RESUMEN
A 62-year-old man exhibiting nasal obstruction and glomerulonephritis with proteinase 3-antineutrophil cytoplasmic antibodies (PR3-ANCAs) was diagnosed with extranodal NK/T-cell lymphoma, nasal type (ENKL) with infiltration of neutrophils with apoptosis. Chemoradiotherapy reduced the tumor, improved the renal function, and decreased the PR3-ANCA levels. ANCA-positivity is observed in immunoinsufficient diseases, in which neutrophils lead to apoptosis and translocate intracellular granules, such as PR3, to the cell surface, triggering the production of ANCAs. In our case, the PR3-ANCA production was derived from the expression of PR3 on the cell surface of apoptotic neutrophils. This is the first report on ENKL describing the mechanism of ANCA development.
Asunto(s)
Anticuerpos Anticitoplasma de Neutrófilos/sangre , Glomerulonefritis/etiología , Linfoma Extranodal de Células NK-T/complicaciones , Neoplasias Nasales/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/etiología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/inmunología , Apoptosis/inmunología , Diagnóstico Diferencial , Glomerulonefritis/inmunología , Granulomatosis con Poliangitis/diagnóstico , Humanos , Linfoma Extranodal de Células NK-T/diagnóstico , Linfoma Extranodal de Células NK-T/inmunología , Linfoma Extranodal de Células NK-T/patología , Masculino , Persona de Mediana Edad , Mieloblastina/inmunología , Neutrófilos/patología , Neoplasias Nasales/diagnóstico , Neoplasias Nasales/inmunología , Neoplasias Nasales/patologíaRESUMEN
A 63-year-old male was diagnosed as having chronic phase CML in 2001. He obtained a major molecular response with imatinib (IM). In 2012, amulodipin was started for hypertension. In January 2013, IM was switched to nilotinib (NIL) in a clinical trial, and in February 2015, NIL was discontinued because MR4.5 had been maintained for two years. One month later, he was admitted to our hospital because of headache and high blood pressure (194/108 mmHg). His urine test showed protein 3+ and occult blood 2+. His eGFR rapidly deteriorated from 45.6 to 28.5 after admission. MR angiography showed left renal artery stenosis. He thus underwent angioplasty of the left renal artery with a stent implantation. His renal function subsequently improved. Cardiovascular events such as PAOD (peripheral artery occlusive disease) during NIL treatment were recently reported. However, to date, only four cases including our present patient with renal artery stenosis associated with NIL have been reported. These observations suggest assessment of risk factors for cardiovascular events at the start of NIL and careful monitoring to be important during tyrosine kinase inhibitor treatment of CML patients.
