PMC9293942_01
CT chest showing diffuse mosaic attenuation in the (a) axial and (b) coronal views.
gr1.jpg
PMC9293942_01_gr1.jpg
fig1
Inspiratory and expiratory computed tomography (CT) chest showed diffuse mosaic attenuation, enlarged mediastinal and hilar lymphadenopathy (Fig. 1A and B& C), enlarged pulmonary trunk (3.4 cm), and enlarged central pulmonary arteries.
Histology from right lower lobe transbronchial lung cryobiopsy. (a). Low-power view reveals central lung parenchyma with overlying bronchial mucosa. There are expansile, vaguely nodular deposits of extramedullary hematopoiesis (black arrow) in a background of diffuse, homogeneous interstitial thickening (red arrow). H&E (4X). (b). Medium sized blood vessels showing moderate (black arrow) to severe (red arrow) vascular medial and intimal thickening with disrupted elastica. H&E (10x). (c). Immunohistochemistry highlights trilinear hematopoiesis. CD61, glycophorin A, and myeloperoxidase reveal frequent presence of megakaryocytes, erythroid, and myeloid precursors, respectively. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
gr2.jpg
PMC9293942_01_gr2.jpg
fig2
Transbronchial lung cryobiopsy revealed expansile, vaguely nodular deposits of extramedullary hematopoiesis in a background of diffuse, homogeneous interstitial thickening (nonspecific interstitial pneumonia (NSIP) pattern) (Fig. 2A) and medium sized blood vessels with moderate to severe vascular medial and intimal thickening with disrupted/reduplicated elastica (Fig. 2B). Immunohistochemistry showed trilinear hematopoiesis with cluster of differentiation 61 (CD61) positive megakaryocytes, focal aggregates of glycophorin A positive erythroid precursors, and rare myeloperoxidase (MPO) positive myeloid precursors (Fig. 2C).