Microangiopathic Hemolytic Anemia as the First Manifestation of Metastatic Signet Ring Cell Carcinoma of Unknown Origin: A Case Report and Review of Literature

Abstract

Microangiopathic hemolytic anemia (MAHA) occurs occasionally as a paraneoplastic syndrome in some solid tumors, but MAHA accompanied by signet ring cell carcinoma of an unknown origin is very rare. In this study, we present the case of an 80-yr-old man who was admitted to the hospital because of a 1-month history of lower back pain and dyspnea. He was diagnosed with MAHA on the basis of the laboratory findings that revealed anemia with schistocytes, decreased haptoglobin levels, and a negative direct Coombs' test. Bone marrow examination, which was performed because of the progression of anemia, revealed bone marrow metastases of signet ring cell carcinoma with extensive bone marrow necrosis. However, the primary origin of this signet ring cell carcinoma was not found. When the cause of progressive MAHA is unknown, the possibility of cancer-associated MAHA must be excluded by performing additional tumor workup, including the detection of tumor markers, gastric and colorectal endoscopic examinations, bone marrow examinations, and positron emission tomography-computed tomography or bone scans.

Signet ring cell carcinoma’s myelophthisis

“…Gastric carcinoma invading medullary tissue, particularly the signet ring cell type, is a rare event and is frequently associated with poor prognosis 1. It can be associated with severe microangiopathic haemolytic anaemia with a complex and multifactorial physiopathological mechanism that is not yet fully understood 1 2…”

“…The only effective treatment seems to be chemotherapy, although in some anecdotical cases, plasmapheresis has been used 1 2. However, despite treatment, survival does not usually exceed 2 months after the diagnosis has been made 1 2…”

“…However, despite treatment, survival does not usually exceed 2 months after the diagnosis has been made 1 2…”

Metastatic signet ring cell carcinoma of unknown primary origin: a case report and review of the literature

“…While 90% of SRCC tumors arise from the stomach, breast, or colon, almost every organ is a potential primary site (3). It is characterized by the histological appearance of signet ring cells in which the nucleus is pushed to the periphery by abundant intracytoplasmic mucin (4). It poses diagnostic and therapeutic difficulties as they tend to be aggressive and present at an advanced stage.…”

Isolated Hemolytic Anemia: An Unusual Manifestation of Occult Malignancy

“…Since this process is associated with metastatic involvement of bone marrow, the culprit disease is typically advanced and readily apparent, but rarely MAHA is the presenting feature of an undiagnosed malignancy. 12,13 In some instances a patient will be misdiagnosed with primary TTP, and subsequently found to harbor an underlying cancer 14 . In 75% of cases, cancer-associated MAHA will respond to medical therapy directed at the underlying tumor 8 …”