platelet count decreases toward 10 000 microl(-1); however, it becomes stable near 100 000 microl(-1). Clinical observations suggest that platelet functional capacity may also play a role in bleeding complications during thrombocytopenia. However, no routine method has been described for evaluation of platelet function during thrombocytopenia. To test if platelet functional capacity is affected by the cell count, as suggested by the platelet count-bleeding time correlation. To evaluate lumiaggregometry as a possible tool for studying platelet functions and prediction of bleeding in thrombocytopenic patients. Collagen-induced ATP release was studied in different dilutions of 22 healthy platelet-rich plasmas. The relationship between platelet count and ATP release was formulized. ATP release was also tested in 24 thrombocytopenic (10 000-50 000 microl(-1). ) patients, and the results were compared with expected levels derived from the formulae. ATP release increased in a cubic fashion as platelet count elevated. ATP secretion values were within normal limits or increased in patients with idiopathic thrombocytopenic purpura. However, some patients with megakaryocyte deficiency had a secretion defect. ATP secretion was decreased in four out of seven patients with bleeding symptoms compared with no persons without bleeding (P = 0.003). Platelet functional capacity is affected by the cell count. Lumiaggregometry is potentially useful in evaluating platelet functions during thrombocytopenia.     

Frontoethmoid sinus osteoma as a cause of subperiosteal orbital abscess

A 65-year-old woman presented with severe periorbital pain and swelling of the left eye, with complete ptosis, proptosis, and conjunctival chemosis. The eye was in a hypotropic position, and activity in the left superior rectus was inadequate. A firm, elastic, 2-cm mass was palpated near the superior orbital rim. Computed tomography revealed a subperiosteal abscess (SPA) at the superior portion of the orbit and a large frontoethmoidal sinus osteoma. After the SPA had been surgically drained and the osteoma completely removed, the patient recovered, with resolution of proptosis, ptosis, and motility limitations. Osteomas of the paranasal sinuses are usually asymptomatic and rarely cause SPA and orbital cellulitis. Therefore, osteoma cases must be closely followed to ensure that early diagnosis and treatment of SPA are successful in preventing severe visual loss and rescuing the patient’s vision.     

Lamotrigine reduces intestinal I/R injury in the rat

I/R injury of the intestine is a life-threatening emergency with mortality rates still more than 60%. We have investigated the protective effect of lamotrigine (LTG), an antiepileptic drug, which has an established neuroprotective effect, on intestinal I/R injury in rats. Forty-eight Wistar albino rats were divided into three groups: a sham-operated group (no I/R injury; n = 16), an ischemic control group (I/R, n = 16), and an LTG-treated group (pretreatment 5 mg kg-1 LTG + IR; n = 16). A marker for lipid peroxidation, malondialdehyde, free radical scavengers, glutathione peroxidase, catalase, and superoxide dismutase levels, an index of polymorphonuclear neutrophils, myeloperoxidase activity, and mucosal damage were investigated. Malondialdehyde levels, myeloperoxidase activity, and the severity of mucosal damage were decreased in the LTG group. Moreover, in the LTG group, glutathione peroxidase and superoxide dismutase levels were higher compared with the I/R group. The pretreatment of rats with LTG before intestinal ischemia ameliorates the mucosal damage in intestinal I/R injury probably by altering lipid peroxidation, neutrophil accumulation, and antioxidant activity.     

The use of low-dose cyclophosphamide followed by AZA/MMF treatment in childhood lupus nephritis

Cyclophosphamide (CYC) has been the landmark in the treatment of lupus nephritis. However, long-term treatment with CYC is associated with significant side effects. We aimed to evaluate the efficacy of short-term intravenous (IV) CYC treatment as a remission induction treatment followed by azathioprine (AZA) or mycophenolate mofetil (MMF) as a maintenance treatment. Twenty patients (18 girls) with biopsy-proven class III (5) and IV (15) lupus nephritis were included in to the study. Detailed clinical and laboratory data and patient outcomes were evaluated. All patients received three methylprednisolone (MP) IV pulses, followed by oral prednisone 0.5−1 mg/kg per day and one IV pulse of CYC per month for 6 months. Azathioprine was started as a remission-maintaining treatment. In ten of 20 patients, treatment was switched to MMF. The mean age at the time of diagnosis was 16.11 ± 3.49 years, and the mean duration of follow-up was 49.6 ± 27 months. Fourteen patients (70%) had complete remission, three (15%) had partial remission, one (5%) continued to have active disease, and two (10%) progressed to end-stage renal disease. Nine of the patients (45%) with complete remission had received AZA, and switching to MMF increased complete remission rate (additional five patients; 25%). In conclusion, short-term (6-month) IV bolus CYC treatment followed by AZA is a safe and effective treatment in children with severe lupus nephritis, and using MMF increases remission rate in resistant cases.     

Hippocampal abnormalities associated with various congenital malformations

Objective  Our objective in this retrospective study was to assess the hippocampal abnormalities, associate them with various congenital brain malformations, and define the frequency of the association in specific anomaly subgroups. Methods  A total of 62 patients with congenital malformations of the brain who had thin-slice coronal T2-weighted, fluid attenuated inversion recovery, IR, or T1-weighted 3D gradient echo images were retrospectively evaluated for the type of congenital brain malformation and morphological or rotational hippocampal abnormalities. Medical records were reviewed for age, sex, and symptoms. Conclusion  Hippocampal abnormalities are found in 55.8% of all patients with different kinds of congenital brain malformations that are mostly associated with cortical dysplasia, lissencephaly, and total agenesis of the corpus callosum. The severity of the injury may have an effect on the extent of the involvement of the brain.     

Can multidetector row CT visualize the right and left inferior phrenic artery in a population without disease of the liver?

Purpose  

To determine the ability to visualize the origin of the right inferior phrenic artery (RIPA) and the left inferior phrenic artery (LIPA) by multidetector row computed tomography (MDCT) in a population without disease of the liver.     

Dendritic cell sarcomas/tumours of the breast: report of two cases

Extranodal follicular dendritic cell sarcoma/tumours (FDCS/Ts) and interdigitating dendritic cell sarcoma/tumours (IDCS/Ts) are rare neoplasms. We present two cases of FDCS/T and IDCS/T of the breast. The FDCS/T case (case 1) presented in a 31-year-old woman and the IDCS/T case (case 2) in a 67-year-old woman who both showed a firm lump in the left breast. The FDCS/T lesion superficially appeared as an anaplastic carcinoma and the IDCS/T was reminiscent of a spindle cell sarcomatoid carcinoma. Nevertheless both lesions were negative for keratins while case 1 displayed neoplastic cells strongly positive for CD21, vimentin and focally for CD68 and S-100 protein. The tumour cells of case 2 were positive for S-100, CD68 and CD45. In breast, an unusual keratin negative tumour composed predominantly of spindle cells arranged in fascicles, storiform pattern or whorls with a lymphoid rich stroma should raise suspicion for FDCS/Ts or IDCS/Ts. The distinction from malignant tumours with similar features is discussed.     

Implant-retained auricular prosthesis

Auricular defects may result from congenital malformations, injuries from motor vehicle crashes, trauma, or tumor resections. An auricular prosthesis was fabricated for a patient who had an injury from a motor vehicle crash. Extraoral implants and bar-and-clip retention for the proper connection of the auricular prosthesis to implant were used. This prosthesis was acceptable to the patient because of excellent support, retentive abilities, and the patient's appearance.