Hyperleukocytosis in childhood acute lymphoblastic leukemia: complications and treatment outcomeGülersu İrken1, Hale Ören1, Haldun Öniz2, Nazan Çetingül3, Canan Vergin4, Berna Atabay2, Hüseyin Gülen4, Meral Türker2, Mehmet Kantar3, Şebnem Yılmaz11Department Of Pediatric Hematology, Dokuz Eylül University Faculty Of Medicine, İzmir, Turkey
2Department Of Pediatric Hematology- Oncology, Tepecik Teaching Hospital, İzmir, Turkey
3Department Of Pediatric Hematology-oncology, Ege University Faculty Of Medicine, İzmir, Turkey
4Department Of Pediatric Hematology-oncology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
Hyperleukocytosis, defined as a peripheral leukocyte count ≥ 100x109/L, is seen in 5-20% of newly diagnosed cases of childhood leukemia and is a poor prognostic factor. In this study, we aimed to examine the presenting clinical and laboratory features, complications, and treatment outcome of 47 children with acute lymphoblastic leukemia (ALL) and hyperleukocytosis who were diagnosed and treated in four medical centers of İzmir between January 1990 and January 2001. The median age was 5.0 years (range: 0.1-16.3 years). Median white blood cell count was 495x109/L (range: 107x109/L- 794x109/L). Forty-two of 47 patients (90%) had hepatosplenomegaly, 5 (11%) had respiratory distress, 3 (6%) had neurologic symptoms, 3 (6%) had diffuse cervical lymphadenopathy, and 3 (6%) had acute renal failure at admission. Ten of 47 patients (21%) had central nervous system involvement, and 17 (36%) had mediastinal mass. Ten patients (21%) had coagulopathy and 15 patients (32%) had metabolic complications (8 patients had hyperuricemia, 4 had hyperphosphatemia, 2 had hyperuricemia, hyperphosphatemia and hypercalcemia, and 1 had hypocalcemia) before the initiation of therapy. Forty of 47 patients (85%) with hyperleukocytosis were effectively managed with intravenous hydration, alkalinization, and allopurinol therapy. Early death during remission induction therapy occurred in 5 patients (11%) with respiratory distress and sepsis. Kaplan-Meier estimates of event free survival and overall survival were 37.0% and 40.5%, respectively. Keywords: Hyperleukocytosis, lymphoblastic leukemia, survival
Çocukluk çağı akut lenfoblastik lösemisinde hiperlökositoz: komplikasyonlar, tedavi, prognozGülersu İrken1, Hale Ören1, Haldun Öniz2, Nazan Çetingül3, Canan Vergin4, Berna Atabay2, Hüseyin Gülen4, Meral Türker2, Mehmet Kantar3, Şebnem Yılmaz11Department Of Pediatric Hematology, Dokuz Eylül University Faculty Of Medicine, İzmir, Turkey
2Department Of Pediatric Hematology- Oncology, Tepecik Teaching Hospital, İzmir, Turkey
3Department Of Pediatric Hematology-oncology, Ege University Faculty Of Medicine, İzmir, Turkey
4Department Of Pediatric Hematology-oncology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
Periferik kanda lökosit sayısının ≥100x109/L olması ile karakterize hiperlökositozis, çocukluk çağı lösemilerinde %5-20 oranında görülebilen ve prognozu kötü olarak etkileyen bir risk faktörüdür. Bu çalışmada, 1 Ocak 1990-1 Ocak 2001 tarihleri arasında İzmir’deki 4 merkezde tanı alıp tedavi edilmiş ve tanı sırasında hiperlökositoz saptanmış 47 akut lenfoblastik lösemili (ALL) çocuğun klinik ve laboratuvar bulguları, gelişen komplikasyonlar ve sağkalımlarının araştırılması amaçlandı. Olguların ortanca yaşı 5.0 yaş (0,1-16,3 yaş) idi. Ortanca beyaz küre sayısı 495x109/L (107x109/L-794x109/L) bulundu. Tanı anında olguların 42’sinde (%90) hepatosplenomegali, 5’inde (%11) respiratuvar distres, 3’ünde (%6) nörolojik semptomlar, 3’ünde (%6) diffuz servikal lenfadenopati, 3’ünde (6%) akut böbrek yetmezliği bulguları vardı. On olguda (%21) santral sinir sistemi, 17 olguda (%36) mediastinal tutulum mevcuttu. Tedavi başlamadan önce 10 olguda (%21) koagulopati, 15 olguda (%32) metabolik bozukluk (8 olguda hiperürisemi, 4 olguda hiperfosfatemi, 2 olguda hiperürisemi, hiperfosfatemi ve hiperkalsemi, 1 olguda hipokalsemi) saptandı. 40 olguda (%85) intravenöz hidrasyon, alkalinizasyon ve allopurinol tedavisi ile hiperlökositoz kontrol altına alındı. İndüksiyon tedavisinin ilk 15 gününde 5 olgu (%11) respiratuvar distres ve sepsisle kaybedildi. Olguların hastalıksız ve genel sağkalımlarının sırasıyla %37,0 ve %40,5 olduğu saptandı. Anahtar Kelimeler: Hiperlökositoz, lenfoblastik lösemi, sağkalım
Gülersu İrken, Hale Ören, Haldun Öniz, Nazan Çetingül, Canan Vergin, Berna Atabay, Hüseyin Gülen, Meral Türker, Mehmet Kantar, Şebnem Yılmaz. Hyperleukocytosis in childhood acute lymphoblastic leukemia: complications and treatment outcome. Turk J Hematol. 2006; 23(3): 142-146
Corresponding Author: Hale Ören, Türkiye |
|