TY - JOUR
T1 - Advanced extramedullary hematopoiesis with a marked increase in reticulin fibers and hemorrhage on various organs
T2 - the first autopsy case report
AU - Watanabe, Hirofumi
AU - Fujishima, Fumiyoshi
AU - Fukuhara, Noriko
AU - Taniuchi, Shinji
AU - Joh, Kensuke
AU - Sasano, Hironobu
N1 - Funding Information:
We thank Ms. Aoyama and Ms. Adachi for assistance in the preparation of the images for immunohistochemistry and electron microscopy.
Publisher Copyright:
© 2021, The Japanese Society for Clinical Molecular Morphology.
PY - 2022/3
Y1 - 2022/3
N2 - Myelofibrosis is characterized by stem cell-derived clonal proliferation potentially resulting in bone marrow fibrosis. As the disease progresses, extramedullary hematopoiesis is frequently detected in the spleen and the liver but rarely in other organs. We report a case of a 68-year-old woman with myelofibrosis with a JAK2 mutation, showing extramedullary hematopoiesis (EMH) in various organs with a marked increase in reticulin fibers, and myeloproliferative neoplasm (MPN)-related necrotizing crescent glomerulonephritis. She was admitted to our hospital owing to respiratory discomfort. Computed tomography revealed a mass in the anterior mediastinum. Ten days later, the patient died owing to respiratory distress. At autopsy, EMH were detected in the anterior mediastinum, heart, lung, spleen, and the kidney with a marked increase in reticulin fibers. We considered that respiratory distress was partially caused by EMH. In the kidney, necrotizing crescent glomerulonephritis was observed. Immunohistochemically, the glomerular basement and mesangial area were IgA- and C3d-positive. Ultrastructural examination revealed the presence of dense deposits in the subendothelial space and the mesangial and paramesangial areas. Thus, we suspected that MPN-related necrotizing crescentic glomerulonephritis harbored a pathogenesis similar to that of IgA-dominant post-infectious glomerulonephritis or IgA nephropathy. This case report could widen the spectrum of MPN- or EMH-related lesions.
AB - Myelofibrosis is characterized by stem cell-derived clonal proliferation potentially resulting in bone marrow fibrosis. As the disease progresses, extramedullary hematopoiesis is frequently detected in the spleen and the liver but rarely in other organs. We report a case of a 68-year-old woman with myelofibrosis with a JAK2 mutation, showing extramedullary hematopoiesis (EMH) in various organs with a marked increase in reticulin fibers, and myeloproliferative neoplasm (MPN)-related necrotizing crescent glomerulonephritis. She was admitted to our hospital owing to respiratory discomfort. Computed tomography revealed a mass in the anterior mediastinum. Ten days later, the patient died owing to respiratory distress. At autopsy, EMH were detected in the anterior mediastinum, heart, lung, spleen, and the kidney with a marked increase in reticulin fibers. We considered that respiratory distress was partially caused by EMH. In the kidney, necrotizing crescent glomerulonephritis was observed. Immunohistochemically, the glomerular basement and mesangial area were IgA- and C3d-positive. Ultrastructural examination revealed the presence of dense deposits in the subendothelial space and the mesangial and paramesangial areas. Thus, we suspected that MPN-related necrotizing crescentic glomerulonephritis harbored a pathogenesis similar to that of IgA-dominant post-infectious glomerulonephritis or IgA nephropathy. This case report could widen the spectrum of MPN- or EMH-related lesions.
KW - Advanced extramedullary hematopoiesis
KW - Hemorrhage
KW - Myelofibrosis
KW - Myeloproliferative neoplasm
KW - Necrotizing crescentic glomerulopathy
KW - Reticulin fiber
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U2 - 10.1007/s00795-021-00300-x
DO - 10.1007/s00795-021-00300-x
M3 - Article
C2 - 34396490
AN - SCOPUS:85112422996
VL - 55
SP - 68
EP - 75
JO - Medical Molecular Morphology
JF - Medical Molecular Morphology
SN - 1860-1480
IS - 1
ER -