CTICasesDiffuse Pulmonary Extramedullary Hematopoiesis in Polycythemia Vera
Imaging findings
CT shows a diffuse, homogeneous ground-glass parenchymal abnormality with subtle crazy paving, sparing only a few lobules, plus sparse septal lines and a little pleural fluid. Comparison with 2017 shows the process is chronic and stable, with splenomegaly. The patient has polycythemia vera, and there is associated pulmonary hypertension; a few nodes in unusual locations may also harbor hematopoietic tissue.
Key takeaways
Diffuse pulmonary extramedullary hematopoiesis can produce a chronic, homogeneous ground-glass and subtle crazy-paving pattern and occurs not only in myelofibrosis but also in polycythemia vera. It falls under group 5 pulmonary hypertension, with the associated (sometimes severe) pulmonary hypertension likely multifactorial; splenomegaly and chronicity are helpful clues, and a sulfur-colloid scan can demonstrate pulmonary uptake.
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