Thrombotic Thrombocytopenic Purpura and Bone Marrow Necrosis Associated with Disseminated Gastric Cancer

Excerpt

Thrombotic thrombocytopenic purpura (TTP) is a disseminated form of thrombotic microangiopathy. Severe deficiency of the von Willebrand factor (VWF)-cleaving protease, now denoted ADAMTS-13, is known to be responsible for the formation of platelet thrombi in the microvasculature which is believed to be the pathophysiological hallmark of acute idiopathic TTP [1]. The classic pentad of clinical findings consists of fever, microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fluctuating neurologic impairment, and renal dysfunction [2]. Although most cases are idiopathic, the association of TTP with malignancy is well recognized [3]. About half of malignancy-associated TTP pertains to gastric carcinoma [4]. It usually occurs at the terminal stage of cancer and is extremely rare as the initial presentation of the disease. Bone marrow necrosis (BMN) is another relatively uncommon entity, partly because it is underdiagnosed. It is defined pathologically as the necrosis of hematopoietic tissue and medullary stroma in large areas of the bone marrow, with preservation of bone [5]. We report here on a patient with the rare association of TTP and BMN displayed as the first manifestation of an advanced gastric cancer. …