Recurrent thrombotic thrombocytopenic purpura-like syndrome as a paraneoplastic phenomenon in malignant peritoneal mesothelioma: a case report and review of the literature

Our plain-English summary of Recurrent thrombotic thrombocytopenic purpura-like syndrome as a paraneoplastic phenomenon in malignant peritoneal mesothelioma: a case report and review of the literature, by F. Socola, A. Loaiza-Bonilla, E. Bustinza-Linares, et al (Case Reports in Oncological Medicine, 2012).

The study

• Authors: F. Socola, A. Loaiza-Bonilla, E. Bustinza-Linares, R. Correa, J.D. Rosenblatt
• Journal: Case Reports in Oncological Medicine
• Published: 2012;2012:619348
• Cited by: 6 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

We report the case of an African American male with no significant past medical history presenting with recurrent, rapidly relapsing episodes of thrombotic thrombocytopenic purpura (TTP) despite aggressive treatment with several lines of treatment. Incidentally, these episodes were associated with severe abdominal pain which eventually developed into acute abdomen and prompted exploratory laparotomy, revealing diffuse carcinomatosis with a tumor located on the left pelvis that was encasing the distal sigmoid colon. Pathology made a final diagnosis of peritoneal mesothelioma. TTP-like syndrome (TTP-LS) has been described as a paraneoplastic phenomenon in several malignancies but never before in the setting of malignant mesothelioma. Paraneoplastic TTP-like syndrome has historically been associated with a dismal prognosis and particular clinical and laboratory abnormalities described in this paper. It is of utmost importance to make a prompt determination whether TTP is idiopathic or secondary to an underlying condition because of significant differences in their prognosis, treatment, and response. This paper also reviews the current literature regarding this challenging condition.

In plain English

This article was a case report with a review of the literature. It described a 61-year-old African American man who came to the emergency department with six months of worsening abdominal pain, loss of appetite and weight loss. He was diagnosed with thrombotic thrombocytopenic purpura (TTP), a life-threatening disorder with low platelet counts and breakdown of red blood cells. He was treated with steroids, plasma exchange and plasma infusions. Early testing, including pleural biopsies, found no cancer. The TTP relapsed repeatedly despite further treatment, including rituximab and CVP chemotherapy. Exploratory surgery for severe abdominal pain found widespread tumor in the abdomen, and pathology diagnosed mesothelioma, which the authors called peritoneal mesothelioma. The tumor could not be removed. The illness was then diagnosed as a paraneoplastic TTP-like syndrome, meaning it was linked to the cancer. After four weeks of cisplatin plus pemetrexed there was no improvement, and he was transferred to hospice. The authors concluded that, to their knowledge, this was the first reported case of this syndrome with peritoneal mesothelioma. The article did not mention asbestos or any other exposure.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report of one patient, with a review of the literature
• Who was studied: One 61-year-old African American man seen at the authors' institution (Jackson Memorial Hospital/Sylvester Comprehensive Cancer Center, Miami). He had six months of worsening abdominal pain, loss of appetite and unintended weight loss. No dates are given.
• Exposure: Not reported. The article does not mention asbestos or any other occupational or environmental exposure.
• Main results:
  • At first presentation the patient was diagnosed with TTP. ADAMTS 13 activity was 14% (low).
  • Initial workup, including pleural biopsies by video-assisted thoracoscopic surgery, found no evidence of malignancy.
  • The TTP relapsed repeatedly after short intervals. At one relapse, ADAMTS13 activity was less than 10%.
  • Rituximab followed by CVP chemotherapy (given for possible low-grade lymphoma) produced no significant response after two cycles.
  • Exploratory laparotomy found diffuse carcinomatosis with a tumor in the left pelvis encasing the distal sigmoid colon.
  • Pathology reported mesothelioma. The tumor cells were positive for calretinin and D240 and negative for CEA and MOC31. The disease was considered nonresectable.
  • The patient received palliative cisplatin plus pemetrexed. After four weeks there was no improvement, and he was transferred to hospice.
• The authors' conclusion: The authors concluded that, to the best of their knowledge, this was the first reported case of paraneoplastic TTP-like syndrome secondary to peritoneal mesothelioma. They stated that the persistently low ADAMTS-13 activity suggests a different disease mechanism from idiopathic TTP. They also stated it is important to determine promptly whether TTP is idiopathic or secondary to an underlying condition.
• Limitations the authors state:
  • The authors noted that their suggestion (treating the underlying cancer may be the best option in paraneoplastic TTP-like syndrome) needs further study. They added that the rarity and underreporting of such cases make a prospective study difficult.

Topics

Medical terms in this study, explained in our Lexicon: Peritoneal Mesothelioma, Laparotomy, Pathology, Mesothelioma, Prognosis, Idiopathic.

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