Factor VII Deficiency Diagnosed During Severe Ulcerative Colitis Flare

Abstract

Ulcerative colitis is a recurring inflammatory disease that typically begins in the rectum and affects the colon in a continuous fashion. Although bleeding and anemia are common in ulcerative colitis, severe bleeding is unusual. Ulcerative colitis combined with a concomitant bleeding disorder represents a rare phenomenon that may exacerbate rectal bleeding. We present a case of severe bleeding during an ulcerative colitis flare that unmasked an underlying Factor VII deficiency. A 22-year-old male with a past medical history including ulcerative colitis and autism presented with a one day history of bloody diarrhea. The patient had been diagnosed with ulcerative colitis five years prior when endoscopic biopsies revealed moderately active chronic colitis from the rectum to the cecum. Initial laboratory investigation revealed white blood cell count of 18.0 (K/MM3), hemoglobin 13.8 (g/dL), platelets 344 (K/MM3), elevated CRP 252 (mg/L), ESR 55 (mm/hr) and prothrombin time 24.2 seconds with INR 2.2. Physical examination revealed a healthy young male with mild bilateral lower quadrant tenderness without rebound or guarding and bright red blood on digital rectal exam. The patient was admitted and started on IV solumedrol and empiric metronidazole for a presumed flare of ulcerative colitis. Over the subsequent three days the patient continued to have severe bloody diarrhea with labs showing hemoglobin 10.6 (g/dL) and INR 2.8 despite the administration of vitamin K. A mixing study was performed and showed no evidence of an inhibitor affecting extrinsic pathway plasma procoagulants. Specific factor activity level testing revealed normal Factor II, V, IX and X activity with markedly reduced Factor VII activity of 4%(normal 55-170%). The diagnosis of factor VII deficiency was confirmed and the patient improved with factor replacement therapy and conservative management. Factor VII deficiency is the most common rare congenital bleeding disorder that effects one per 500,000 individuals and typically manifests with prolonged prothrombin time. Bleeding disorders such as Factor VII deficiency may exacerbate the degree of hemorrhage in inflammatory bowel disease (IBD) flares, making prompt recognition of underlying coagulopathies crucial in IBD patients. It is important for clinicians to be familiar with various factor deficiencies and their treatment options in order to control bleeding.Figure: Colonoscopy. Endoscopic images suggest features of ulcerative colitis with profound inflammation in a continuous and circumferential pattern.Figure: Endoscopic Colon Biopsy Specimen: Pathological analysis reveals microscopic features of ulcerative colitis with crypt shortening and distortion.Figure: Endoscopic Colon Biopsy Specimen: Pathological analysis showing paneth cell metaplasia in the left colon, consistent with the diagnosis of ulcerative colitis.