Rabdomiosarkoma Pada Anak: Luaran Klinis Pada Pasien Yang Mendapat Terapi

Latar belakang. Rabdomiosarkoma (RMS) merupakan keganasan jaringan lunak yang banyak terjadi pada anak umur 1 sampai 5 tahun dan remaja. Sekitar 15 % anak dengan RMS datang dalam keadaan metastasis dan prognosisnya tidak ada perbaikan dalam 15 tahun terakhir.Tujuan. Penelitian ini untuk mengetahui gambaran epidemiologi RMS, hasil penanganannya dan luaran klinis untuk pengembangan selanjutnya.Metode. Penelitian dilakukan secara retrospektif dari data pada catatan medis 44 pasien yang diterapi di RS Cipto Mangunkusumo (RSCM), Jakarta bulan Juni 2000 sampai Juli 2008. Data dikumpulkan untuk melihat gambaran epidemiologi klinik dan luaran klinis.Hasil. Terdapat 44 pasien RMS di RSCM selama tahun 2000-2008.. Perbandingan antara laki-laki dan perempuan adalah 2:1 .Kebanyakan menyerang anak umur antara 3 bulan sampai 5 tahun (47,7% ) dengan median antara 6 -7 tahun. Gambaran patologi terbanyak yaitu embrional (65,9% ). Lokasi primer terbanyak pada bagian kepala dan leher (47,7% ). Berdasarkan sistem TNM , didapatkan stadium lanjut sebesar 61,4% . Sebagian besar metastasis ditemukan pada sumsum tulang (74%). Hasil luaran klinis didapatkan yang masih dalam terapi 52,3% , meninggal 36,4% dan lost to follow up 11,3% .Kesimpulan. RMS kebanyakan didapatkan pada anak umur 3 bulan sampai 5 tahun. Gambaran histologi tipe embrional dan lokasi primer pada kepala dan leher merupakan yang tersering. Pada umumnya, pasien datang dalam stadium lanjut tetapi jika datang pada stadium awal akan memberikan hasil yang lebih baik.Kata kunci Rabdomiosarkoma, epidemiologi klinik, luaran klini

Integrative Approach in Haemophillic Arthropathy of The Knee: a Case Report

Haemophilic arthropathy is the most prevalent joint disorder in haemophilia. This disorder is characterized by chronic synovitis and progressive destruction of joint cartilage. We report a case of arthroscopic synovectomy to reduce bleeding frequency in haemophilic arthropathy of the knee. Patient was a 15 years old male with haemophilic arthropathy of the left knee. We performed an arthroscopic synovectomy under tightly regulated factor VIII replacement therapy. There were villous synovial hypertrophy at all part of the joint, multiple bone and cartilage defect, and also anterior cruciate ligament (ACL) and posterior cruciate ligament (PCL) deficiency found intraoperatively. After 6 month follow up, subjective complain and bleeding frequency decreased significantly. The visual analog scale improved from 5-6 to 1-2, and the International Knee Documentation Committee Score increased from 49 to 66. Bleeding frequency decreased from 4-8 times per month to less than 1 time per month. Arthroscopic synovectomy performed in this case could reduce the pain, decrease the frequency of bleeding, and improve patient’s functional outcome.Key words: arthroscopic synovectomy, haemophilic arthropathy, haemophilia, anterior cruciate ligament, posterior cruciate ligament

MTHFR C677T and TS 5’-UTR 3R/3R Gene Polymorphism in Methotrexate-Resistant Childhood Acute Lymphoblastic Leukemia

BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy in Indonesia and often treated by methotrexate (MTX). Though it can be cured in 30-60% of patients, MTX resistance remains the major cause of treatment failure in childhood ALL. Previous sudies showed that its anti-leukemic property was moderated by MTX ability to inhibitmethylene tetra hydrofolate reductase (MTHFR) and thymidylate synthase (TS) in folate metabolism. This study investigates the correlation between MTHFR and TS polymorphism and MTX resistance in ALL children.METHODS: A total of 155 subjects obtained from all subjects prior to chemotherapy. DNA from blood samples were extracted and underwent polymerase chain reactionrestriction fragment length polymorphism (PCR-RFLP) to evaluate MTHFR C677T and TS 5’-UTR 3R/3R polymorphism.RESULTS: There was significant correlation between MTHFR C677T and TS 5’-UTR 3R/3R gene polymorphism with MTX resistance. Subjectswith MTHFR C677T and TS 5’-UTR 3R/3R gene polymorphism were 4 times (p=0.007) and 6.4 times (p=0.001) more likely to be MTX resistant than those without gene polymorphisms, respectively.CONCLUSION: MTHFR C677T andTS 5’-UTR 3R/3R represent dominant gene polymorphism related to MTX resistance in childhood ALL.KEYWORDS: gene polymorphism, folate metabolism, acute lymphoblastic leukemi

Infeksi Jamur Sistemik pada Pasien Immunocompromised

Dalam keadaan normal relatif sedikit spesies jamur yang patogenik. Akan tetapi pada beberapa keaaan tertentu seperti defisiensi imun (immunocompromised) beberapa spesies jamur dapat menyebabkan infeksi, terutama pada pasien dengan keadaan defisiensi imun (immunocompromised). Beberapa kondisi yang dapat menimbulkan keadaan immunocompromised antara lain ialah neutropenia, adanya kerusakan pada imunitas seluler dan humoral, perubahan pada sawar fisik, gizi buruk, adanya obstruksi dan perubahan flora bakteri. Secara klinis infeksi jamur dibagi menurut tempat infeksi dan jenis patogenesisnya. Keadaan yang perlu mendapat perhatian khusus ialah infeksi jamur sistemik karena dapat meningkatkan angka mortalitas. Ada beberapa faktor yang menyebabkan jumlah kasus infeksi jamur sistemik bertambah pada kelompok pasien yang berbeda. Kelompok pasien ini perlu diketahui sehingga diagnosis dapat ditegakkan dan pencegahan infeksi jamur dapat dilakukan sehingga diharapkan dapat menurunkan angka morbiditas dan moralitas

Hemartrosis pada Hemofilia

Hemofilia merupakan penyakit gangguan perdarahan yang bersifat herediter dengan berbagai macam manifestasi perdarahan, salah satunya ialah hemartrosis atau perdarahan sendi. Hemartrosis dapat menimbulkan masalah jangka panjang yaitu terjadinya artropati kronik. Hal ini dapat dicegah dengan tatalaksana hemartrosis secara baik dan optimal

Treatment of childhood acute lymphoblastic leukemia in Jakarta: Result of modified Indonesian National Protocol 94

Background Before 1990, the survival rates of childhood acute lymphoblastic leukemia (ALL) patients remained low. In 1994, the Hematology Oncology Working Group of the Indonesian Pediatric Association constructed a national protocol based on standard in- ternational protocol. As the outcome was still not promising, in 1998 the protocol was modified by introducing low dose MTX infusion for CNS prophylaxis. Objective To analyze the survival of pediatric ALL patient treated with the modified protocol in Cipto Mangunkusumo Hospital, Jakarta. Methods A prospective study was carried out to all newly diag- nosed and relapsed children with ALL from January 1998 through December 2004. Patients were stratified into standard risk group (SRG) and high risk group (HRG). HRG met with one of these criteria: WBC >50 000/ìl, the presence of CNS involvement, medi- astinal mass, relapse, or L 3 morphology. After completing induc- tion therapy, all patients received low-dose MTX (LDMTX) infusion (500 mg/m 2 ), especially for those aged less than 3 years. If the patient could not afford LDMTX, cranial irradiation (CRT) was given. Results There were 309 patients, consisted of 190 SRG and 119 HRG patients. Male to female ratio was 1.8:1. Complete remission was achieved in 86.3% SRG patients compared with 63.8% in HRG pa- tients (P<0.05). Event-free survival (EFS) rate in SRG and HRG were 65.9% (95%CI 59.8; 71.9%) and 40.4% (95%CI 32.5; 48.4%), respec- tively. The overall survival (OS) rates in SRG was 81.2% (95%CI 76.3; 86.2%) and in HRG was 56.0% (95%CI 47.8; 64.2%). The overall OS and EFS for both groups were 71.6% (95%CI 67.0; 76.2%) and 59.6% (95%CI 54.5; 64.7%), respectively. Failure of therapy was mostly due to severe aplasia resulted in bleeding and severe infection. CNS re- lapse was rare in both groups, i.e. 3.1% in SRG and 0.8% in HRG. Conclusion Treatment of ALL using modified national protocol for SRG shows promising results. However, the outcome of HRG pa- tients is still inferior to those reported elsewhere. The use of low- dose MTX infusion can replace the role of cranial irradiation as CNS prophylaxis measure

Treatment of childhood acute lymphoblastic leukemia in Jakarta: Result of modified Indonesian National Protocol 94

Background Before 1990, the survival rates of childhood acute lymphoblastic leukemia (ALL) patients remained low. In 1994, the Hematology Oncology Working Group of the Indonesian Pediatric Association constructed a national protocol based on standard in- ternational protocol. As the outcome was still not promising, in 1998 the protocol was modified by introducing low dose MTX infusion for CNS prophylaxis. Objective To analyze the survival of pediatric ALL patient treated with the modified protocol in Cipto Mangunkusumo Hospital, Jakarta. Methods A prospective study was carried out to all newly diag- nosed and relapsed children with ALL from January 1998 through December 2004. Patients were stratified into standard risk group (SRG) and high risk group (HRG). HRG met with one of these criteria: WBC >50 000/ìl, the presence of CNS involvement, medi- astinal mass, relapse, or L 3 morphology. After completing induc- tion therapy, all patients received low-dose MTX (LDMTX) infusion (500 mg/m 2 ), especially for those aged less than 3 years. If the patient could not afford LDMTX, cranial irradiation (CRT) was given. Results There were 309 patients, consisted of 190 SRG and 119 HRG patients. Male to female ratio was 1.8:1. Complete remission was achieved in 86.3% SRG patients compared with 63.8% in HRG pa- tients (P<0.05). Event-free survival (EFS) rate in SRG and HRG were 65.9% (95%CI 59.8; 71.9%) and 40.4% (95%CI 32.5; 48.4%), respec- tively. The overall survival (OS) rates in SRG was 81.2% (95%CI 76.3; 86.2%) and in HRG was 56.0% (95%CI 47.8; 64.2%). The overall OS and EFS for both groups were 71.6% (95%CI 67.0; 76.2%) and 59.6% (95%CI 54.5; 64.7%), respectively. Failure of therapy was mostly due to severe aplasia resulted in bleeding and severe infection. CNS re- lapse was rare in both groups, i.e. 3.1% in SRG and 0.8% in HRG. Conclusion Treatment of ALL using modified national protocol for SRG shows promising results. However, the outcome of HRG pa- tients is still inferior to those reported elsewhere. The use of low- dose MTX infusion can replace the role of cranial irradiation as CNS prophylaxis measure

Sindrom Nefrotik Sekunder pada Anak Dengan Limfoma Hodkin

Sindrom nefrotik sekunder ialah sindrom nefrotik yang berhubungan dengan penyakit atau kelainan sistemik, seperti keganasan. Diantara keganasan tersebut adalah penyakit Hodgkin yang ditandai dengan limfadenopati. Beberapa kasus datang dengan gejala yang tidak biasa, di antaranya adalah sindrom nefrotik (0,4%). Sindrom nefrotik dapat merupakan salah satu sindrom paraneoplastik yaitu kumpulan sindrom klinis yang menyertai penyakit keganasan, yang timbul akibat efek sistemik keganasan tersebut namun bukan akibat metastasis. Laporan kasus ini membahas tentang sindrom nefrotik sekunder sebagai manifestasi sindrom paraneoplastik pada pasien limfoma Hodgkin. Seorang anak laki-laki berusia 5 tahun datang ke poliklinik Hematologi-Onkologi Departemen Ilmu Kesehatan Anak FKUI/RS Dr. Cipto Mangunkusumo (RSCM) dengan keluhan bengkak di seluruh tubuh dengan oliguria. Diagnosis limfoma Hodgkin telah ditegakkan satu bulan sebelumnya, berdasarkan pemeriksaan patologi anatomi. Tumor primer berada di kuadran kanan bawah rongga abdomen, serta didapatkan pembesaran kelenjar getah bening, soliter, di daerah inguinal kanan. Pemeriksaan laboratorium menunjukkan adanya anemia, peningkatan laju endap darah (LED), proteinuria masif, hipoalbuminemia, hiperkolesterolemia, dan fungsi ginjal yang normal. Pemeriksaan foto toraks, CT-scan abdomen, aspirasi sumsum tulang, tes sitologi terhadap urin dan cairan serebrospinal, tidak menunjukkan adanya metastasis jauh. Pasien didiagnosis sebagai sindrom nefrotik sekunder dan penyakit Hodgkin stadium 2. Pasien mendapat terapi berupa furosemid, infus albumin, prednison, dan sitostatik, yang terdiri dari siklofosfamid, vinkristin, etoposid, doxorubisin, bleomisin serta vinblastin

Coagulation abnormality as a complication of L-asparaginase therapy in childhood lymphoblastic leukemia

Background Bleeding, one of the most common symptoms of acute leukemia in untreated patients, is mostly due to thrombocy- topenia as a result of myeloinvasion by leukemic cells. Neverthe- less, a further contributory factor for the additional hemorrhagic complication during intensive chemotherapy is the myelosuppressive effect of most active drugs. L-asparaginase, one of the cytostatics used during remission induction therapy for childhood of acute lymphoblastic leukemia (ALL), is widely reported to impair the he- mostatic system. Objective To determine the influence of shorter courses of L-as- paraginase (L-Ase) on some of the hemostatic parameters in the treatment of childhood ALL. Methods A prospective analytical study was carried out in the Department of Child Health, Cipto Mangunkusumo Hospital Jakarta from July 1, 1999 to June 30, 2001 on newly diagnosed ALL pa- tients with normal liver function tests treated according to our na- tional ALL protocol which one of its composition contained 6 in- stead of 9 injections of L-asparaginase. Results All of 30 children with ALL included in the study, experi- enced prolongation of prothrombin time (PT), activated partial thromboplastin time (aPTT), and decreased fibrinogen concentra- tion, markedly during the administration of L-asparaginase. How- ever, none of the patients had additional hemorrhage or evidence of disseminated intravascular coagulation (DIC). Conclusion The use of shorter courses of L-asparaginase, 6 in- jections, in the remission induction chemotherapy of childhood ALL in our department may reduce the blood clotting factors without further hemorrhage complication or evidence of DI