140 Acute Pancreatitis SPECIFIC ENTITIES ASCENDING CHOLANGITIS PATHOPHYSIOLOGY biliary calculi (choledocho lithiasis), post ERCP, tumors, primary sclerosing cholangitis, or benign stricture ! biliary obstruc tion and stasis ! bacterial colonization and infec tion (E coli, Klebsiella, Enterobacter, Enterococcus, anaerobes) ! liver failure, sepsis CLINICAL FEATURES Charcot’s triad consists of fever, right upper quadrant pain, and jaundice Reynold’s pentad is associated with the addition of hypotension and confusion SPECIFIC ENTITIES (CONT’D) " bilirubin, ALP, and potentially AST and ALT Blood cultures essential U/S abd to check for common bile duct dilatation and stones, ERCP, MRCP TREATMENTS antibiotics (imipenem 500 mg IV q6h, ampicillin plus gentamicin) Facilitate biliary drainage (ERCP with sphincterotomy, stone extraction, stent insertion, percutaneous drainage, surgical decompression) DIAGNOSIS Notes Notes 141 142 Notes Notes HEMATOLOGY Section Editor: Dr Michael Kroll Polycythemia DIFFERENTIAL DIAGNOSIS INVESTIGATIONS SPURIOUS stress (Geisboăcks syndrome), decrease intravascular volume PRIMARY polycythemia rubra vera SECONDARY wHERAw HYPOXIA obstructive sleep apnea, COPD, smok ing, high altitude EPO-SECRETING TUMORS renal, hepatoma, cere bellar, pheochromocytoma RENAL polycystic kidney disease, hydronephro sis, post transplant ADRENAL Cushing’s syndrome BASIC PATHOPHYSIOLOGY DEFINITION OF POLYCYTHEMIA in ?, hematocrit >0.5 in / hematocrit >0.6 Related Topics Hypoxemia (p 92) Myeloproliferative Disorders (p 165) CLINICAL FEATURES HISTORY hyperviscosity (headache, blurred vision, epistaxis), dyspnea, epigastric pain, weight loss, fever, night sweats, pruritus, erythromelalgia, recent travel to high altitude areas, past medical history (respira tory diseases, myeloproliferative disorders, myocar dial infarction, stroke, pulmonary embolism, DVT, renal disorders, smoking), medications (androgens, EPO) PHYSICAL hypertension, oxygen saturation, facial plethora, conjunctival injections, engorgement of the veins of the optic fundus, abdominal mass, hepato megaly, splenomegaly, excoriations, stigmata of a prior arterial or venous thrombotic event, gouty arthritis, and tophi CBCD, lytes, urea, Cr, LAP, vitamin B12, RBC mass (total blood volume  Hct, to rule out spurious causes), carboxyhemoglobin level, cor tisol level, peripheral blood smear IMAGING CXR SPECIAL JAK2 MUTATION JAK2 is a cytoplasmic tyrosine kinase activated by EPO binding to its receptor; the V617F mutation activates JAK2 and thereby drives EPO independent erythropoiesis EPO LEVEL low in PRV, high if secondary causes HYPOXIA WORKUP oximetry, ABG, CO hemoglobin SOLID TUMOR WORKUP CT abd, MRI head (if tumors) BONE MARROW BIOPSY rule out myelofibrosis and CML LABS DIAGNOSTIC ISSUES CRITERIA FOR POLYCYTHEMIA RUBRA VERA (PRV) ABSOLUTE " RBC mass, no secondary cause (nor mal PaO2, EPO not elevated) MAJOR splenomegaly, JAKV617F 3 MINOR WBC >12Â10 /mL, platelet >400 Â10 /mL, LAP >100U/L and vitamin B12 >650pmol/L [>880 pg/mL] DIAGNOSIS need absolute criteria plus one major or two minor criteria for the diagnosis of poly cythemia rubra vera See myeloproliferative disor ders (p 165) for more details MANAGEMENT TREAT UNDERLYING CAUSE relative (hydration), CO hemoglobinemia (smoking cessation See p 418), sleep apnea (CPAP See p 17), polycythe mia vera (cytoreduction with hydroxyurea is prefer able to phlebotomy to keep hematocrit