Case Presentation:

A 36-year-old African American male, with accelerated phase Philadelphia chromosome positive CML  on dasatinib, presented to a community hospital with abdominal pain, distention, and melenic stools 10 days after being discharged.  During the previous admission, he received treatment for recurrent upper gastrointestinal (GI) hemorrhage due to a 1cm duodenal bulb ulcer with underlying large arterial vessel.  The patient reported two dark stools in the last several hours and sharp epigastric pain without radiation but with abdominal distention, similar to his prior admission.  The patient denied nausea or vomiting, but did report having constipation over the past several days for which he took a laxative which ultimately ended up revealing the melenic stools.  At presentation, the patient had a WBC count of 20.1×10^3, bandemia 20%, hemoglobin 5.1, platelet count 113,000, and a positive FOBT.

Gastroenterology performed an EGD for re-evaluation of the duodenal ulcer found on previous admission now showing an exposed vessel and adherent clot.  Due to the unstable nature of the clot, the ulcer was observed and treated with epinephrine injection, H/H was trended and repeat EGD was performed the next morning, then a large ulcer with a large branch of the gastroduodenal artery and active bleeding was seen in the duodenal bulb. This was aggressively cauterized and hemoclip was used for arterial occlusion. Due to the nature of the patient’s ulcer and high likelihood of rebleeding, surgical consultation was obtained.

Unfortunately, re-bleeding occurred and two days later the patient underwent exploratory laparotomy with Bilroth II hemigastrectomy and antecolic gastrojejunostomy.  Profuse hemorrhage and perforation were observed during the surgery and patient went into disseminated intravascular coagulation (DIC).  Massive resuscitation including packed RBCs, FFP, and platelets as well cryoprecipitate were given.  Several days later, pathology showed a perforated ulcer with findings consistent with chloroma (extramedullary myeloid sarcoma (MS)).

Discussion: This unfortunate case represents a unique presentation of MS secondary to CML blast crisis. MS are known to present in soft tissue, however to occur as a perforating ulcer with resulting fatality is rare.  In literature, there are a handful of case reports describing presentations of MS in the setting of blast crisis, however there have not been any large studies describing the epidemiology of MS.  This is likely due to the fatality often associated with the progression of the underlying disease process.

Conclusions: The patient remained in DIC for several days, but this resolved with aggressive resuscitation and critical care.  Regrettably, the patient developed multi-organ failure and anasarca.  The family decided to withdraw care and the patient expired shortly after from cardiac and respiratory failure.