Abstract:Microangiopathic hemolytic anemia (MAHA) can be an uncommon presentation of an underlying malignancy, most often due to signet-ring cell carcinoma (SRCC). Additionally, pure SRCC in a breast primary-tumor comprises <2% of all breast cancers (Shin SY, Park H, Chae SW, Woo HY. Microangiopathic hemolytic anemia as the first manifestation of metastatic signet-ring cell carcinoma of unknown origin: a case report and review of literature. Kor J Lab Med 2011;31:157–61). To the best of our knowledge, the combination o… Show more
“…90% of the patients did not survive beyond 4 weeks. Since the publication of this case series, signet ring cell carcinomas of intrahepatic bile duct, breast and jejunum have been reported with MAHA and poor outcomes on chemotherapy [22][23][24][25][26][27][28].…”
Microangiopathic hemolytic anemias (MAHA) are secondary to damage of RBCs because of endothelial vascular damage of blood vessels leading to hemolysis. MAHAs are characterized by negative coombs test and are associated with several etiologies that include can be either hereditary complement or ADAMTS13 deficiency or sepsis or malignancy. Cancer associated MAHA (CA-MAHA) is a rare and is seen in patients with advanced metastatic disease. CA-MAHA has significant mortality rate and chemotherapy is the only therapeutic option, however overall survival is poor. Here we, present a rare case of CA-MAHA secondary to metastatic signet ring cell carcinoma with an unknown primary. In patients, when the cause of progressive MAHA is unknown, the possibility of cancer associated MAHA must be considered and a comprehensive work up for an underlying malignancy must be done.
“…90% of the patients did not survive beyond 4 weeks. Since the publication of this case series, signet ring cell carcinomas of intrahepatic bile duct, breast and jejunum have been reported with MAHA and poor outcomes on chemotherapy [22][23][24][25][26][27][28].…”
Microangiopathic hemolytic anemias (MAHA) are secondary to damage of RBCs because of endothelial vascular damage of blood vessels leading to hemolysis. MAHAs are characterized by negative coombs test and are associated with several etiologies that include can be either hereditary complement or ADAMTS13 deficiency or sepsis or malignancy. Cancer associated MAHA (CA-MAHA) is a rare and is seen in patients with advanced metastatic disease. CA-MAHA has significant mortality rate and chemotherapy is the only therapeutic option, however overall survival is poor. Here we, present a rare case of CA-MAHA secondary to metastatic signet ring cell carcinoma with an unknown primary. In patients, when the cause of progressive MAHA is unknown, the possibility of cancer associated MAHA must be considered and a comprehensive work up for an underlying malignancy must be done.
“…So far, only a few dozen cases of hemolytic anemia accompanying breast cancer have been described in the literature. To the best of our knowledge, about 60 cases of Cr-mAHA associated with breast cancer were found in the mED-lInE via Pubmed database [1][2][3][4][5][6][7]. The survival in these cases from the diagnosis of mAHA did not exceed 3 years [1,2].…”
Cancer-related microangiopathic hemolytic anemia is rarely recognized as a paraneoplastic syndrome with a very poor prognosis in cancer patients. The treatment and prognosis are significantly different from that in other thrombotic microangiopathies, such as thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. The case of described patient demonstrates the diagnostic difficulties in diagnosing the causes of hemolytic anemia in patient with breast cancer and appropriate treatment.
A 53-year-old man presented with a 3-month history of progressive, non-productive cough followed by occasional swelling of the face and upper extremities. Physical examination on admission revealed prominent superficial vessels at the neck and upper extremity swelling. Bronchoscopy revealed the superior segment of the right lower lobe was narrow but without visible mass; cell block and biopsy done revealed signet-ring cell carcinoma with an immunohistochemistry pattern favouring the primary site of malignancy as either gastric or of the pancreaticobiliary tree. However, CT scan of the abdomen and pelvis with triple contrast revealed only slight gastric wall thickening; the pancreas was unremarkable. The patient underwent radiotherapy with noted improvement of symptoms. He was then discharged with plans for further work-up on an outpatient basis. This work highlights the importance of a comprehensive histopathological and immunohistochemical work-up which can help predict a site of origin to be able to provide the appropriate management.
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