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Midline Granuloma
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<p><strong>Pendahuluan</strong><strong>:</strong></p><p><em>Midline granuloma</em> atau<em> nasal type extranodal T/NK-cell non-Hodgkin’s lymphoma </em>sering menimbulkan berbagai permasalahan.</p><p><strong>Pembahasan</strong><strong>:</strong></p><p><em>Midline granuloma</em> adalah kelainan bagian tengah wajah dengan ciri khas destruksi dan ulserasi yang progresif, meliputi hidung, sinus paranasal, palatum, mata, serta jaringan lunak wajah.Etiologi dan patofisiologi belum jelas antara reaksi radang dan tumor.<em> Midline granuloma</em> termasuk dalam penyakit granuloma hidung berdasarkan pemeriksaan biopsi, sedangkan<em>The Revised European American Lymphoma</em> (REAL) / <em>Wordl Health Organization</em> (WHO) mengelompokkan <em>midline granuloma</em> dalam <em>extranodal </em><em>T/NK-cellnon-Hodgkin’s lymphoma</em> berdasarkan pemeriksaan imunohistologi. Infeksi virus Epstein-Barr (EBV) 90-100% terlibat dalam proses pembentukan tumor.Diagnosis sulit dan diagnosis banding luas, sehingga diperlukan anamnesis dan pemeriksaan yang lengkap, terutama biopsi, imunohistologi, dan sitogenetik didapatkan gambaran nekrosis disertai destruksi dan ulserasi pembuluh darah, radang dengan infiltrat sel pleomorfik, CD56+, CD3+, protein sitotoksik+, dan EBV+. Penatalaksanaan dengan radioterapi, kemoterapi, transplantasi sumsum tulang, dan imunoterapi. Komplikasi dapat terjadi lokal maupun sistemik dengan penyebaran ke sirkulasi darah tepi, jaringan lunak, paru-paru, hati, kulit, saluran pencernaan, testis, saraf pusat, dan sumsum tulang. Prognosis buruk, hanya kurang dari 50% penderita yang berespon lengkap setelah kemoterapi dan radioterapi. Prognosis dapat dinilai dengan <em>International Prognostic Index</em> (IPI).</p><p><strong>S</strong><strong>impulan:</strong></p><p>Diagnosis<em>Midline granuloma</em>sulit, penatalaksanaan sering terlambat, sedangkan sifatnya yang progresif menimbulkan berbagai komplikasi, sehinggasemakin memperburuk prognosis.</p><p><strong> </strong></p><strong>Kata Kunci: midline granuloma, EBV, biopsi, imunohistologi, sitogenetik</strong>
Title: Midline Granuloma
Description:
<p><strong>Pendahuluan</strong><strong>:</strong></p><p><em>Midline granuloma</em> atau<em> nasal type extranodal T/NK-cell non-Hodgkin’s lymphoma </em>sering menimbulkan berbagai permasalahan.
</p><p><strong>Pembahasan</strong><strong>:</strong></p><p><em>Midline granuloma</em> adalah kelainan bagian tengah wajah dengan ciri khas destruksi dan ulserasi yang progresif, meliputi hidung, sinus paranasal, palatum, mata, serta jaringan lunak wajah.
Etiologi dan patofisiologi belum jelas antara reaksi radang dan tumor.
<em> Midline granuloma</em> termasuk dalam penyakit granuloma hidung berdasarkan pemeriksaan biopsi, sedangkan<em>The Revised European American Lymphoma</em> (REAL) / <em>Wordl Health Organization</em> (WHO) mengelompokkan <em>midline granuloma</em> dalam <em>extranodal </em><em>T/NK-cellnon-Hodgkin’s lymphoma</em> berdasarkan pemeriksaan imunohistologi.
Infeksi virus Epstein-Barr (EBV) 90-100% terlibat dalam proses pembentukan tumor.
Diagnosis sulit dan diagnosis banding luas, sehingga diperlukan anamnesis dan pemeriksaan yang lengkap, terutama biopsi, imunohistologi, dan sitogenetik didapatkan gambaran nekrosis disertai destruksi dan ulserasi pembuluh darah, radang dengan infiltrat sel pleomorfik, CD56+, CD3+, protein sitotoksik+, dan EBV+.
Penatalaksanaan dengan radioterapi, kemoterapi, transplantasi sumsum tulang, dan imunoterapi.
Komplikasi dapat terjadi lokal maupun sistemik dengan penyebaran ke sirkulasi darah tepi, jaringan lunak, paru-paru, hati, kulit, saluran pencernaan, testis, saraf pusat, dan sumsum tulang.
Prognosis buruk, hanya kurang dari 50% penderita yang berespon lengkap setelah kemoterapi dan radioterapi.
Prognosis dapat dinilai dengan <em>International Prognostic Index</em> (IPI).
</p><p><strong>S</strong><strong>impulan:</strong></p><p>Diagnosis<em>Midline granuloma</em>sulit, penatalaksanaan sering terlambat, sedangkan sifatnya yang progresif menimbulkan berbagai komplikasi, sehinggasemakin memperburuk prognosis.
</p><p><strong> </strong></p><strong>Kata Kunci: midline granuloma, EBV, biopsi, imunohistologi, sitogenetik</strong>.
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