Calciphylaxis with evidence of hypercoagulability successfully treated with unfractionated heparin: a multidisciplinary approach

Abstract

Calciphylaxis is characterized by abnormal calcification of vessels and skin; however, its aetiology and pathogenesis remain unclear. Entities frequently associated with calciphylaxis are end-stage renal disease, diabetes mellitus, hypercalcaemia, hyperphosphataemia, elevated calcium-phosphate product, hyperparathyroidism and possible hypercoagulable states. Skin lesions may remain quiescent or may develop suddenly and progress rapidly. They are more common on the legs. Treatment of calciphylaxis is very challenging and requires interdisciplinary management. We present a case that highlights the difficulty of treating calciphylaxis. A multidisciplinary approach was vital for the proper treatment of our patient. This case also demonstrates the importance of searching for underlying hypercoagulable states, especially in recalcitrant cases. In cases of calciphylaxis with vessel occlusion from microthrombi, heparin therapy would be a logical next step. The effect of anticoagulation may be rapid and impressive.

Association Between Hypercoagulable Conditions and Calciphylaxis in Patients With Renal Disease

“…Hypercoagulability has been posited as a potential risk factor for calciphylaxis . This hypothesis arose from numerous case reports and case series of patients with calciphylaxis with hypercoagulable conditions . Although descriptive studies have suggested that hypercoagulable states may predispose patients to calciphylaxis, lack of comparison to a control group has potential to overestimate the association.…”

“…7 This hypothesis arose from numerous case reports and case series of patients with calciphylaxis with hypercoagulable conditions. [8][9][10][11][12] Although descriptive studies have suggested that hypercoagulable states may predispose patients to calciphylaxis, lack of comparison to a control group has potential to overestimate the association. It is noteworthy that thrombophilias are also present at high rates in patients with chronic kidney disease (CKD), a group that significantly overlaps with patients with calciphylaxis, further complicating study of hypercoagulable risk factors.…”

Therapeutic effects of human amnion-derived mesenchymal stem cells on hypercoagulability in a uremic calciphylaxis patient

“…Calciphylaxis patients treated with tissue plasminogen activator had approximately 30% greater survival than controls, but significant difference was not observed 11 . Therapeutic success in a case of calciphylaxis is thought to be associated with low molecular weight heparin for hypercoagulable condition 12 . Hence, as a potential risk factor, hypercoagulability plays an important role in the pathogenic mechanism and is proposed to be the treatment target for calciphylaxis.…”

“…Although microvascular calcification is regarded as the basic histopathological characteristic, it is noteworthy that hypercoagulable states seem to be crucial in the development of CUA 6,10 , and anticoagulants including tissue plasminogen activator or low molecular weight heparin have been reported effective 6,[10][11][12] . We have reported an innovative therapeutic strategy for a CUA patient via intravenous combined with local treatment of hAMSCs, with speculated mechanisms including the inhibition of vascular calcification, the stimulation of neovascularization and myogenesis, as well as antiinflammation, immunoregulatory actions, and re-epithelialization 13 .…”

DOACs least harmful for patients with calciphylaxis on hemodialysis needing anticoagulation

“…This study is less relevant because it did not subcategorize patients on dialysis; the primary objective was to investigate the prevalence of thrombotic disorders in patients with calciphylaxis Carter et al discussed a case report of a patient with recalcitrant calciphylaxis affecting nearly 50% of both legs, which responded to daily subcutaneous unfractionated heparin therapy. 4 However, a clinical decision for our patient cannot be based on a case report.…”