Mastocytosis

History 67 y/o male with CAD, s/p CABG, evaluated for recurrent ascites requiring frequent LVP Past medical history: Coronary artery disease s/p CABG 2007 Type 2 diabetes Hypertension Medications: Lisinopril Insulin 70/30 ASA 81mg

History Social history: Family history: No Etoh, tobacco, drug use Negative for gastrointestinal malignancy or liver disease

Exam Well nourished, well developed Clear lung and cardiac exam Soft, non-tender, distended, +fluid wave, easily palpable liver below CM, +spleen Erythematous lesions predominantly on back

Intensely pruritic lesions upon slight rubbing

Laboratory Testing Total protein: 6.8 Alb: 2.9 Tbil: 0.7 AST 14 ALT 24 AlkPhos: 324 GGTP: 75 INR: 1.2 WBC: 4.3 Hgb: 10.6 HCT 31.3 Plat: 190 Cr: 1.5 Viral hepatitis panel, ANA, AMA, ASMA negative

Ascitic fluid 2D echo Total protein: 2.8 SAAG>1.1 LDH 47 WBC 268 (PMN 0%, Mono 100%) Culture and gram stain: negative 2D echo Left ventricular ejection fraction: 60% Normal right ventricular size Normal tricuspid, mitral, aortic valve

Imaging Studies Abdominal US CT scan abdomen Hepatosplenomegaly Patent portal vein with hepatopetal flow CT scan abdomen Nodular liver Splenomegaly Ascites Cholelithiasis Multiple blastic lesions throughout the thoracic/lumbar spine and pelvic bones consistent with metastasis

Laparoscopy

Laparoscopy

Pathology Portal fibrosis with focal bridging S2-3 Mild to moderate inflammatory infiltrate in portal triad with eosinophils mast cells lymphocytes Special stains confirmed the diagnosis of mastocytosis

Histology

Histology

Histology