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Limfoma Non-Hodgkin (LNH)

Definisi

 Kelompok keganasan primer limfosit yg dapat berasal dari


limfosit B, limfosit T, dan kadang (amat jarang) berasal
dari sel NK (“natural killer”) yg berada dlm sistem limfe;
yg sangat heterogen, baik tipe histologis,gejala, perjalanan
klinis, respon thdp pengobatan, maupun prognosis.
 Pada LNH sebuah sel limfosit berploriferasi secara tidak
tekendali tumor
Epidemiologi

 Pada th 2000 di AS diperkirakan terdapat 54.900


kasus baru , dan 26.100 meninggal karena LNH.
 Insidensi LNH di AS menurut National Cancer
Institute th 1996 a/ 15.5 per 100.000. LNH secara
umum lebih sering pd pria.
 LNH meningkat seiring dg bertambahnya usia dan
mencapai puncak pd usia kelompok 80-84 th.
 Di indonesia sendiri LNH bersama sama dg
penyakit hodgkin dan leukimia menduduki urutan
ke-6.
Etiologi dan faktor resiko

• Idiopatik
F. resiko:
1. Imunodefisiensi
2. Agen infeksius. Ex: EBV, HTLV-1, HCV, helicobacter pylori
3. Paparan lingkungan dan pekerjaan
ex: peternak, pekerja hutan dan pertanian akibat
adanya paparan herbisida dan pelarut organik
4. Diet dan paparan lainnya
ex: makanan tingggi lemak hewani, merokok dan terkena
paparan sinar uv
Sign & symptoms

 Signs and symptoms of low-grade lymphomas include the


following:
 Peripheral adenopathy: Painless and slowly progressive;
can spontaneously regress
 Primary extranodal involvement and B symptoms:
Uncommon at presentation; however, common with
advanced, malignant transformation or end-stage disease
 Bone marrow: Frequent involvement; may be associated
with cytopenias(s) ; fatigue/weakness more common in
advanced-stage disease
 Intermediate- and high-grade lymphomas have a more
varied clinical presentation, including the following:
 Adenopathy: Most patients
 Extranodal involvement: More than one third of patients;
most common sites are GI/GU tracts (including Waldeyer
ring), skin, bone marrow, sinuses, thyroid, CNS
 B symptoms: Temperature >38°C, night sweats, weight loss
>10% from baseline within 6 months; in approximately 30-
40% of patients
Stadium penyakit
Diagnosis
• Examination in patients with  Hepatomegaly
low-grade lymphomas may  Large abdominal mass: Usually
demonstrate peripheral in Burkitt lymphoma
adenopathy, splenomegaly, and
hepatomegaly.  Testicular mass

• Intermediate- and high-grade  Skin lesions: Associated with


lymphomas may result in the cutaneous T-cell lymphoma
following examination findings: (mycosis fungoides), anaplastic
large-cell lymphoma, and
 Rapidly growing and bulky
angioimmunoblastic lymphoma
lymphadenopathy
 Splenomegaly
 Testing
• Laboratory studies in a patient with suspected NHL should
include the following:
 CBC count: May be normal in early-stage disease; in more
advanced stages, may demonstrate anemia,
thrombocytopenia/leukopenia/pancytopenia,
lymphocytosis, thrombocytosis
 Serum chemistry studies: May show elevated LDH and
calcium levels, abnormal liver function tests
 Serum beta2-microglobulin level: May be elevated
 HIV serology: Especially in patients with diffuse large cell
immunoblastic or small noncleaved histologies
 Human T-cell lymphotropic virus–1 serology: For patients
with adult T-cell leukemia/lymphoma
 Hepatitis B testing: In patients in whom rituximab therapy is
planned because reactivation has been reported
 Other tests that may be helpful in evaluating suspected NHL
include the following:
 Immunophenotypic analysis of lymph node, bone marrow,
peripheral blood
 Cytogenetic studies: NHL occasionally associated with
monoclonal gammopathy; possible positive Coombs test;
maybe hypogammaglobulinemia
Imaging test

 The following imaging studies should be obtained in a


patient suspected of having NHL:
 Chest radiography
 Upper GI series with small bowel follow-through: In
patients with head and neck involvement and those with a
GI primary lesion
 CT scanning of the neck, chest, abdomen, and pelvis
 PET scanning
 Bone scanning: Only in patients with bone pain, elevated
alkaline phosphatase, or both
 Testicular ultrasonography: For opposite testis in male
patients with a testicular primary lesion
 Multiple gated acquisition (MUGA) scanning: For patients
being considered for treatment with anthracyclines
 MRI of brain/spinal cord: For suspected primary CNS
lymphoma, lymphomatous meningitis, paraspinal lymphoma,
or vertebral body involvement by lymphoma
Terapi

Chemotherapy: Most common; usually combination regimens


 Radiation therapy
 Rituximab administration
 Bone marrow transplantation: Possible role in relapsed high-
risk disease
 Radioimmunotherapy
 Transfusions of blood products
 Antibiotics
b. Biopsi
• biopsi KGB dilakukan hanya 1 kelenjar yg paling
respresentatif, superfisial dan perifer. Jika terdapat kelenjar
perifer/superfisial yg respresentatif, maka tidak perlu biopsi
yg intra abdominal atau intratorakal . Spesimen kelenjar
diperiksa:
- rutin : hitopatologi ( REAL-WHO dan WF)
- khusus : imunoglobulin permukaan, histo/sitokimia
• Diagnosis ditegakkan berdasarkan histopatologi dan sitologi
c. Aspirasi sumsum tulang dan biopsi sumsum tulang dari 2 sisi
spina iliaca dg hasil spesimen dg panjang 2cm
Farmakologi
 Cytotoxic agents (eg, chlorambucil, cyclophosphamide, doxorubicin,
vincristine, fludarabine, pralatrexate, nelarabine, etoposide, mitoxantrone,
cytarabine, bendamustine, carboplatin, cisplatin, gemcitabine, denileukin
diftitox, bleomycin)
 Histone deacetylase inhibitors (eg, vorinostat, romidepsin, belinostat)
 Colony-stimulating factor growth factors (eg, epoetin alfa, darbepoetin
alfa, filgrastim, pegfilgrastim)
 Monoclonal antibodies (eg, rituximab, ibritumomab
tiuxetan, alemtuzumab, ofatumumab, obinutuzumab, pembrolizumab)
 mTOR (mammalian target of rapamycin) kinase inhibitors (eg,
temsirolimus), Proteasome inhibitors (eg, bortezomib),
Immunomodulators (eg, interferon alfa-2a or alfa-2b),Corticosteroids
Prognosis

 Indolent lymphoma : prognosis relatif baik, dg median


survival 10 th , tetapi biasanya tdk bisa disembuhkan pd
stadium lanjut
 Agresif lymphoma : memiliki perjalanan almaiah yg lebih
pendek, namun lebih dapat disembuhkan secara signifikan
dg kemoterapi kombinasi intensif. Resiko kambuh lebih
tinggi pd pasien dg gambaran histologi “ divergen” baik pd
kelompok indolent maupun agresif