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Mastocytosis
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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
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History Social history: Family history: No Etoh, tobacco, drug use
Negative for gastrointestinal malignancy or liver disease
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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
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Intensely pruritic lesions upon slight rubbing
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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
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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
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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
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Laparoscopy
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Laparoscopy
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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
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Histology
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Histology
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Histology
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