CTICasesPulmonary Extramedullary Hematopoiesis in Myelofibrosis
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Imaging findings
Initial CT (2012) showed small nodules along fissures and pleural surfaces, massive splenomegaly, small paravertebral soft tissue foci, and perirenal soft tissue. By 2014, with worsening shortness of breath, chest CT revealed diffuse crazy paving, ground glass, smooth septal thickening, and more pronounced pleural and fissural nodules. There was a minor component of hemosiderin. Later imaging (2015) showed worsening perirenal infiltration, almost resembling Erdheim-Chester disease, all consistent with extramedullary hematopoiesis. Sclerotic bone foci were also present.
Key takeaways
The patient has a history of myelofibrosis. Lung biopsy confirmed pulmonary extramedullary hematopoiesis (EMH) with tri-lineage stem cells, which is consistent with the massive splenomegaly, paravertebral EMH, and perirenal involvement. This rare manifestation of myelofibrosis often responds poorly to chemotherapy, leading to whole lung radiation in this case. The lung parenchyma essentially transforms, trying to become bone marrow.
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