Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension

Figure 1 Gastric antral vascular ectasia observed on upper gastrointestinal endoscopy.

Figure 2 Images on abdominal computed tomography scan. A: Homogeneous hepatosplenomegaly; B: Normal spleen on computed tomography scan performed two years earlier.

Figure 3 Immunohistochemistry study identified positive staining for CD117 and CD25. A: Atypical spindle-shaped MC on bone marrow aspirate (HE × 100); B: Hypercellularity due to infiltration by atypical MC on bone biopsy (HE × 100); C: MC were strongly positive for CD25; and D: CD117 (D) (IHC × 100).

Figure 4 Microscopic images of the liver. A: Liver biopsy showed an increased number of spindled MC (HE × 40); B: The MC are highlighted by positive CD117 staining (IHC × 100)

Figure 5 Extensive involvement of the stomach. A: Endoscopic image of the stomach showed diffuse polypoid lesions with active bleeding; B: Microscopic appearance of the gastric mucosa showed infiltration by atypical MC strongly positive for CD117 (IHC × 100).