Thrombotic thrombocytopenic purpura is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large vWF multimers and the cleaving protease ADAM TS 131 (A disintegrin and metalloproteinase with a thrombospondin type 13). This disease was first described by Moshococowitz in 1924.2 It is clinically characterized by five typical syndromes: thrombocytopenia, microangiopathic hemolytic anaemia, renal impairment, fluctuating neurological abnormalities and fever. It is a rare disease and fatal in 50 to 80 per cent of cases5. TTP can be idiopathic or secondary. Here we are describing a case who developed fever, purpuric spot, jaundice, renal impairment and fluctuating neurological features. Initially it seems to us as a case of DIC, but later hematological evaluation reveals it as a case of TTP, as the patient fulfills the pentad criteria. Unfortunately we could not do the serum ADAM TS 13 level because of lack of facility in our country and in abroad it is costly.Bangladesh J Medicine Jan 2015; 26 (1) : 35-38
Amyloidosis and multiple myeloma are included in the same spectrum of clonal plasma cell disorder. Amyloidosis can present with localized deposits or manifest as systemic disease involving multiple organs such as kidney, heart, intestine. Free Ig subunits ,mostly light chains secreted by a single clone of B cells, are the cause of the most frequent and severe amyloidosis affecting the kidney. The incidence of AL amyloidosis is nine per 1 million populations per year. Amyloid deposists are found in approximately 10% of all patients with myeloma and in 20% of those with pure light chain myeloma[1].Here we are reporting a case who initially presented with generalized swelling and subsequently found to have AL amyloidosis involving multiple organs due to multiple myeloma.J Dhaka Medical College, Vol. 26, No.2, October, 2017, Page 173-177
Gitelman'?s syndrome, discovered in 1966 by Gittleman,Graham and Welt, is an autosomal recessive renal tubular disorder and characterized by hypokalemic metabolic alkalosis, hypomagnesaemia, hypocalciuria. This is a rare cause of hypokalemia which has an autosomal recessive inheritance. Here we are reporting a case of a 35 years old lady presented with recurrent attacks of quadriparesis due to hypokalemia and pain in multiple joints. Later we diagnosed the case as Gitelman?'s syndrome.DOI: http://dx.doi.org/10.3329/jom.v16i1.22407 J MEDICINE 2015; 16 : 59-60
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