The variant translocation of ABL1 gene t(2;9)(q21;q34) in a childhood T-cell acute lymphoblastic leukemia.

We present the case of the childhood ALL that was identified by the translocation of the ABL1 gene to the q21 band of chromosome 2 without t(9;22)(q34;q11) translocation. The observation of a poor clinical course of the case may contribute to explanation of the action of t(9;22)(q34;q11) translocation, of which poor prognostic action is known on ALL's, in terms of ABL1 gene, independent of the BCR gene. On the other hand, the prognostic significance of this variant ABL1 translocation detection, which is very rarely observed, will cast a light on future cases (Tab. 1, Fig. 1, Ref. 11).

Neonatal outcomes of pregnancy complicated by idiopathic thrombocytopenic purpura.

OBJECTIVE: The aim of this study was to determine the factors associated with the prognosis of newborns born to mothers with idiopathic thrombocytopenic purpura (ITP), and to compare the infants with/without thrombocytopenia in terms of maternal and neonatal characteristics. STUDY DESIGN: We reviewed the charts of 29 parturients with ITP and their newborns who were born between January 1998 and December 2008. RESULT: A total of 16 (55%) gravidas had been diagnosed with ITP before pregnancy and 13 (45%) were diagnosed during pregnancy. Thrombocytopenia was observed in 21 gravidas. In total, 17 (58%) gravidas received treatment to increase the platelet count. The majority of deliveries (72.5%) were vaginal. The infant platelet counts at birth ranged from 20 to 336 x 10(9) per liter. None of the neonates had complications attributable to the mode of delivery. Normal platelet counts were determined in 15 newborns, whereas 14 infants had thrombocytopenia at birth. Three (10.3%) neonates had mild, four neonates (13.7%) had moderate and seven neonates (24.1%) had severe thrombocytopenia. The age of the mothers having infants with thrombocytopenia was significantly higher (30+/-5.3 vs 25.3+/-3.8 years), most of the infants (10/14 (71%)) were males (P<0.05). CONCLUSION: Pregnancy complicated with ITP generally has a good outcome. Although ITP in pregnancy carries a low risk, careful observation is required for the newborn of gravidas with ITP even when the infant has no bleeding complications at delivery, and infants may require treatment for thrombocytopenia.

Low-dose immune tolerance induction for paediatric haemophilia patients with factor VIII inhibitors.

The development of an inhibitor against factor VIII (FVIII) is a serious complication in children with haemophilia A. Immune tolerance induction (ITI) therapy is generally considered to be the best approach to eradicate the inhibitor. In this paper, the low-dose (< or =50 IU kg(-1) twice or three times weekly with plasma-derived factor concentrates) ITI regimen used in Turkey is discussed. This regimen was given to 21 haemophilia A patients with high titer inhibitors. The median age at the beginning of ITI was 9 years and exposure days were 25. The median pre-ITI historical peak inhibitor titer, and inhibitor titer when ITI started were 80 BU (range 6.0-517), 19.2 BU (range 3.6-515), respectively. Complete immune tolerance was defined as the time at which at least two negative inhibitor assays was obtained with no anamnestic response. Our two cases were not reached in follow-up period. Immune tolerance could be achieved in 5 of 19 (26.3%) patients within a median time of 6 months. Partial tolerance was obtained in 7 patients while treatment failed in spite of significant decreased inhibitor levels in the other patients. A relapse developed in one immune-tolerized patient, one year later. The level of inhibitor titer at the beginning of ITI (< or =10 BU), the pre-ITI historical peak inhibitor titer (<50 BU), and the time between the first diagnosis inhibitor to starting ITI (<12 months) were main factors in the success (complete or partial tolerance) of ITI. In conclusion, the outcome of low-dose ITI protocol was not satisfactory in this retrospective study.