TY - JOUR
T1 - Plexogenic pulmonary hypertension associated with POEMS syndrome
AU - Czeczok, Thomas
AU - Lin, Peter
AU - Yi, Eunhee
N1 - Publisher Copyright:
© 2017 The Authors
PY - 2017
Y1 - 2017
N2 - Pulmonary hypertension is one of the well-known clinical manifestations of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, occurring in approximately 25–30% of the affected individuals. However, the histopathologic spectrum of pulmonary hypertension associated with POEMS syndrome has not been fully documented in the literature. Herein, we report an autopsy case of POEMS syndrome in a patient whose lung tissues showed histopathology indistinguishable from that of idiopathic pulmonary arterial hypertension with abundant plexiform lesions in the small pulmonary arteries.
AB - Pulmonary hypertension is one of the well-known clinical manifestations of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, occurring in approximately 25–30% of the affected individuals. However, the histopathologic spectrum of pulmonary hypertension associated with POEMS syndrome has not been fully documented in the literature. Herein, we report an autopsy case of POEMS syndrome in a patient whose lung tissues showed histopathology indistinguishable from that of idiopathic pulmonary arterial hypertension with abundant plexiform lesions in the small pulmonary arteries.
KW - POEMS
KW - Plexiform
KW - Pulmonary hypertension
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U2 - 10.1016/j.rmcr.2017.08.011
DO - 10.1016/j.rmcr.2017.08.011
M3 - Article
AN - SCOPUS:85027524129
SN - 2213-0071
VL - 22
SP - 168
EP - 170
JO - Respiratory Medicine Case Reports
JF - Respiratory Medicine Case Reports
ER -