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Plummer-Vinson syndrome and reactive thrombocytosis mask a JAK2-V617F positive myeloproliferative neoplasm

Plummer-Vinson syndrome and reactive thrombocytosis mask a JAK2-V617F positive myeloproliferative neoplasm
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Author: Emma Catherine Scott and Stephen Schuster
Category: Red Cell: Disorders of Iron Metabolism and Heme Synthesis > Iron Deficiency and related disorders > Acquired
Published Date: 07/28/2012

A 52-year-old woman presented with abdominal pain, dysphagia, and fatigue. She had gastric adenocarcinoma treated with lower esophago-gastroduodenectomy without splenectomy and chemoradiotherapy 6 years prior. CT angiography demonstrated a thrombus in the abdominal aorta with bilateral embolic kidney infarcts, but no tumor recurrence nor organomegaly. Laboratory findings included: hemoglobin 7 g/dL, MCV 61 fL, platelet count 750 thou/µL, normal WBC, reticulocytes 2.5%, serum ferritin 6 ng/mL, serum iron 12 ug/dL, and transferrin 400 µg/dL. Peripheral smear showed thrombocytosis (normal morphology), hypochromia, and anisopoikolocytosis (main panel). Oral iron absorption test showed no absorption, presumed secondary to extensive surgery. Upper endoscopy demonstrated upper esophageal webs (inset). It is challenging to diagnose a myeloproliferative neoplasm (MPN) in the setting of severe iron deficiency and possible reactive thrombocytosis. Plummer-Vinson syndrome is a manifestation of sev