Blue kidney in a pale patient-a case for a causal association between renal haemosiderosis in paroxysmal nocturnal haemoglobinuria and chronic kidney disease.

A 50-year-old man presented with pancytopenia and chronic renal impairment. He had evidence of intravascular haemolysis. The direct antiglobulin (Coomb's) test was negative. Paroxysmal nocturnal haemoglobinuria (PNH) was diagnosed by the Ham acid haemolysis test. There were no other clinical risk factors that could be implicated in chronic kidney disease (CKD). A renal biopsy revealed extensive haemosiderosis affecting proximal tubular cells and associated interstitial fibrosis as well as tubular atrophy. No glomerular or vascular lesions were seen. These findings strengthen the case for a causal relationship between renal haemosiderosis in PNH and CKD.

Retroperitoneal lymphangiomyomatosis diagnosed by fine needle aspiration: a case report.

BACKGROUND: Lymphangiomyomatosis is a rare condition affecting women of childbearing age. It is characterized by an abnormal proliferation of smooth muscle cells around lymphatics, giving rise to blockage of the large lymphatics, including the thoracic duct, and resulting in chylothorax and/or chyloascitis. The lung is the most common site of involvement. Retroperitoneum and lymph nodes can be also involved. CASE: A 40-year-old woman presented with lower urinary tract symptoms after a history of trauma and was found to have a retroperitoneal mass. Fine needle aspiration cytologic examination of the milky fluid aspirated from the mass revealed a few cohesive, 3-dimensional clusters of medium-sized cells with scanty cytoplasm, and ovoid and hyperchromatic nuclei. The background contained numerous mature lymphocytes. Laparoscopy revealed a multicystic mass filled with milky fluid. Histologic examination confirmed the cytologic diagnosis of lymphangiomyomatosis. CONCLUSION: Fine needle aspiration of lymphangiomyomatosis can be performed if cohesive clusters and a lymphoid background are present in chylous-type fluid and provided that adequate clinical information is available.

AIDS-related progressive multifocal leukoencephalopathy.

A case of progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection with a fatal outcome is presented. The disease has not been reported from our region before. The patient presented initially with hemiparesis and non-enhancing lesion on computed tomography scan that was thought to be an infarct. After a delay of 4 months, the diagnosis was made by brain biopsy. This delay probably contributed to his unfortunate outcome. The case is described, and methods of diagnosis and treatment are discussed.