TS: A Quiet Storm

There is an ongoing quest for information on this rare and fast-paced form of deep vein thrombosis.

Last month was Deep Vein Thrombosis (DVT) Awareness Month, and during that time I had the opportunity to attend a thrombosis forum and listen to various important updates. I was reminded that although it is important to keep up with the novel approaches to patient care for now- established disease states, what about those lesser-known disease states? I thought we could use the month of April to sound off on a rarer, fast-paced form of DVT presentation—the Thrombotic Storm (TS).

First described by Dr. Craig Kitchens, TS is a rare but serious, and often life-threatening, syndrome whereby patients may experience progressive serial thrombosis over a period of days to weeks in atypical or “unusual” sites.¹ The literature describing this disorder is quite limited and, as such, the clinical characteristics and manifestations of it are not well defined.

A Big-Picture Diagnosis

While much progress in the way of thrombosis management, treatment, and prevention has occurred in recent years, there is much “gray” area that exists in terms of why clotting occurs in a subset of patients. Similar to other complex processes such as disseminated intravascular coagulation (DIC) and Trousseau’s syndrome, TS is recognized based on a pattern of events or behaviors seen at the patient bedside.¹ The syndrome cannot be defined solely on a solitary laboratory result or clinical finding. It is a “big picture” diagnosis; therefore, it may only become apparent after much physician speculation and time spent piecing together a patient’s clinical picture. It calls for the use of a health care provider’s clinical senses—it is the look, feel, and listen approach—and professional experience.

To date, TS investigators have compiled 10 published case reports representing the clinical manifestations thought to be associated with TS.² Here is what we know: the differential diagnosis upon initial presentation can be as extensive as those found in an episode of House, as there is not 1 unique characteristic. One can begin the differentiation from other thromboses, as its presentation has the tendency to have an accelerated onset of days to weeks, occurs in multiples in “unusual” locations—not the more common lower extremity (calf) or lung presentation—and clot formation is progressive and often recurrent in those younger than 55 years.

In several of the described case reports, the occurrence can be arterial or venous in nature and is usually the result of a hypercoaguable trigger.² The case reports associate the triggers as being any 1 of the following: surgery, trauma, pregnancy, inflammation, infection, or medications (oral contraceptives was noted in 1 case). The presence of antiphospholipid antibodies seems to be a commonality among some, but not all, of the patients described, thus it is thought that catastrophic antiphospholipid syndrome and TS may be related.^2,3 There was no association with any particular race. It was noted that 8 out of 10 of the cases affected women. This finding can not be explained at this time and whereas the group is quite limited, this information is confounding. Patients ranged between the ages of 14 and 38 years; therefore it is arbitrarily thought that this phenomenon may be associated with individuals under 40 or 50 years of age.²

Treatment

Treatment of TS warrants a look at each patient’s presentation and can be controversial, as is the ongoing theme for management of patients on anticoagulation. If caught early enough, these patients can typically be managed with an initial course of aggressive, uninterrupted parenteral anticoagulation with a favorable prognosis. The positive response to injectable anticoagulation bridged to an oral agent, often warfarin, distinguishes this disease from DIC and Trousseau’s syndrome.¹ Some patients will be candidates for short or long-term oral anticoagulation relative to their presentation and existing risk factors.

There is ongoing research and a quest to build a foundation of knowledge via the use of genomic technology by the Thrombotic Storm Study Group. Identification of familial or genetic factors may help reveal those patients with a predisposition to this disorder.² Investigators across various institutions and practicing within a variety of specialties are collaborating with the hope of constructing inclusion and exclusion criteria that may better facilitate the diagnosis, treatment, and possible prevention of TS.

Dr. Stevens is an advanced practice anticoagulation pharmacist for the Partners Healthcare System and an adjunct clinical assistant professor of pharmacy at Northeastern University’s Bouvé College of Health Sciences in Boston, Massachusetts. This column’s information is based on current studies and references, but it may be updated without notice with newer studies or with different populations.

References

• Kitchens CS. Thrombotic storm: when thrombosis begets thrombosis. Am J Med 1998;104:381-385.
• Kitchens CS, Erkan D, Brandão LR, et al. Thrombotic storm revisited: preliminary diagnostic criteria suggested by the thrombotic storm study group. Am J Med. 2011;124:290-296.
• Erkan D, Asherson RA, Espinosa G, et al. Long term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis. 2003;62:530-533.