Phlegmasia alba dolens and phlegmasia cerulea dolens

Morales MH, Leigh CL, Simon EL. COVID-19 infection with extensive thrombosis: A case of phlegmasia cerulea dolens. Am J Emerg Med. 2020 May 15. Epub ahead of print.

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“Cytokine storm has been implicated in COVID-19 and associated with severe infection [4], allowing for a focus on cytokine and other proinflammatory markers. It is suspected that the extensive release of cytokines causing a proinflammatory state may play a role in thrombus formation [5]. Tanaka et al. reported that IL-6 could activate the coagulation cascade [6], increasing the risk of thrombosis and complication. Our patient did have an elevated level of IL-6, in addition to hypertension and elevated CRP, which are all independent risk factors for increased severity of COVID-19 infection [7].

Helms et al. found that 50 of 57 patients had positive lupus anticoagulant and antiphospholipid (aPL) antibodies [1], both of which have been associated with thrombotic complications.”

Shackford SR. (2018). Venous Disease. In: Abernathy’s Surgical Secrets, 7th ed.: p. 357.

What is the difference between phlegmasia alba dolens and phlegmasia cerulea dolens? 

“These two entities occur following iliofemoral venous thrombosis, 75% of which occur on the left side presumably because of compression of the left common iliac vein by the overlying right common iliac artery (May-Thurner syndrome). Iliofemoral venous thrombosis is characterized by unilateral pain and edema of an entire lower extremity, discoloration, and groin tenderness. In phlegmasia alba dolens (literally, painful white swelling), the leg becomes pale. Arterial pulses remain normal. Progressive thrombosis may occur with propagation proximally or distally and into neighboring tributaries. The entire leg becomes both edematous and mottled or cyanotic. This stage is called phlegmasia cerulea dolens (literally, painful purple swelling). When venous outflow is seriously impeded, arterial inflow may be reduced secondarily by as much as 30%. Limb loss is a serious concern and aggressive management (i.e., venous thrombectomy, catheter-directed lytic therapy, or both) is necessary.”

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