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Kuliah FK UNIBA 31 Januari 2015

LIMPHADENOPATI

Oleh : dr. Nida Sufrida Gumar, SpPA.,MKes


Pendahuluan
Kelenjar getah bening:
-bulan ke 3 pertumbuhan janin
-unsur dlm kgb: limfosit, sel retikulum,fibroblast
-semua sel, jaringan, organ yg mengandung kumpulan
limfosit(limfa, tonsil,thymus,usus,sumsum tulang, paru-paru dll)
o Sistim limforetikuler : merupakan sistim kekebalan tubuh
perlindungan dari antigen
o Fungsi perlindungan : limfosit T danB
o Limfosit B: limfonodus,limfa,jaringan ikat
o Limfosit T: kelenjar timus
Pendahuluan
KGB bereaksi thd stimuli perubahan aktif :
reaksi inflamasi dan reaksi imun
Gambaran mikroskopis menyerupai, walaupun penyebab
berbeda
Beberapa agen gambaran karakteristik
Reaksi berbagai kompartemen: korteks, parakorteks,
sinus evaluasi lanjut
Setiap kompartemen masalah diagnosis banding dengan
keganasan pada KGB
Limfadenopati reaktif : 5 pola
folikular/nodular, interfolikular/parakortikal, difus, sinusal
dan campuran
Pola ini juga berlaku pada berbagai tipe limfoma maligna
Hiperplasia rektif harus dapat dibedakan dengan limfoma
maligna terapi dan prognosis jauh berbeda
Hiperplasia folikular = penambahan jumlah dan ukuran yang
bervariasi dari folikel limfoid
Limfadenopati jinak, ganas
Anatomi
Histologi
At high magnification is seen a lymph node follicle with a germinal
center containing larger lymphocytes undergoing activation. At the
lower right is the subcapsular sinus.
Reactive germinal center. Dark zone of centroblasts (lower center) light
zone of centrocytes (upper center). Small lymphocytes of mantle zone
(lower right).
Epidemiologi
Limfadenopati (jinak): radang
Hiperplasia folikular hiperplasia reaktif tersering
Pada anak-anak dan dewasa
Limfadenopati yang berhubungan dengan AIDS/HIV:
pria homoseksual, pecandu obat , PSK.
PTGC : pria>wanita, usia muda rata-rata 28 th.
Penyakit Castleman : semua umur sering pada usia
dewasa, laki-laki=perempuan
Limfadenitis lues : pria homoseksual AIDS/HIV,
wanita pecandu dan bayi yang dilahirkan.
Epidemiologi

Limfadenopati yang berhubungan dengan rheumatoid


arthritis : semua umur sering pada usia 35 dan 50 th,
wanita > pria
Penyakit Kimura : sering pada laki-laki usia muda sp
pertengahan, endemis di Asia.
Etiologi
Penyebab : bakteri, virus, bahan kimia, polutan, obat-obatan,
antigen atau alergen , agen iatrogenik.
MIAMI (malignancies, infections, autoimmune, miscellanous
dan iatrogenics)
Penyakit Castleman : diduga respon imun dan infeksi virus (HHV-
8) interleukin 6
Rheumatoid arthritis : autoimun, penyebab pasti tidak diketahui
berhub dengan lingkungan, genetik dan infeksi virus.
Sifilis Treponema palidum
Penyakit Kimura : tidak diketahui pasti
Fungsi utama kgb : filter bagi drainase pembuluh limfe
berupa benda asing amorfus (difagosit dan degradasi) dan
antigen (memicu reaksi imun).
Reaksi KGB :
reaksi inflamasi : bakteri dan jamur
reaksi imun : virus dan obat-obatan
Respon imun
dominasi sel B : hiperplasia folikular atau plasmasitosis
dominasi sel T : hiperplasia sel T
Limfadenopati(ganas): Limfoma - hodgkin
- non hodgkin
Patologi
Makroskopis
Makroskopis tidak berbeda satu sama lain
Dapat solid atau multipel
Ukuran antara 1-3 cm
Pada penyakit castleman: ukuran kelenjar getah bening
dapat lebih dari 15 cm
Pada penampang melintang berwarna putih
kecoklatan/pucat sampai kecoklatan, padat
Konsistensi kenyal atau lunak
KGB normal
Mikroskopis
Reactive follicular hyperplasia
Explosive follicular hyperplasia (AIDS related
lymphadenopathy)
Pola C

Folikel berukuran kecil, limfosit berkurang, pembuluh darah mengalami kolagenisasi.


Involusi kgb : atrofi folikel, angiogenesis dan fibrosis
Progressive transformation of germinal
centre
Penyakit Castleman

Tipe vascular-hyaline
Rheumatoid lymphadenopathy
Vascular proliferation and plasmacytosis in the
interfollicular region.
Luetic lymphadenitis
Luetic lymphadenitis
Penyakit Kimura

Warthin-Finkeldey polykaryocytes,
Eosinophilic microabscess eosinophils, and proliferation of
capillaries in cervical lymph node
In sarcoid granulomas, the Langhans giant cells are usually smaller with fewer
nuclei than those seen in tuberculosis. Necrosis is usually absent. Necrotizing
variant of sarcoidosis does exist, but it is usually extranodal.
Cat-scratch disease usually presents with a skin lesion (7 to 12 days after
contact) and enlargement of regional lymph nodes. The patient in this case
was a 40 y/o male who presented with a 6.0 cm right axillary mass. He had
history of being scratched by a cat on the right arm. The image shows multiple
microabscesses of varying sizes (pink areas).
Proliferasi sel dalam kgb:
Limfosit:-limfositoma,limfosarkomaleukemia
Limfoblast:limfosarkoma
Sel Retikulum: giant folickle limfoma, sarkoma sel
retikulum,hodgkin
Sel Plasma Plasmasitoma
Gejala Klinis
Sesuai dengan etiologi atau penyakit yang melatarbelakangi
dapat disertai rasa sakit atau tidak
Limfadenopati
Lokasi dapat mengarah pada etiologi:
- colly infeksi virus, gigi, tbc
- inguinal Peny. menular seksual
Gejala sistemik : demam, keringat malam dan penurunan
berat badan
Diagnosis
Keterangan klinis
Pemeriksaan fisik
Laboratorium
Histopatologi
Diagnosis pasti : kombinasi dari keterangan klinis,
laboratorium dan histopatologi
Untuk menentukan diagnosis jinak, ganas sel-sel kgb:
Lokasi limfadenopati
Pemeriksaan mikroskopis
Pemeriksaan Imunohistokimia
Diagnosis Banding
Hiperplasia reaktif folikular didiagnosis banding dengan
limfoma folikular
Hiperplasia folikular eksplosif didiagnosis banding dengan
luetic limfadenitis
Luetic limfadenitis didiagnosis banding dengan rheumatoid
limfadenopati
Rheumatoid limfadenopati didiagnosis banding dengan
penyakit Castleman
Penyakit Kimura didiagnosis banding dengan Penyakit
Hodgkin
Fungsi utama kgb : proses antigen dan reaksi antigen terhadap
limfosit B atau T
Proliferasi dari sel B atau T pada kompartemen kgb >>>
: limfadenopati
Limfadenitis : infeksi pada klenjar getah bening dan
ditemukan agen infeksius
Hiperplasia limfoid :tanpa infeksi pada kgb
Respon kgb thd antigen bervariasi : folikel, sinus, difus
Masing-masing pola berhubungan dengan antigen
Jika hiperplasia limfoid sangat intens sulit dibedakan dengan
limfoma maligna
Pada hiperplasia : struktur, sel beragam, sel atipik sedikit
Pada limfoma : struktur abnormal dan banyak sel atipik
Apabila secara morfologi tidak bisa dibedakan pem. IHK,
genetik dan analisa genotipik (monoklonal atau poliklonal)
Limfoma folikular
Hiperplasia folikular
Hiperplasia folikular reaktif Limfoma folikular
Prognosis
Secara umum dipengaruhi oleh etiologi, terapi dan perjalanan
penyakit
Rheumatoid limfadenopati limfoma
Hiperplasia folikular eksplosif sarkoma kaposi dan
limfoma
Penyakit Castleman komplikasi paru, ginjal dan limfoma
Penyakit Kimura baik, kadang-kadang rekurensi lokal
Terapi
Bervariasi, sesuai etiologi dan penyakit yang mendasari
Terapi konservatif, antibiotik, medikamentosa atau
pembedahan
Penyakit Castleman eksisi komplit Kgb
Penyakit Kimura bedah reseksi + terapi steroid
Luetic limfadenitis antibiotika : penisilin
Kesimpulan

Limfadenopati: jinak, ganas


Tergantung etiologi diagnosis, terapi, prognosis
Terimakasih
Selamat belajar dan sukses
Intermediate-power view shows numerous sarcoid granulomas
composed of epithelioid histiocytes, occasional Langhans giant cells
and lymphocytes.
Acellular, amorphous, eosinophilic amyloid deposits accounted for approximately 90% of
the nodal volume. The residual lymphoid tissue was benign with small but well-formed
germinal centers. Foreign body giant cells surround many of the amyloid deposits.
Amyloid deposits stain orange-red with Congo Red stain.
The follicles show abnormal germinal centers with marked vascular proliferation and hyalinization.
The center of the follicle is usually depleted of lymphocytes and shows follicular dendritic cells. The
follicle is surrounded by a broad mantle zone consisting of a concentric layering of lymphocytes
resulting in an onion-skin appearance. The follicles are frequently penetrated radially by a sclerotic
blood vessel.
The interfollicular stroma shows abundant hyperplastic vessels of post-capillary venule
type lined by plump endothelial cells. A mixed infiltrate of plasma cells, lymphocytes,
eosinophils, immunoblasts, and plasmacytoid monocytes is also present in the stroma.
Multicentric or Systemic form of Castlemans disease is usually of plasma cell type which shows diffuse
plasma cell proliferation in the interfollicular region. The image shows a small follicle in the center with
eosinophilic deposits of fibrin and immune complexes. Hyaline-vascular changes are absent. Plasma cell type
of Castlemans disease is often symptomatic and is accompanied by fever, anemia, elevated erythrocyte
sedimentation rate, hypergammaglobulinemia, and hypoalbuminemia. In contrast, hyaline-vascular type is
often asymptomatic.
High-power view of plasma cell type shows massive infiltration of the interfollicular region by plasma cells.
Occasionally, the plasma cells may be multinucleated or show atypia. The solitary form of Castlemans
disease (usually hyaline-vascular type) can be treated by surgical excision. The long-term prognosis of
systemic form of Castlemans disease (usually plasma cell type) is poor and it may evolve into a clonal
lymphoproliferative disorder.
The patient was a 35 y/o female from Vietnam with history of recurrent inguinal masses. Resection of
one of the subcutaneous nodules revealed hyperplasia of germinal centers and a prominent
perinodal eosinophilic infiltrate. The features are diagnostic of Kimuras disease an inflammatory
disorder of unknown origin endemic in the far east.
In the currently used REAL/WHO Classification, Hodgkins lymphoma has been subdivided into two main
categories: 1) Nodular Lymphocyte-predominant Hodgkins Lymphoma (NLPHL), and 2) Classical Hodgkins
Lymphoma. Recent molecular genetic evidence suggests that NLPHL is a form of B-cell lymphoma with kappa
light chain restriction. At microscopic level, the lymph node architecture is partially or totally effaced by
nodules which are more irregular in size and have mottled appearance as compared to those seen in
follicular lymphoma.
In Nodular Lymphocyte-predominant Hodgkins Lymphoma, classic Reed-Sternberg cells,
eosinophils, plasma cells, and areas of fibrosis are absent. Instead, numerous L&H cells
with multilobed, folded nucleus with small nucleoli are present in a background rich in
lymphocytes.
The neoplastic nodules of follicular lymphoma are more numerous and closely packed
together in contrast to those seen in reactive follicular hyperplasia. Loss of nodular
architecture and its replacement with diffuse growth signals worsening of prognosis.
Follicular limfoma
The neoplastic nodules in a follicular lymphoma are composed of small and large lymphoid cells. The
small cells have an irregular, elongated, cleaved nucleus with indentations and foldings surrounded
by scant cytoplasm. They are referred to as centrocytes, germinocytes, or small cleaved follicular cells.
The larger cells have round or oval vesicular nucleus with one or more prominent nucleoli. They are
referred to as centroblasts, germinoblasts, or lymphoblasts.
his image shows a homogenous population of small cleaved cells in a follicular lymphoma. The
monoclonal B-cells in neoplastic follicles express pan-B-cell markers, such as CD19, CD20, CD22,
and CD79a. Surface immunoglobulin, usually IgM, with light chain restriction is expressed. About
60% cases are CD10 positive; CD5 and CD43 are generally negative. Almost 85% of cases show
t(14;18) (q32;q21) translocation resulting in bcl-2 deregulation. Rearrangements of bcl-6 (located on
3q27) are seen in about 15% of cases.
Hiperplasia limfoid reaktif