Peripheral Digit Ischemic Syndrome Can Be a Manifestation of Postoperative Thrombotic Thrombocytopenic Purpura

Abstract

: In addition to common dysfunction of the brain and kidney, thrombotic thrombocytopenic purpura (TTP) may present with atypical clinical features due to the involvement of other organs such as the lung, pancreas, heart, eye, and skin. We have also observed the unusual presentation of peripheral digit ischemic syndrome (PDIS) in some patients with postoperative TTP. To clarify this relationship between TTP and PDIS, the hematologic data from the medical records of patients with known diagnoses of thrombotic microangiopathy (TM) were examined in a single institution. A total of 94 patients were diagnosed with TM. Among these patients, PDIS developed in six patients and in all these patients PDIS occurred with postoperative TTP. Four patients also had acute respiratory distress syndrome (ARDS). Because of delayed diagnosis of TTP, only two patients survived and four died. One patient responded to plasma exchange and survived, and another patient recovered from postoperative TTP without plasma exchange. However, both patients required the amputation of multiple digits. In conclusion, PDIS is another atypical manifestation of TTP and has occurred exclusively in patients with postoperative TTP in this series. Once PDIS developed, the prognosis was poor and amputation of digits was needed in surviving patients. Early recognition of this atypical manifestation of TTP is essential for a favorable outcome.

Postsurgical thrombotic microangiopathy: Case series and review of the literature

“…Unlike reports in the literature, our clinical cohort did not contain patients presenting with clinical manifestations such as peripheral ischemia leading to limb loss. 17 Similar to our cohort, most patients with postsurgical TMA from the literature review were female and 70% presented with TMA within one week after surgery. The most common reported surgery to precipitate postoperative TMA from the literature was cardiovascular surgery.…”

“…The majority of patients presented with renal and liver injury while 40% of patients presented with neurologic manifestations. Unlike reports in the literature, our clinical cohort did not contain patients presenting with clinical manifestations such as peripheral ischemia leading to limb loss . Similar to our cohort, most patients with postsurgical TMA from the literature review were female and 70% presented with TMA within one week after surgery.…”

Post‐appendectomy thrombotic thrombocytopenic purpura: A case report and review of the literature

“…A National Library of Medicine search identified only eight cases of postoperative TTP after abdominal or general surgical procedures: four cases after bowel resection, two after cholecystectomy, and two post-hysterectomy (Table I) [11][12][13][14][15][16][17]. Of the four cases occurring after bowel resection, two were performed for colon cancer and one for bowel adhesions [11][12][13][14].…”

“…TTP occurring in the nontransplant postsurgical setting has only rarely been reported, but has been best described after cardiothoracic and vascular surgeries [9,10]. Case reports of TTP occurring after noncardiovascular surgeries are reported even less frequently, but have been described after abdominal and orthopedic surgeries as summarized in Table I [ [11][12][13][14][15][16][17]. The etiology of postoperative TTP remains uncertain, but may be due to endothelial damage and the subse-quent release of high molecular weight vWF multimers.…”

Redefining the Syndromes of Thrombotic Microangiopathy

“…Why is the consideration of ‘schistocyte negative TMS’ so important? With the exception of TMS associated with cancer (12) and transplants (13, 14), TTP‐like syndromes frequently respond to therapeutic plasma exchange if the diagnosis is established at an early stage and the treatment is initiated in a timely fashion (3, 4, 15–18). Recognizing the diagnosis can be life‐saving.…”