An extremely uncommon complication of ITP: Spontaneous rupture of an ovarian follicle cyst and massive intra-abdominal bleeding

Idiopathic thrombocytopenic purpura usually presents with minor bleeding such as petechia and purpura. Rarely, life-threatening events as intracranial and intra-abdominal bleedings can be seen. We would like to present a rare case diagnosed as idiopathic thrombocytopenic purpura, presenting with abdominal pain and paleness. In this 17-year-old female patient, extensive abdominal sensitivity was revealed on physical examination and massive intra-abdominal hemorrhage secondary to distended ovarian follicle rupture was seen on ultrasonography and abdominal computed tomography. The case was treated successfully with intravenous immunoglobin, thrombocyte suspension, and pulse methylprednisolone

Evaluation of plasma tissue factor and tissue factor pathway inhibitor levels in childhood hemangiomas

Background The purpose of the study was to evaluate the plasma levels of tissue factor (TF), an angiogenic marker, and tissue factor pathway inhibitor (TFPI), an anti proliferative protein, in the childhood hemangiomas at proliferative and regressive stages

Thrombocytopenia in neonates: causes and outcomes

Thrombocytopenia is one of the most common hematological abnormalities found in neonates. The causes, treatment modalities, and outcomes of neonatal thrombocytopenia have to be evaluated for providing better care and follow-up. In this study, our aim was to assess the prevalence, causes, treatment modalities, and outcomes of thrombocytopenia in neonates. A retrospective analysis was conducted on the medical records of all neonates hospitalized at our hospital between January 2007 and December 2011 and those with thrombocytopenia were included in the study. Of the 3,515 neonates, 134 (3.8 %) had thrombocytopenia. Ninety-seven of them (72 %) were preterm. In the patients admitted to neonatal intensive care unit, the prevalence of thrombocytopenia was found as 12 %, whereas it was found as 1.2 % in neonatal service. The highest prevalence was detected in the year 2008 by 5.3 %, and the lowest prevalence was detected in the year 2011 by 2.4 %. Sepsis was the most common etiologic factor between years 2007 and 2009. Intrauterine growth restriction, metabolic disorders, drugs, and asphyxia were more common causes in the recent years. Severe thrombocytopenia was found in 26 % of neonates and 11 % of thrombocytopenic neonates had major hemorrhage. Intracranial hemorrhage ratio was 5.9 % and all of these patients were preterm. Thrombocytopenia improved in 92.5 % of patients and persisted in 3 % of patients. Death occurred in 4.5 % of neonates. This study shows that the causes of neonatal thrombocytopenia may show variations with respect to time and the prevalence, complications, and risks of thrombocytopenia may be lowered by eliminating preventable factors

B-cell-activating factor, a proliferation inducing ligand and co-stimulatory molecules in the pathogenesis of immune thrombocytopenia in childhood

The aim of this study was to measure the levels of B-cell-activating factor (BAFF), a proliferation-inducing ligand (APRIL), and co-stimulatory molecules in immune thrombocytopenia (ITP) of childhood to investigate the interaction between T and B lymphocytes and the impact of proliferation of B lymphocytes in the pathogenesis. Twenty newly diagnosed ITPs, 20 chronic ITPs, and 20 healthy controls between 2 and 18 years were enrolled in this study. Hemogram, BAFF, APRIL, interleukin-4, and interferon (IFN)-gamma levels in sera and expressions of CD19, CD 3, CD21, CD40, and CD 154 on leukocytes were measured by ELISA and flow cytometry. Mean BAFF level in newly diagnosed ITP group was higher than the mean BAFF level in other groups. BAFF levels were significantly decreased after the treatment in newly diagnosed ITP group. APRIL, interleukin-4, and IFN-gamma in newly diagnosed ITP group and BAFF, APRIL, interleukin-4, and IFN-gamma in chronic ITP group were similar before and after treatment. There was no statistical difference for expressions of CD 19 and CD3 on lymphocytes, CD40 on leukocytes, CD154 on T cells, and for percentages of CD21(+)/CD40(+), CD21(-)/CD40(+), CD21(+)/CD40(-) B cells, and CD19(-)/CD3(-) lymphocytes for pretreatment and posttreatment levels in both ITP groups. In conclusion, our study strongly demonstrated that BAFF has an important role in the pathogenesis of newly diagnosed childhood ITP. It may be important in the follow-up and in novel therapy modalities of these patients. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved

The evaluation of protein Z levels of children with acute lymphoblastic leukaemia during induction therapy

The objective of this study was to evaluate the protein Z levels of children with acute lymphoblastic leukaemia (ALL) during induction therapy. Although several studies investigated the association between steroid and L-asparaginase (L-ASP) administration and levels of coagulation proteins such as protein C, protein S and antithrombin in children with ALL, protein Z levels have not been examined in any study yet. Peripheral blood was drawn from the study group before chemotherapy (PZ0) at diagnosis, at 12th day (PZ1), 15th day (PZ2), 18th day (PZ3) and 21st day (PZ4) of treatment wherein L-ASP treatment is given along with steroid administration according to ALL BFM-1995 chemotherapy protocol. Plasma protein Z levels were measured by enzyme immunoassay method. Mean protein Z level at PZ0 was 1.628 +/- 0.485 mu g/ml in the study group and 1.672 +/- 0.662 mu g/ml in the control group. No statistical difference was observed. In the study group, there was a slight increase in protein Z levels between the PZ0 and PZ1 periods in which only steroid therapy was administered. Statistically significant decrease was observed between protein Z levels in PZ0 - PZ4, PZ1 - PZ2, PZ1 - PZ3, PZ1 - PZ4 and PZ3 - PZ4 periods. During the induction treatment, symptomatic haemorrhage or thrombosis was not followed up in any patients. We demonstrated that children with ALL have similar protein Z values to those of the control group at diagnosis. A significant decrease occurs at the end of the induction treatment with steroid and L-ASP administration. However, this deficiency does not result in development of symptomatic thrombosis or bleeding in these patients. (c) 2013 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins

Veno-occlusive disease in a child with rhabdomyosarcoma after conventional chemotherapy: Report of a case and review of the literature

Although veno-occlusive disease of the liver is a well-known complication of high-dose chemotherapy and bone marrow transplantation, it has rarely been observed in children who receive conventional chemotherapy. Most cases in the literature consists of children with Wilms tumor. It has been very uncommon in rabdomyosarcoma patients until recently, although they commonly receive similar anticancer agents. Here the authors report a 2-year-old boy with rhabdomyosarcoma who developed veno-occlusive disease while receiving VAC (vincristine, actinomycin D, cyclophosphamide) chemotherapy regimen according to the IRS-IV protocol. The patient gradually recovered during 2 weeks with supportive treatment only

Multicentric Plasma Cell Type of Castleman Disease in a Child: Difficulty in Diagnosis and Treatment

Multicentric plasma cell variant of Castleman disease (CD) has rarely been reported and the optimal therapeutic approach is unknown, especially in childhood. In this case report, we discuss the case of a 7-year-old boy with multicentric plasma cell variant of CD, who presented with cervical lymphadenopathies, autoimmune hemolytic anemia, bone marrow insufficiency, pulmonary, renal, hepatic, and gastrointestinal involvement, emphasizing the difficulty in diagnosis and treatment approach

Evaluation of the Clinical and Laboratory Characteristics of Previously Followed-up Thalassemia Intermedia Patients to Provide Them Better Care in the Future

The increased awareness about the severity of complications in thalassemia intermedia patients led authorities to develop strategies for better management and follow-up of these patients. In this study, we aimed to define the clinical and laboratory characteristics in previously followed-up -thalassemia intermedia patients and wanted to gain an insight about the follow-up of this patient population in a developing country to provide them better care in the future. The mean age at diagnosis was 4 years, and the mean hemoglobin was 7.13 g/dL. The mean age at the beginning of regular transfusion was 4.8 years. An overall 74% of patients were on a regular transfusion program. The mean ferritin values at diagnosis and the last follow-up were 487 and 1225 ng/mL, respectively. The most common mutations detected in patients were IVS-I-110, IVS-I-6, IVS-II-1, and FCS 8/9 in order of frequency. Complications were seen in 48% of patients. The most common complications were osteopenia/osteoporosis (34%), growth retardation (24%), hypogonadism (18%), and cardiomyopathy (13%). In conclusion, the relatively higher complication rate in our patients who were previously treated highlights once again the need for an increased effort for optimal management and follow-up of this specific group of patients