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      A Metastatic Signet Ring Cell Carcinoma Presented as Acquired Thrombotic Thrombocytopenic Purpura: A Case Report

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          Abstract

          Microangiopathic hemolytic anemia (MAHA) may occur as a paraneoplastic syndrome in some solid tumors, but MAHA accompanied by signet ring cell carcinoma (SRCC) of an unknown origin is very rare. We report a patient who presented with an acute onset of Coombs negative hemolytic anemia and frequent schistocytes in the peripheral blood smear which are typical for MAHA as initial presentation of metastatic SRCC. Our patients fulfilled the criteria of thrombotic thrombocytopenic purpura (TTP) and received the specific treatment for TTP without improvement.

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          Syndromes of thrombotic microangiopathy.

          James N. George, Carla Nester (2014)
          This review article covers the diverse pathophysiological pathways that can lead to microangiopathic hemolytic anemia and a procoagulant state with or without damage to the kidneys and other organs.
          Article 0 comments Cited 336 times – based on 0 reviews     Review now
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            Cytokeratins 20 and 7 as biomarkers: usefulness in discriminating primary from metastatic adenocarcinoma.

            T. Tot (2002)
            Metastatic adenocarcinoma from an unknown primary site is a common clinical problem. The use of cytokeratins 20 (CK20) and 7 (CK7) was proposed to identify the primary sites in this situation. In this review, the results of 29 studies were summarised and the difficulties of data comparison described. Most tumours retained the CK20 phenotype during metastasis, but lung, non-mucinous ovarian, and gastric adenocarcinomas showed statistically significant differences in CK20 expression in the reported primary and metastatic cases. Ductal breast carcinomas, lung and non-mucinous ovarian adenocarcinomas showed significant differences in CK7 expression when primary and metastatic tumours were compared. CK20 positivity alone indicates metastatic spread of adenocarcinoma in several organs. CK7 negativity is consistent with metastases of adenocarcinomas in the lungs, ovaries, liver or serous membranes. CK20/7 phenotyping of adenocarcinomas is a useful diagnostic tool if based on algorithmic and probabilistic approaches and a detailed database.
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              Cancer awareness in atypical thrombotic microangiopathies.

              Dominique Valla, L Armand-Lefevre, … (2009)
              To specify the clinical and biological characteristics of thrombotic microangiopathies (TMAs) associated with a recent diagnosis of cancer. PATIENTS AND Methods. Multicenter study involving 14 national centers. Cross-sectional analysis of 20 patients with cancer-associated TMAs included in our national registry from October 2000 to July 2007. Patients were also compared with 134 adult patients with an acquired idiopathic TMA by univariate analysis. Patients with a cancer-associated TMA typically displayed severe weight loss, dyspnea, bone pain, as well as disseminated intravascular coagulopathy and massive erythromyelemia (75%, 55%, 50%, 41%, and 85% of cases, respectively). By contrast, these features were observed with a much lower incidence in patients with an idiopathic TMA (8.9%, 19.7%, 0.8%, 7.1%, and 17.5%, respectively). Moreover, median platelet count was higher (48 x 10(9)/l; range, 21-73 x 10(9)/l versus 19 x 10(9)/l; range, 10-38 x 10(9)/l, respectively) and median serum creatinine level was lower (74 microM [range, 68-102] versus 113 microM [range, 80-225], respectively). The activity of the specific von Willebrand factor-cleaving proteinase ADAMTS13 was detectable in 14/17 studied patients. Platelet count improvement was observed in only seven patients and paralleled the response to chemotherapy. Prognosis of patients with cancer-associated TMAs was very poor, with a 30-day and 2-year mortality rate of 50% and 95%, respectively. Cancer-associated TMAs display specific features at onset that should prompt investigation of an underlying disseminated malignancy. In this context, chemotherapy rather than plasma is mandatory since TMA prognosis parallels that of cancer.
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Hematol (Brossard)
                Journal ID (iso-abbrev): J Hematol (Brossard)
                Journal ID (publisher-id): Elmer Press
                Title: Journal of Hematology
                Publisher: Elmer Press
                ISSN (Print): 1927-1212
                ISSN (Electronic): 1927-1220
                Publication date (Print): May 2018
                Publication date (Electronic): 10 May 2018
                Volume: 7
                Issue: 2
                Pages: 72-75
                Affiliations
                [a ]Hematology Unit, Oncology Center, Mansoura University, Egypt
                [b ]Medical Oncology Unit, Oncology Center, Mansoura University, Egypt
                [c ]Department of Pathology, Faculty of Medicine, Mansoura University, Egypt
                Author notes
                [d ]Corresponding Author: Noha Eisa, Hematology Unit, Oncology Center, Faculty of Medicine, Mansoura University, Egypt. Email: drNohaessa@ 123456gmail.com
                Article
                DOI: 10.14740/jh386e
                PMC ID: 7155867
                SO-VID: 0708bf56-1eca-4e33-8876-55d26bef3b5a
                Copyright © Copyright 2018, Eisa et al.
                License:

                This article is distributed under the terms of the Creative Commons Attribution Non-Commercial 4.0 International License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 5 February 2018
                Date accepted : 1 March 2018
                Categories
                Subject: Case Report

                Keywords: microangiopathic hemolytic anemia,signet ring cell carcinoma,thrombotic thrombocytopenic purpura
                Data availability:
                Keywords: microangiopathic hemolytic anemia, signet ring cell carcinoma, thrombotic thrombocytopenic purpura

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