A case of essential thrombocytosis developing nephrotic syndrome and severe endothelial damage.
We present a 75-year-old Japanese man with essential thrombocytosis presenting nephrotic syndrome. Proteinuria developed soon after the patient was given a diagnosis of essential thrombocytosis and, 4 years later, it increased to a nephrotic range. Renal biopsy revealed one third of the obtained glomeruli totally sclerotic and the other glomeruli showed a marked thickening of the glomerular basement membrane and mild mesangial proliferation. Remarkable widening of the subendothelial space was evident on electron microscopy. Increased expression of platelet derived growth factor receptor beta was detected in the mesangium and interstitium by immunohistochemistry. Abnormal platelet activation in myeloproliferative disease has been shown to contribute in glomerulosclerosis by releasing various growth factors and cytokines including PDGF. Considering his clinical course and the pathological findings, the probable risk factor for developing severe endothelial damage and glomerulosclerosis is due to the persistence of high platelet count and platelet abnormality.
Saigusa
Takamitsu
T
Second Department of Internal Medicine, National Defense Medical College, Saitama-Japan. tsaigusa@mtd.biglobe.ne.jp
Kikuchi
Yuichi
Y
Yamada
Muneharu
M
Imakiire
Toshihiko
T
Hyodo
Toshitake
T
Suzuki
Shigenobu
S
Miura
Soichiro
S
D000368:Aged; D044466:Asian People; D001706:Biopsy; D001792:Blood Platelets / Q000378:metabolism / Q000648:ultrastructure; D049109:Cell Proliferation; D004727:Endothelium / Q000378:metabolism / Q000648:ultrastructure; D050533:Glomerular Basement Membrane / Q000378:metabolism / Q000648:ultrastructure*; D005920:Glomerular Mesangium / Q000378:metabolism / Q000648:ultrastructure*; D005923:Glomerulosclerosis, Focal Segmental / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007120:Immunochemistry; D007150:Immunohistochemistry; D007564:Japan; D008297:Male; D046529:Microscopy, Electron, Transmission; D009196:Myeloproliferative Disorders / Q000150:complications / Q000473:pathology; D015539:Platelet Activation; D010976:Platelet Count; D010982:Platelet-Derived Growth Factor / Q000378:metabolism; D011507:Proteinuria / Q000209:etiology / Q000378:metabolism / Q000473:pathology*; D018125:Receptors, Transforming Growth Factor beta / Q000378:metabolism; D013577:Syndrome; D013922:Thrombocytosis / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D013997:Time Factors
D010982:Platelet-Derived Growth Factor; D018125:Receptors, Transforming Growth Factor beta