Acute lymphoblastic leukemia

Sahara Effendy

Acute lymphoblastic leukemia

Def : Keganasan klonal dari sel-sel pre-kursor limfoid Epidemiologi :
Insidensi 1/60.000 orang per tahun 75% kurang dari 15 tahun Insidensi puncak usia 3-5 tahun Pria > Wanita Saudara kandung dr pasien ALL punya resiko 4x lebih besar Kembar monozigot dr pasien ALL punya resiko 20% menderita ALL

Etiologi :
Penyebab pasti belum diketahui Faktor lingkungan yang diketahui dapat berpengaruh
Radiasi Ionik Paparan benzene kadar tinggi Merokok Obat kemoterapi Infeksi Virus Epstein Barr Pasien Sindroma Down, Wiskoot-Aldrich

Cytogenetic translocations associated with specific molecular genetic abnormalities in ALL

12:21 is the most common translocation and portends a good prognosis. 4:11 is the most common in children under 12 months and portends a poor prognosis.

Translokasi kromosom pd ALL

-Hanya dapat dideteksi dengan pulse-field gel electroporesis atau reverse transcriptase polymerase chain reaction - ABL : Non receptor tyrosine protein kinase yang secara enzim mentrasfer molekul fosfat ke substrat protein, sehingga terjadi aktivasi jalur transduksi sinyal yang penting dalam regulasi proliferasi dan pertumbuhan sel

Klasifikasi ALL
Morfologik Imunologik Precursor B-Acute Lympoblastic Leukimia (ALL)-70%, Common ALL (50%), null ALL, pre-B ALL T-ALL (25%) B-ALL (5%)

Clinical Features at Diagnosis in Children with ALL

Fever is the most common finding, occurring in approximately 60% of patients. Progressive bone marrow failure leads to pallor (anemia), bleeding (thrombocytopenia) and susceptibility to infections (neutropenia). Over one third of patients may present with a limp, bone pain, arthralgia or refusal to walk due to leukaemia infiltration of the periosteum, bone or joint, or to the expansion of the marrow cavity by leukaemia cells. 60 to 70% of children have enlargement of the liver and/or spleen Lymphadenopathy (usually painless, localized or generalized) in the neck, under arms, and groin due to leukaemia infiltration is an equally frequent presenting sign The first symptoms are usually non-specific and include anorexia, weight loss and/or loss of appetite Generalized weakness and fatigue

Daftar Pustaka
Harrisons Principles of Internal Medicine,16th Edition Chapter 97. Malignancies of Lymphoid Cells. Clinical Features, Treatment, and Prognosis of Specific Lymphoid Malignancies. Collier, J.A.B (1991). Oxford Handbook of Clinical Specialties, Third Edition. Oxford. pp. 810. http://www.nlm.nih.gov/medlineplus/ency/article/000541.htm

KARISS SAYAANGG, MAKASIIH YAA :* Hehehehe