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Primary Amyloidosis - In a Case with Normal Plasma Cell Counts

机译:原发性淀粉样变性-血浆细胞计数正常的情况

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摘要

Amyloidosis is a group of disease that is characterized by the deposition of extracellular abnormal proteinaceous material (amyloid), in various organs. Amyloidosis involving the liver is common and the radiological findings are often nonspecific. We present the case of a 40-year-old female who presented with abdominal pain. Ultrasound abdomen was reported as massive hepatomegaly with diffuse liver parenchymal disease. Bone marrow aspiration showed normomegaloblastic erythroid hyperplasia and plasma cells were within normal limits (5%). Also, amorphous, eosinophilic fragmented to smudgy material within the interstitium of cell trails was seen. Bone marrow biopsy and liver biopsy also showed similar kind of homogenous eosinophilic material. Both liver biopsy and bone marrow biopsy were subjected to special stains which confirmed the presence of amyloid. The patient did not have clinical or laboratory findings suggestive of any other organ involvement. Thus, we conclude that clinical and imaging presentations of amyloidosis are often nonspecific, hence biopsy is always required to confirm the diagnosis. Amyloid deposits on bone marrow aspiration are a rare occurrence and are often missed. It is an unusual sighting with very few studies mentioning its occurrence.
机译:淀粉样变性病是一组疾病,其特征在于细胞外异常蛋白物质(淀粉样蛋白)在各种器官中的沉积。涉及肝脏的淀粉样变性病很常见,而且放射学结果常常是非特异性的。我们介绍了一名40岁女性腹部疼痛的病例。据报道,腹部超声是巨大的肝肿大,伴有弥漫性肝实质疾病。骨髓穿刺显示正常巨幼红细胞增生,浆细胞在正常范围内(5%)。此外,还观察到无定形的嗜酸性粒细胞碎片化成细胞痕迹间的污迹。骨髓活检和肝活检也显示出相似类型的均质嗜酸性物质。肝活检和骨髓活检均经过特殊染色,证实存在淀粉样蛋白。患者没有任何暗示其他器官受累的临床或实验室检查结果。因此,我们得出结论,淀粉样变性病的临床表现和影像学表现通常是非特异性的,因此始终需要进行活检以确认诊断。骨髓穿刺术中的淀粉样蛋白沉积很少见,经常被遗漏。这是一个不寻常的发现,很少有研究提到它的发生。

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