Light chain deposition disease presenting as cholestatic jaundice: a case report

Light-chain deposition disease [LCDD] is characterized by tissue deposition of the immunoglobulin light chains in multiple organs. These deposits appear similar to amyloid on routine sections, but differ in their staining properties and ultrastructural appearance. The deposits of LCCD are non -Congophilic and do not exhibit a fibrillar ultrastructure; while, the proteinaceous substance seen in primary amyloidosis is Congo red positive and fibrillar. One of the most common organs to be involved in LCDD is the kidney. Earlier reports on cases of LCDD have mostly shown simultaneous liver and renal involvement, there are very few cases in the literature describing LCDD of the liver without renal involvement. This report describes a patient who presented with severe cholestatic jaundice and liver cell failure with normal renal function

Omenn's syndrome. A rare primary immunodeficiency disorder

Over the last 17 years different forms of severe combined immunodeficiency have been diagnosed at Sultan Qaboos University Hospital, Muscat. Omenn's syndrome is a rare autosomal recessive form of severe combined immunodeficiency. We report a 6 weeks old Omani infant who presented with the characteristic clinical and immunological phenotype of Omenn's syndrome. We take the opportunity to discuss and review the immunological aspect of this rare syndrome

Nodular lymphocyte predominant Hodgkin's lymphoma presenting as severe hypercalcaemia: a case report

Nodular lymphocyte predominant Hodgkin's lymphoma [NLPHL] is a recently described type of Hodgkin's lymphoma [HL] and accounts for 5-6% of all the cases of HL. Here we report the case of an elderly man who presented to Sultan Qaboos University Hospital, Oman, with severe hypercalcemia, and was diagnosed to have stage IV NLPHL. Although the incidence of hypercalcemia is estimated to be between 1-5% in classical HL, to our knowledge this is the first report of NLPHL presenting with severe hypercalcemia. The patient responded to the anti-CD20 monoclonal antibody, Rituximab, and has been in clinical remission for more than 3 years

Are scintigraphy and ultrasonography necessary before fine-needle aspiration cytology for thyroid nodules?

To evaluate the effi cacy of scintigraphy, ultrasound and fi ne-needle aspiration in thyroid nodules and to establish the best diagnostic pathway in detecting thyroid cancer. Two hundred and sixteen patients with thyroid nodules were examined using high-resolution ultrasonography, 99mTc thyroid scintigraphy and ultrasound-guided fi ne-needle aspiration. Of these, 113 patients subsequently underwent thyroidectomy. The remaining 103 were followed up for two years without any evidence of malignancy. Cytopathology classifi ed 71% of the aspirate as benign, 3% as positive for malignancy, 21% as suspected neoplasia and 5% as unsatisfactory. Fine- needle aspiration cytology had a sensitivity of 87.5% and specifi city of 80%. On ultrasound 33% of malignant nodules were hypo-echoic and on scintigraphy 16% of solitary cold nodules were malignant. Neither test could reliably diagnose thyroid cancer. Ultrasound-guided fi ne-needle aspiration cytology should be the fi rst test performed in euthyroid patients with a thyroid nodule. Scintigraphy and ultrasound imaging should be reserved for follow-up studies and patients who have suppressed levels of thyroid stimulating hormone