Kandel Saroj, Budhathoki Nibash, Pandey Shanta, Bhattarai Bikash, Baqui Aam, Pandey Ramesh, Salhan Divya, Enriquez Danilo, Quist Joseph, Schmidt Frances M
Interfaith Medical Center, Brooklyn, NY, USA.
SAGE Open Med Case Rep. 2017 Jun 6;5:2050313X17713149. doi: 10.1177/2050313X17713149. eCollection 2017.
We present a rare case of pernicious anemia presented as multi-organ dysfunction syndrome, later found to have pseudo-thrombotic thrombocytopenic purpura.
An 86-year-old female presented with respiratory distress, altered mental status, acute renal failure and was intubated in emergency room. She was found to have severe anemia, thrombocytopenia, high lactate, high lactate dehydrogenase and low haptoglobin. Peripheral smear revealed multilobulated neutrophils with schistocytes, poikilocytes and anisocytes.
She was admitted to intensive care unit for altered mental status, multi-organ dysfunction syndrome with severe metabolic acidosis in setting of hemolysis. She was intubated and managed with intravenous antibiotics and blood transfusion. Patient improved significantly after blood transfusion. Lactic acid normalized, acute kidney injury resolved and mentation improved after transfusion. Laboratory investigation revealed low vitamin B12, high methylmalonic acid, high homocysteine, high lactate dehydrogenase, low haptoglobin, high anti-parietal antibody and high anti-intrinsic factor antibody. Patient was diagnosed with pernicious anemia and pseudo-thrombotic thrombocytopenic purpura with concomitant intramedullary hemolysis. Her hematological parameters and her clinical condition improved significantly after starting therapy with cyanocobalamin.
Pernicious anemia is a chronic disease with subtle presentation but may present as life-threatening complications. Hemolysis and pseudo-thrombotic thrombocytopenic purpura may present as multi-organ dysfunction syndrome which has dramatic response to appropriate therapy.
我们报告一例罕见的恶性贫血，最初表现为多器官功能障碍综合征，后来发现合并有假性血栓性血小板减少性紫癜。
一名86岁女性因呼吸窘迫、精神状态改变、急性肾衰竭在急诊室插管。发现她有严重贫血、血小板减少、高乳酸血症、高乳酸脱氢酶和低触珠蛋白。外周血涂片显示有分叶过多的中性粒细胞，伴有破碎红细胞、异形红细胞和大小不均一红细胞。
她因精神状态改变、多器官功能障碍综合征合并严重代谢性酸中毒及溶血入住重症监护病房。她接受了插管治疗，并使用静脉抗生素和输血进行管理。输血后患者明显好转。输血后乳酸水平恢复正常，急性肾损伤得到缓解，精神状态改善。实验室检查显示维生素B12水平低、甲基丙二酸水平高、同型半胱氨酸水平高、乳酸脱氢酶水平高、触珠蛋白水平低、抗壁细胞抗体水平高和抗内因子抗体水平高。患者被诊断为恶性贫血和假性血栓性血小板减少性紫癜，伴有骨髓内溶血。开始使用氰钴胺治疗后，她的血液学参数和临床状况明显改善。
恶性贫血是一种临床表现隐匿的慢性病，但可能会出现危及生命的并发症。溶血和假性血栓性血小板减少性紫癜可能表现为多器官功能障碍综合征，对适当治疗有显著反应。
