Long-term Rituximab Therapy in Adult Patients with Idiopathic Nephrotic Syndrome.

Patients with refractory idiopathic nephrotic syndrome (INS) are at risk of infections, renal failure, and the inherent side effects of immunosuppressive therapy. In the present study, we investigated the efficacy of yearly rituximab therapy in adult patients with this syndrome over 5-10 years. In the minimal change disease group, 14 of the 15 patients had complete remission (CR) and one had partial remission (PR). Of those who achieved CR, eight patients did not require rituximab 4 years later. The patient with PR was treated in the same way as those with focal segmental glomerulosclerosis (FSGS) group and remained stable by the end of the study. In the FSGS group, rituximab therapy resulted in the amelioration of INS in nine patients, although two were non-responders (NR) and developed end-stage renal disease (ESRD). In the membranous glomerulopathy group, 36 patients had CR but two were NR and developed ESRD. Twenty (55%) of those did not require treatment after 4 years, whereas the rest maintained CR with yearly rituximab. Nine women received rituximab after the third month of pregnancy without significant adverse effects on the fetus or subsequent conception. In conclusion, rituximab is a safe, practical, and effective long-term therapy for adult patients in these three groups of INS.

Complementary bodybuilding: A potential risk for permanent kidney disease.

We report our experience of renal disease associated with bodybuilders who had been on high-protein diet, anabolic androgenic steroids (AASs), and growth hormone (GH) for years. A total of 22 adult males who volunteered information about use of high protein diet and AAS or GH were seen over a six-year period with renal disease. Kidney biopsy revealed focal segmental glomerulosclerosis (FSGS) in eight, nephroangiosclerosis in four, chronic interstitial nephritis in three, acute interstitial nephritis in two, nephrocalcinosis with chronic interstitial nephritis in two, and single patients with membranous glomerulopathy, crescentic glomerulopathy, and sclerosing glomerulonephritis. Patients with FSGS had a longer duration of exposure, late presentation, and worse prognosis. Those with interstitial disease had shorter exposure time and earlier presentation and had improved or stabilized after discontinuation of their practice. There is a need for health education for athletes and bodybuilders to inform them about the risks of renal disease involved with the use of high-protein diet, AAS, and GH.

Splenectomy complicated by sustained extreme thrombocytosis and extensive portosplenomesenteric vein thrombosis in pyrimidine 5'-nucleotidase deficiency.

Reactive and redistributional thrombocytosis is a well-known postsplenectomy occurrence .Usually it is transient and it rarely reaches extreme levels. We report a rare case of haemolytic anaemia where splenectomy was carried out following trauma to a massively enlarged spleen and was followed by extreme sustained thrombocytosis associated with extensive portal, splenic and mesenteric vein thrombosis despite standard antithrombotic prophylaxis.

Rituximab therapy for severe cutaneous leukocytoclastic angiitis refractory to corticosteroids, cellcept and cyclophosphamide.

We report our clinical experience with rituximab in the treatment of 2 patients with idiopathic cutaneous angiitis who relapsed after treatment with high-dose corticosteroids and cyclophosphamide. A 39-year-old woman and a 51-year-old man presented with ulcerating maculopapular rash in both lower limbs which relapsed 6 months after treatment with a combination of high-dose corticosteroids and cyclophosphamide. After treatment with 2 g of rituximab, the first patient has still been in clinical remission for 32 months while the second has finished 28 months. Interestingly, CD19 which had dropped to <0.1 one week following the start of retuximab had increased to >0.5% 8 months later in both patients. Despite that, our patients are still in clinical remission. No significant side effects were noted during infusions and up to the period of follow-up. In conclusion, rituximab is a useful and safe agent in the treatment of idiopathic cutaneous angiitis refractory to conventional therapy. Clinical remission persists years after improvement of B-cell suppression.

Accuracy of the Fischer scoring system and the Breast Imaging Reporting and Data System in identification of malignant breast lesions.

BACKGROUND AND OBJECTIVES: Fischer developed a scoring system in 1999 that made identifying malignnant lesions much easier for inexperienced radiologists. Our study was performed to assess whether this scoring system would help beginners to accurately diagnose breast lesions on magnetic resonance (MR) imaging and to assess the correlation between the magnetic resonance mammography Breast Imaging Reporting and Data System (MRM BI-RADS) grade and the final diagnosis. PATIENTS AND METHODS: The lesion morphology and contrast kinetics of 63 masses in 41 patients were evaluated on MRI and accorded a MRM BI-RADS final assessment category using the Fischer scoring system. The accuracy was evaluated after the final diagnosis was obtained by tissue sampling and follow-up imaging. RESULTS: There were 25 malignant and 30 benign lesions. Eight lesions were seen by MRI only and we could not verify their pathology since we did not have MR-guided biopsy facilities at the time of the study. On MR mammography, the proven carcinomatous lesions were characterized as BI-RADS category V in 16 (64%), category IV in 7 (28%), and category III in 2 (8%) lesions. Benign lesions were graded as category V in 3 (10%), category IV in 6 (20%), and category III in 3 (10%), category II in 10 (33%) and category I in 8 (27%) lesions. The MRM BI-RADS category accurately predicted malignancy in 92% and a benign pathology in 70% of the lesions. The overlap between the MRM features of chronic inflammatory lesions and carcinomas resulted in a lower accuracy in diagnosing benign as compared to malignant lesions. CONCLUSION: The MRM BI-RADS lexicon using the Fischer scoring system is useful and has a high predictive value, especially for malignant breast lesions, and is easy to apply. Overlapping features between benign inflammatory and malignant lesions might yield a reduced accuracy in inflammatory pathologies.

Breast magnetic resonance imaging: initial experience in Kuwait.

OBJECTIVE: To report our initial experience of breast magnetic resonance imaging (MRI) in Kuwait in order to identify and characterize breast lesions. SUBJECTS AND METHODS: In 58 patients ranging in age from 25 to 64 years, breast MRI was performed as a problem-solving tool (29); for suspicious local relapse of the treated breast (6); to search for a primary breast cancer in patients with metastatic axillary lymph nodes (5); for local staging of breast cancer (5); breast implants (6); screening in high-risk patients (3), and differentiation between inflammation and inflammatory carcinoma (4). Sagittal fat-saturated T(2) and axial T(1) images were obtained before, and axial fat-saturated T(1) and dynamic sagittal fat-saturated T(1)-weighted images after contrast enhancement in a 1.5-tesla closed magnet. The diagnostic criteria were based on the morphology and kinetics of the lesion. Findings were validated by tissue sampling or radiological follow-up. RESULTS: Seventy breast lesions (25 malignant, 38 benign and 7 lesions detected by MRI only) were identified in the 58 patients. The sensitivity, specificity, and positive and negative predictive values of MRI in diagnosing malignant breast lesions were 96, 67, 71 and 95%, respectively, while the accuracy was 80%. CONCLUSION: This initial experience is comparable to other published data. Future plans for improving image spatial resolution and MR-guided procedures have been taken into consideration.

Accuracy of the Fischer scoring system and the Breast Imaging Reporting and Data System in identification of malignant breast lesions.

BACKGROUND AND OBJECTIVES: Fischer developed a scoring system in 1999 that made identifying malignant lesions much easier for inexperienced radiologists. Our study was performed to assess whether this scoring system would help beginners to accurately diagnose breast lesions on magnetic resonance (MR) imaging and to assess the correlation between the magnetic resonance mammography Breast Imaging Reporting and Data System (MRM BI-RADS) grade and the final diagnosis. PATIENTS AND METHODS: The lesion morphology and contrast kinetics of 63 masses in 41 patients were evaluated on MRI and accorded a MRM BI-RADS final assessment category using the Fischer scoring system. The accuracy was evaluated after the final diagnosis was obtained by tissue sampling and follow-up imaging. RESULTS: There were 25 malignant and 30 benign lesions. Eight lesions were seen by MRI only and we could not verify their pathology since we did not have MR-guided biopsy facilities at the time of the study. On MR mammography, the proven carcinomatous lesions were characterized as BI-RADS category V in 16 (64%), category IV in 7 (28%), and category III in 2 (8%) lesions. Benign lesions were graded as category V in 3 (10%), category IV in 6 (20%), and category III in 3 (10%), category II in 10 (33%) and category I in 8 (27%) lesions. The MRM BI-RADS category accurately predicted malignancy in 92% and a benign pathology in 70% of the lesions. The overlap between the MRM features of chronic inflammatory lesions and carcinomas resulted in a lower accuracy in diagnosing benign as compared to malignant lesions. CONCLUSION: The MRM BI-RADS lexicon using the Fischer scoring system is useful and has a high predictive value, especially for malignant breast lesions, and is easy to apply. Overlapping features between benign inflammatory and malignant lesions might yield a reduced accuracy in inflammatory pathologies.

Clinical characteristics and pathological classification of non-Hodgkin's lymphoma in Kuwait. Results of a collaborative study with the International Lymphoma Study Group (ILSG).

Kuwait was chosen by the International Lymphoma Study Group (ILSG) as one of the sites attending in the project on "Clinical characteristics and pathological classification of non Hodgkin's lymphoma (NHL) in the developing countries". The Kuwait study involved 206 cases of NHL, diagnosed, staged and treated in the Kuwait Cancer Control Center (KCCC). All cases were reviewed and reclassified independently by the pathologists of KCCC and the International Lymphoma Study Group (ISLG) using the latest World Health Organization (WHO) classification of neoplastic disease of the hematopoietic and lymphoid tissues. Immunophenotyping as to B- or T-cell was documented in all cases. Three main pathological entities (diffuse large B-cell lymphoma, follicular lymphoma, peripheral T-cell lymphoma) were identified and studied thoroughly. The intense cooperation between experts of the ISLG and pathologists of the KCCC proved that the WHO classification was fully reproducible in Kuwait. The high incidence of extranodal lymphomas (53%) observed in the KCCC may not be due to special ethnic or environmental conditions in Kuwait but rather be due to a selection of patients coming to our center.

Granular congenital cell tumor in the newborn: a case report.

This report represents a case of unusual large size congenital granular cell tumor appearing on the maxillary alveolar ridge in a newborn. Positive staining was found for S-100 protein. The authors discuss the clinical picture, histological findings, etiology and treatment of this rare lesion.