Acute compartment syndrome as the initial manifestation of chronic-phase chronic myeloid leukemia: a case report and review of the literature

Abstract

BackgroundAcute compartment syndrome is an orthopedic emergency requiring urgent fasciotomy to prevent irreversible damage. In hematological malignancies, acute compartment syndrome caused by severe soft tissue bleeding is extremely rare. We present a patient with chronic-phase chronic myeloid leukemia who had acute compartment syndrome caused by severe soft tissue bleeding in her right forearm.Case presentationA 72-year-old Japanese woman was referred to our hospital with swelling and pain of her right forearm without a previous history of trauma. She was diagnosed with chronic-phase chronic myeloid leukemia. Extreme thrombocytosis was present, although no evidence of acquired von Willebrand disorder was found. Compartment syndrome caused by soft tissue bleeding was confirmed. An emergency fasciotomy for decompression was conducted. However, sustained postoperative bleeding occurred and required massive red cell concentrate transfusion. As her platelet count decreased by cytoreductive therapy, complete hemostasis was achieved.ConclusionsPatients with an extremely high platelet count might be at high risk for severe bleeding complications even without acquired von Willebrand disease. For the control of severe bleeding complications in patients with myeloproliferative disorder, the importance of thrombocyte reduction should be recognized.

Review article: A comprehensive review of unusual causes of acute limb compartment syndrome

“…Platelet deficiency or dysfunction also causes ALCS, and has been reported with human immunodeficiency virus 23,24 . Extreme thrombocytosis may also cause spontaneous haemorrhage leading to compartment syndrome 22,25 . Anticoagulant therapy may cause ALCS, especially with minor trauma, such as simple venepuncture or acupuncture 26–29 .…”

“…Pro‐ and anti‐coagulant clotting abnormalities are also associated with ALCS, including spontaneous haemorrhage from haemophilia, factor deficiencies and haematological malignancy. 11 , 18 , 19 , 20 , 21 , 22 Platelet deficiency or dysfunction also causes ALCS, and has been reported with human immunodeficiency virus. 23 , 24 Extreme thrombocytosis may also cause spontaneous haemorrhage leading to compartment syndrome.…”

“… 23 , 24 Extreme thrombocytosis may also cause spontaneous haemorrhage leading to compartment syndrome. 22 , 25 Anticoagulant therapy may cause ALCS, especially with minor trauma, such as simple venepuncture or acupuncture. 26 , 27 , 28 , 29 Hypercoagulable disorders such as protein S deficiency may also present with ALCS related to thrombosis.…”

Unusual Presentation of Compartment Syndrome

“…Another cause for hematologicaloriginated ACS apart from infiltration is the excessive bleeding. Chronic phase of myeloid leukemia (CML) as a myeloproliferative disorder with excessive platelet number was found to cause ACS through excessive bleeding which persisted after fasciotomies and seized only when their number was controlled with cytosuppresive treatment [48]. However, it must be noted that in chronic CML, platelet dysfunction is not always due to their number, and urgent hematological consult should be sought [49].…”

Acute compartment syndrome resultant from alcohol abuse

“…Acute compartment syndrome (ACS) is an increase in compartment extremity pressure that subsequently prevents microvascular perfusion. ACS is usually the result of a traumatic incident such as a crush injury, long bone fracture or arterial thrombosis; however, in cases without a traumatic origin, a diagnostic dilemma is common [ 1 ]. The most common cause of ACS is fractures, accounting for ~69–75% of cases with the remaining few accounted for by ischemic events, iatrogenic procedures, idiopathic causes and rarely coagulopathies [ 2 , 3 ].…”