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Pulmonary Extramedullary Hematopoiesis in Myelofibrosis

Imaging findings

Serial chest CTs over several months in a patient with known myelofibrosis show diffuse, bilateral ground-glass opacity throughout the lungs without airway wall thickening or other findings to suggest an infectious or inflammatory airway process, with the pattern essentially unchanged across multiple follow-up studies. PET imaging shows diffuse pulmonary FDG uptake corresponding to the ground-glass opacity as well as marrow uptake at other sites.

Key takeaways

The combination of unchanging diffuse ground-glass opacity and diffuse pulmonary FDG avidity in a patient with myelofibrosis is characteristic of pulmonary extramedullary hematopoiesis, a rare but well-described manifestation in which hematopoietic cells accumulate within the pulmonary interstitium. The patient also has pulmonary hypertension attributed to the underlying hematologic disorder, classified as Group 5 pulmonary hypertension, rather than left heart disease, and biopsy was not felt to be necessary given the characteristic imaging and PET findings.

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