Emel Ozyurek

Successful Use of Recombinant Factor VIIa (NovoSeven) During Cardiac Surgery in a Pediatric Patient with Glanzmann Thrombasthenia

Glanzmann thrombasthenia is a rare, hereditary, congenital disorder of platelet function characterized by inappropriate bleeding that is difficult to control. Recombinant activated factor VII (rFVIIa) is a new treatment that is used to stop bleeding and provide surgical support for these patients. This report describes the use of rFVIIa to prevent serious bleeding during and after open-heart surgery in a child with Glanzmann thrombasthenia.

FEBRILE NEUTROPENIA AS THE PRESENTING SIGN OF APPENDICITIS IN AN ADOLESCENT WITH ACUTE MYELOGENOUS LEUKEMIA

The diagnosis and management of a surgical abdomen in patients with acute leukemia is quite difficult because of the complications and treatment of disease itself. A 13-year-old boy with acute myelogenous leukemia developed 2 episodes of febrile neutropenia during induction therapy. The second one was treated with a 5-day course of parenteral antimicrobial therapy, but the patient then presented with right lower quadrant abdominal tenderness, guarding, and rebound tenderness. Abdominal ultrasonography and computed tomography revealed appendicitis. Conservative medical management was unsuccessful, and appendectomy was performed 5 days after appendicitis was diagnosed. The patients clinical manifestations resolved 5 days later. The case illustrates that fever may be the first manifestation of appendicitis in a child with acute myelogenous leukaemia who is neutropenic. Surgery is acceptable as first-line treatment in such cases.

HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS ASSOCIATED WITH VISCERAL LEISHMANIASIS: A Case Report

Leishmania-associated hemophagocytic lymphohistiocytosis is a rare clinicopathological entity. This condition is often difficult to diagnose, so treatment is often delayed. This report describes the case of a 5-year-old boy who was admitted with fever of 1 months duration, hepatosplenomegaly, and pancytopenia. Serum testing showed elevated transaminase levels, hypertriglyceridemia, hyperferritinemia, and normal fibrinogen level. Hemophagocytic lymphohistiocytosis was diagnosed on bone marrow examination. The patient was tested for various infectious agents. He was negative for all except Leishmania, which was detected by indirect fluorescent antibody testing. Treatment with amphotericin B resulted in a dramatic resolution of all signs and symptoms within 1 week.

Incidence of and risk factors for childhood thrombosis: a single-center experience in Ankara, Turkey.

This study was conducted to analyze the incidence of and risk for thrombosis in thrombotic children monitored in the Department of Pediatric Hematology of our hospital at the time of diagnosis, in addition to the clinical characteristics of those patients. The clinical and laboratory findings of 122 patients diagnosed with thrombosis from 1997 to 2006 were retrospectively analyzed. The incidence of thrombosis was 88.6/10,000 hospital admissions. The authors found that 31.1% of the patients studied had a thrombosis in more than 1 region. The incidence of thrombosis by anatomic site was as follows: 42 thromboses in the peripheral arterial system, 39 in an intracardiac region, 38 in the abdominal venous system, 36 in the deep peripheral venous system, and 28 in the cerebral vascular system. The mean age of the patients at the time of diagnosis was 4.9 years. Of the patients studied, 10.7% were neonates, 35.3% were infants younger than 1 year, and 48.4% were younger than 2 years. Most of the patients had a congenital cardiac disease and spontaneous thrombosis, and 66.1% had at least 1 acquired risk factor, the most common of which were having undergone surgery (42%) or wearing a central venous catheter (39%). A hereditary factor for the development of thrombosis was present in 54% of the patients. The most frequently observed hereditary risk factor was the MTHFR 677C-T mutation, and the second most common was the factor V Leiden mutation. Thrombosis should be considered a systemic disorder, and thrombotic patients should be evaluated with appropriate methods. Acquired and hereditary risk factors should be analyzed systematically in thrombotic patients.

Delayed recognition of intrathecal methotrexate overdose.

A 10-year-old girl who presented to our hospital was diagnosed as having B-precursor cell acute lymphoblastic leukemia. St Judes Total XIII protocol was started. In the second block of the consolidation phase, 10 hours after triple intrathecal treatment, we realized that instead of 12 mg, 120 mg of methotrexate had accidentally been given. Although the patient had no symptoms 10 hours after intrathecal treatment, to prevent the possible neurotoxic effects of methotrexate, a cerebrospinal fluid exchange was performed. Simultaneously, systemic dexamethasone and calcium folinic acid were given. At the time of this writing (2 y), the patient has had no symptoms and has continued on the chemotherapy protocol as planned. Administration of high-dose intrathecal methotrexate may not lead to symptoms, as was the case in our patient. This may be related to individual variations in cerebrospinal fluid dynamics and drug metabolism.

Hydrops fetalis in a neonate with down syndrome, transient myeloproliferative disorder and hepatic fibrosis.

Transient myeloproliferative disorder is a self limiting disorder characterized by leukocytosis with the presence of megakaryoblasts in the peripheral blood and bone marrow, anemia, thrombocytopenia, and organomegaly. It occurs in approximately 10% of newborn infants with Down syndrome. Hepatic fibrosis is seen in the severe form of transient myeloproliferative disorder with Down syndrome that is characterized by diffuse intralobular sinusoidal fibrosis and extramedullary hematopoesis. We describe a patient with hydrops fetalis, Down syndrome, and transient myeloproliferative disorder. We suggest that patients with the severe form of transient myeloproliferative disorder should be examined for hepatic fibrosis.

Successful use of short-course high-dose methylprednisolone in a child with acute myeloblastic leukemia (FAB M2) and myeloid tumor

ture myeloid precursors in blood, splenomegaly, fibrosis or huge megakaryocytes. Although some patients with thrombocytosis and ringed sideroblasts have been classified as having essential thrombocythemia or prefibrotic chronic idipathic myelofibrosis in previous reports [10], in our opinion, these 11 cases should be not classified within any of these entities because ringed sideroblasts are not found in classic MPD. In summary, all 11 patients presented features of both MDS and MPD and would be better classified as MDS/MPD rather than as RARS. Of importance, none of these 11 patients satisfies the criteria of CMML, aCML or JCML, which comprise MDS/ MPD characterized by the predominance of neutrophilic or monocytic effective proliferation, but usually with a normal or decreased platelet count. By contrast, these 17 patients are characterized by megakaryocytic proliferation and thombocytosis with leukocyte counts that are normal or only mildly increased. In our opinion, these patients may constitute one subgroup within the MDS/MPD U; MDS/MPD with thombocytosis.

CARDIAC INVOLVEMENT IN AN ADOLESCENT WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Cardiac complications of the pediatric patients with acute leukemia are common. Most of the cardiac complications may be due to chemotherapeutics such as antracyclins, besides anemia, infections, or direct leukemic infiltrations of the heart. It is reported that leukemic infiltration is frequent in the postmortem examination of the myocardium and pericardium. However, at the antemortem examination, pericardial involvement is rare and there is no myocardial involvement reported at the time of diagnosis in patients with acute leukemia in the English literature. Here, the authors report an adolescent with acute lymphoblastic leukemia who had myocardial infiltration at the time of diagnosis.

Bone fracture: an unusual presentation of acute megakaryoblastic leukemia.

Some clinical manifestations of acute leukemia in children can mimic orthopedic conditions, and t is variable presentation often makes diagnosis difficult. Bone changes in leukemia are well documented, but there are only a few accounts of children with acute leukemia who present with bone fractures. This report describes a case of this rare combination in a very young boy who presented with fractures of both proximal humerus and left proximal femur and massive periosteal reactions of both humerus and femur and also cystic lesions of proximal femur and iliac bone accompanying aggressive acute megakaryoblastic leukemia.

PERIPHERAL BLOOD PICTURE FOLLOWING MILD HEAD TRAUMA IN CHILDREN

Background: The aim of the present study was to investigate changes in peripheral white blood cell, and differential counts following mild head trauma in a pediatric population.