Podder Sidhertha, Cervates Jose, Dey Bimalangshu R
Department of Internal Medicine, Jamaica Hospital Medical Center, Jamaica, New York, USA.
Department of Hematology-Oncology, Jamaica Hospital Medical Centre, Jamaica, New York, USA.
BMJ Case Rep. 2015 Oct 13;2015:bcr2015211989. doi: 10.1136/bcr-2015-211989.
Pernicious anaemia is an autoimmune disease caused by intrinsic factor antibody; it leads to vitamin B12 deficiency and is marked by ineffective erythropoiesis. Haematological features reveal macrocytosis, hyperchromasia and hypersegmented neutrophils. Schistocytes are typically seen in microangiopathy, such as in thrombotic thrombocytopaenic purpura (TTP)/haemolytic uraemic syndrome or disseminated intravascular haemolysis (DIC). We report a case of a patient with severe anaemia who presented to the emergency room. Peripheral smear revealed macrocytosis, hypersegmented neutrophils and marked schistocytosis. The patient also had high reticulocyte count with high serum lactate dehydrogenase, elevated D-dimer, low fibrinogen and low haptoglobin. Vitamin B12 level came back low and the presence of intrinsic factor antibody confirmed pernicious anaemia. ADAMTS13 level was noted to be mildly reduced, which raised the suspicion of the association of acquired TTP with pernicious anaemia. Acquired TTP is another autoimmune disorder and its association with pernicious anaemia needs further evaluation.
恶性贫血是一种由内因子抗体引起的自身免疫性疾病；它会导致维生素B12缺乏，并以无效红细胞生成作为特征。血液学特征表现为大细胞性、高色素性和中性粒细胞核分叶过多。裂红细胞通常见于微血管病，如血栓性血小板减少性紫癜（TTP）/溶血尿毒综合征或弥散性血管内溶血（DIC）。我们报告一例严重贫血患者到急诊室就诊的病例。外周血涂片显示大细胞性、中性粒细胞核分叶过多和明显的裂红细胞症。该患者还具有高网织红细胞计数、高血清乳酸脱氢酶、D-二聚体升高、纤维蛋白原降低和触珠蛋白降低。维生素B12水平偏低，内因子抗体的存在确诊为恶性贫血。注意到ADAMTS13水平轻度降低，这增加了获得性TTP与恶性贫血相关联的怀疑。获得性TTP是另一种自身免疫性疾病，其与恶性贫血的关联需要进一步评估。
