A 36 year old female presented to the hospital with tongue swelling and bleeding after she bit her tongue. Due to worsening swelling of her tongue, she was emergently intubated in the emergency department and transferred to the ICU for further management of her airway. Her recent history was significant for fatigue, menorrhagia, multiple “colds” and sore throats, and recurrent infections of her umbilical piercing. On exam she was noted to have shotty cervical lymphadenopathy and a swollen tongue without evidence of lip swelling. There was evidence of cellulitis around her umbilical piercing, which was subsequently removed. CT neck demonstrated no abscess of the posterior oropharynx but was limited in ability to evaluate the tongue due to the presence of the endotracheal tube. Initial laboratory work showed a pancytopenia with a white blood cell count of 4.0 × 10³/μL, hemoglobin 7.5 g/dl, and platelets 116 × 10³/μL. Her peripheral smear showed evidence of “rare” blasts. Her coagulation derangements consisted of a prolonged prothrombin time (PT) of 16.1 s, INR of 1.32, partial thromboplastin time (PTT) of 36.5 s. Her D-dimer was greater than the upper level of detection at 500 μg/dL. Fibrin split products were >20 μg/dL, and her Fibrinogen was 344 mg/dL. Because of the patient’s pancytopenia, a bone marrow biopsy was obtained and showed a hypercellular marrow with markedly increased blasts, accounting for 95 % of the total cells. Chromosomal analysis demonstrated t(15;17) rearrangement.

Acute Promyelocytic leukemia (APML) is a form of acute myelocytic leukemia (AML), comprising approximately 10 % of AMLs diagnosed. APML is specifically characterized by a reciprocal translocation between the retinoic acid receptor-alpha (RAR-α) gene on chromosome 17 and the PML gene on chromosome 15. This rearrangement is known as t(15;17). APML often acutely presents clinically with extensive bleeding diathesis requiring ICU admission at the time of diagnosis [1]. Up to 15 % of APMLs will present with DIC. This can be due to the disease itself or the effect of undergoing induction chemotherapy [2, 3].

In the setting of acute APML, the extensive bleeding characteristics are due to hyper fibrinolysis driven primarily by fibrinolytics such as u-PA, t-PA, as well as protease elastase and chemotrypsin [4, 5] which are found in high levels in leukemic blast cells [4, 5]. The over production of these fibrinolytics in the setting of profound thrombocytopenia due to bone marrow dysfunction yield the profound bleeding disorder [1].

DIC is also closely associated with the induction therapy in APML. Treatment of APML consists primarily of use of All-trans-retinoic acid (ATRA). ATRA promotes differentiation of leukemic blasts. This process can transiently worsen DIC by consumption of pro-coagulation factors, namely Tissue Factor (TF) and Cancer Procoagulant (CP) [6, 7]. However, within approximately 48 h, as the leukemic blast burden improves so do the markers of DIC.