TY - JOUR
T1 - Myelodysplastic syndrome with pulmonary tumor thrombotic microangiopathy in an 11-year-old male patient
AU - Perrino, Carmen M.
AU - Dehner, Louis P.
AU - Hartman, Mary E.
AU - Agarwal, Ashima
PY - 2014
Y1 - 2014
N2 - Myelodysplastic syndrome (MDS) and pulmonary tumor thrombotic microangiopathy (PTTM) are independently rare in the pediatric population. This report describes an 11-year-old male patient who initially presented with respiratory distress and cardiovascular collapse. A large left main pulmonary artery embolus and multiple, smaller pulmonary thromboemboli were widely dispersed throughout both lungs. Despite aggressive supportive care, he died within seven hours of admission. A complete postmortem examination was performed, leading to the diagnoses of primary MDS and microthrombi in the lungs, including the characteristic fibroproliferative lesions seen in PTTM. Individually, both conditions are extremely uncommon, and therefore the coincidence of these 2 conditions in a child is singularly unique.
AB - Myelodysplastic syndrome (MDS) and pulmonary tumor thrombotic microangiopathy (PTTM) are independently rare in the pediatric population. This report describes an 11-year-old male patient who initially presented with respiratory distress and cardiovascular collapse. A large left main pulmonary artery embolus and multiple, smaller pulmonary thromboemboli were widely dispersed throughout both lungs. Despite aggressive supportive care, he died within seven hours of admission. A complete postmortem examination was performed, leading to the diagnoses of primary MDS and microthrombi in the lungs, including the characteristic fibroproliferative lesions seen in PTTM. Individually, both conditions are extremely uncommon, and therefore the coincidence of these 2 conditions in a child is singularly unique.
KW - Cor pulmonale
KW - Myelodysplastic syndrome
KW - Pulmonary hypertension
KW - Pulmonary tumor thrombotic microangiopathy
KW - Right heart failure
UR - http://www.scopus.com/inward/record.url?scp=84895472774&partnerID=8YFLogxK
U2 - 10.2350/13-05-1339-CR.1
DO - 10.2350/13-05-1339-CR.1
M3 - Article
C2 - 24099418
AN - SCOPUS:84895472774
SN - 1093-5266
VL - 17
SP - 44
EP - 49
JO - Pediatric and Developmental Pathology
JF - Pediatric and Developmental Pathology
IS - 1
ER -