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A blue, painful limb in a cancer patient

Published:January 13, 2020DOI:https://doi.org/10.1016/j.ejim.2019.12.026

      1. Clinical case

      A 40-year-old man with metastatic colorectal cancer (peritoneum and lymph-node metastases) under therapy with palliative chemotherapy, with a previous history of deep venous thrombosis in the lower left leg complained to the assistant physicians of sudden worsening of the symptoms despite optimal anticoagulation with warfarin (International normalised ratio [INR]: 3,87). In the physical examination, the patient presented extreme pain followed by severe swelling, blue discoloration, and paresthesia in the lower left extremity (Fig. 1). The limb was cold and with no palpable pulses. The patient was treated with catheter-directed thrombolytic therapy without improvement, and underwent subsequent amputation. After 14 days, this patient developed the same condition in the contralateral extremity and went to obit in the palliative care unit.
      Fig 1
      Fig. 1A complicated case of deep venous thrombosis in a cancer patient (Phlegmasia cerulean dolens).

      2. What is the diagnosis?

      This patient has Phlegmasia cerulea dolens.
      Phlegmasia cerulean dolens is a rare and life-threating form of deep venous thrombosis caused by diffuse venous thrombosis associated with interruption of the arterial blood flow provoked by the compartment syndrome related to the severe edema. [
      • Chaochankit W.
      • Akaraborworn O.
      Phlegmasia cerulea dolens with compartment syndrome.
      ] It evolves to limb ischemia, and is characterized by severe swelling and blue discoloration of the affected extremity. The 4 cardinal signs of this condition are: edema, violaceous discoloration, pain and severe venous outflow obstruction. It affects more frequently males, and the most common risk factor is malignancy. [
      • Elsaid A.S.
      • AlQattan A.S.
      • Elashaal Ehab
      • AlSadery H.
      • AlGhanmi I.
      • Aldhafery B.F.
      The ugly face of deep vein thrombosis: phlegmasia cerulea dolens-case report. - PubMed - NCBI.
      ] Treatment consists in anticoagulation, thrombolytic therapy (catheter-directed preferred) and venous trombectomy. The risk of amputation is high (50%) and the overall mortality rate is around 40%, especially due to pulmonary embolism. [
      • Chinsakchai
      • ten Duis K.
      • Moll F.L.
      • de Borst G.J.
      Trends in management of Phlegmasia cerulea dolens.
      ] Due to its rarity, there is no prospective data evaluating specifically the best management for this condition.

      Declaration of Competing Interest

      Maria Alice Franzoi and Analu Vivian have no conflicts of interest to declare.

      References

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        • Akaraborworn O.
        Phlegmasia cerulea dolens with compartment syndrome.
        Ann Vasc Dis. 2018; : 355-357https://doi.org/10.3400/avd.cr.18-00030
        • Elsaid A.S.
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        The ugly face of deep vein thrombosis: phlegmasia cerulea dolens-case report. - PubMed - NCBI.
        Int J Surg Case Rep. 2019; 59: 107-110https://doi.org/10.1016/j.ijscr.2019.05.021
        • Chinsakchai
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        Trends in management of Phlegmasia cerulea dolens.
        Vasc Endovascular Surg. 2011; 45: 5-14https://doi.org/10.1177/1538574410388309