Anti B cell targeted immunotherapy for treatment of refractory autoimmune haemolytic anaemia in a young infant

We report the case of an 8 week old infant with fulminant autoimmune haemolytic anaemia refractory to conventional immunomodulating treatment. Massive haemolysis resulted in cardiac decompensation and acute renal failure which necessitated mechanical ventilation and peritoneal dialysis. Rituximab, a chimeric anti-CD20 monoclonal antibody, halted progression of the haemolytic process, but the patient died of acute viral pneumonia and disseminated fungal infection. Earlier introduction of rituximab might have prevented the renal complications. Paediatricians should be aware of this useful therapeutic tool for treatment of refractory autoimmune haemolytic anaemia and balance its use against the risk of potential life threatening infection.     

Primary periosteal lymphoma: an unusual presentation of non-Hodgkin's lymphoma with radiographic,MR imaging,and pathologic correlation

This report describes a primary periosteal location of non-Hodgkin's lymphoma, without nodal disease, and without adjacent intramedullary disease at presentation. The clinical and imaging appearance of periosteal lymphoma simulates other neoplastic osseous surface tumors more than that of lymphoma in other locations. Consideration of this rare presentation of non-Hodgkin's lymphoma in the differential diagnosis of periosteal bone lesions can be helpful to ensure proper diagnosis and treatment.     

Diagnostic and therapeutic percutaneous gallbladder procedures

The authors report their experience with 24 patients who underwent a variety of percutaneous procedures involving the gallbladder. Twenty diagnostic and 13 therapeutic procedures were performed under sonographic, computed tomographic (CT), or fluoroscopic guidance; these procedures included biopsy of the gallbladder, diagnostic cholecystography, diagnostic aspiration of bile, gallstone dissolution and removal, cholecystostomy for drainage, and gallbladder abscess drainage. The indications for percutaneous cholecystostomy (performed in 11 patients) included relief of hydrops and empyema, gallstone dissolution, mechanical gallstone removal, and drainage for malignant obstruction. Each procedure was successful. There was one complicating episode of cholecystitis and four previously described episodes of vagal hypotension. Bile peritonitis did not occur in any of the patients. The authors discuss the various percutaneous gallbladder procedures and specific technical considerations in performing them.     

Abrogation of Microsatellite-instable Tumors Using a Highly Selective Suicide Gene/Prodrug Combination

A substantial fraction of sporadic and inherited colorectal and endometrial cancers in humans is deficient in DNA mismatch repair (MMR). These cancers are characterized by length alterations in ubiquitous simple sequence repeats, a phenotype called microsatellite instability. Here we have exploited this phenotype by developing a novel approach for the highly selective gene therapy of MMR-deficient tumors. To achieve this selectivity, we mutated the VP22FCU1 suicide gene by inserting an out-of-frame microsatellite within its coding region. We show that in a significant fraction of microsatellite-instable (MSI) cells carrying the mutated suicide gene, full-length protein becomes expressed within a few cell doublings, presumably resulting from a reverting frameshift within the inserted microsatellite. Treatment of these cells with the innocuous prodrug 5-fluorocytosine (5-FC) induces strong cytotoxicity and we demonstrate that this owes to multiple bystander effects conferred by the suicide gene/prodrug combination. In a mouse model, MMR-deficient tumors that contained the out-of-frame VP22FCU1 gene displayed strong remission after treatment with 5-FC, without any obvious adverse systemic effects to the mouse. By virtue of its high selectivity and potency, this conditional enzyme/prodrug combination may hold promise for the treatment or prevention of MMR-deficient cancer in humans.     

OC5Diagnosis of secondary syphilis due to oral lesions

Central giant cell granuloma (CGCG) is a benign lesion of unknown etiology that especially affects the jaws, usually appears in patients younger than 30 years and is more common in females than in males. Lesions generally occur in the anterior portion of the jaws and mandibular lesions frequently cross the midline. The conventional treatment of CGCG is surgical removal. According to clinical and radiological features, the extent of tissue removal ranges from simple curettage to an en bloc resection. We present an 8-year-old male patient, lack of any systemic disease or syndrome, complaining from painless swelling of the left posterior part of the mandible. In intraoral examination, a firm, expansive swelling with overlying intact mucosa, extending from mandibular primary canine to the distal portion of the left permanent first molar, was detected. A panoramic radiograph exhibited an ill-defined radiolucent lesion of 1.5 x 2 cm, extending from left permanent first molar to angulus mandibula and a well-defined, unilocular radiolucent lesion of 1 x 2 cm, extending from angulus to ramus mandibula on the asymptomatic right side. After the assessment of MRI, surgical removal was planned with the otorhinolaryngology department and lesions were totally removed under general anesthesia in two consecutive operation. Histopathologic examination revealed typical giant cell granuloma containing numerous multinucleated giant cells embedded in a fibrous stroma. In presenting this case we aimed to share occurrence of bilateral central giant cell granulomas of the mandible in the absence of hyperparathyroidism and associated syndromes, which is very rare in the literature.     

Metallic hip implants: CT with multiplanar reconstruction

Thirty consecutive patients with metallic implants in the hip were evaluated with both standard transaxial computed tomography (CT) and multiplanar reconstruction (MPR) to assess the contribution of MPR. The metallic devices included total hip replacements (n = 6), one or more surgical plates with screws (n = 9), acetabular pins or screws (n = 9), and Knowles pins (n = 6). The metallic implants were on the side of interest in 25 hips and in the contralateral hip in five cases. One patient had bilateral hardware. MPR reformats axially acquired data into other planes. The reconstruction program weights the true signal over the randomly distributed artifacts by integrating adjacent axial images. Examination using standard transaxial imaging was graded as excellent in five cases, adequate in 15 cases, and inadequate in ten cases. After MPR, 19 studies were classified as excellent, 11 were classified as adequate, and none were classified as inadequate. It is concluded that MPR significantly reduced artifacts caused by metal present in transaxial images.