Feride Duru

Most reliable indices in differentiation between thalassemia trait and iron deficiency anemia

Background : Iron deficiency anemia (IDA) and thalassemia trait (TT) are the most common forms of microcytic anemia. Some discrimination indices calculated from red blood cell indices are defined and used for rapid discrimination between TT and IDA. However, there has been no study carried out in which the validity of all of the defined indices are compared in the same patient groups. Youdens index is the most reliable method by which to measure the validity of a particular technique, because it takes into account both sensitivity and specificity.

Hematologic Manifestation of Childhood Celiac Disease

We wanted to describe the hematologic manifestations of celiac disease (CD) in childhood. This study included 22 children with CD in whom the disease remained undiagnosed until they had presented with hematological abnormalities, such as anemia, thrombocytopenia, leukopenia or prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT). Anemia was present alone in 19 (86.3%) patients, and leukopenia coexisted with anemia in 2 (9%) patients. Thrombocytopenia was found alone in 1 (4.5%) patient. Twelve patients had an iron deficiency anemia. Iron deficiency coexisted with zinc and vitamin B12 deficiency in 3 patients, copper and vitamin B12 deficiency in two, vitamin B12 deficiency in two, zinc deficiency in two and one patient had combined iron, zinc, and copper deficiency. Males had significantly lower values of hemoglobin (p < 0.05) and MCV (p < 0.05) compared to the females. In conclusion CD should be included in the differential diagnosis in children who present with anemia, leukopenia, thrombocytopenia or prolonged PT and APTT, especially in geographical areas where the prevalence of the CD is high.

CLINICAL COURSE OF CHILDREN WITH IMMUNE THROMBOCYTOPENIC PURPURA TREATED WITH INTRAVENOUS IMMUNOGLOBULIN G OR MEGADOSE METHYLPREDNISOLONE OR OBSERVED WITHOUT THERAPY

The authors compared the prognosis in 50 children with acute immune thrombocytopenic purpura (ITP) who received intravenous immunoglobulin G (IVIG), megadose methylprednisolone (MDMP), or no therapy. Twenty-six children were observed with no therapy, 12 children received IVIG, and 12 children received MDMP. The percentage of the patients whose platelet counts increased at a level of > 20 2 10 9 /L and > 50 2 10 9 /L at 3 days after starting therapy was significantly higher in both IVIG and MDMP groups than in the no therapy group ( p < .01), but there was no significant difference at 10 and 30 days after initiation between the 3 groups ( p > .05 in each comparison). This result suggested that therapy does not increase the rate of recovery but shortens the duration of thrombocytopenia in the first days. Management decision in ITP is made on clinical condition rather than on platelet count and no treatment options is to be preferred even in the face of mucosal bleeding. If the patient has extensive bleeding and the decision is to treat, both IVIG and MDMP are equally effective in providing a safe platelet level early on.

A comparison of the effect of high-dose methylprednisolone with conventional-dose prednisolone in acute lymphoblastic leukemia patients with randomization.

In this preliminary study the efficacy of high-dose methylprednisolone (HDMP) during remission-induction chemotherapy was evaluated on 166 children with acute lymphoblastic leukemia (ALL). The St. Jude Total Therapy Study XI protocol with minor modifications was used in this trial. Patients were randomized into two groups. Group A received conventional-dose (2 mg/kg/day orally) prednisolone, and group B received high-dose methylprednisolone (HDMP, Prednol-L, 900-600 mg/m2 orally) during remission-induction chemotherapy. Complete remission was achieved in 97% of the children. For the 80 patients who were followed up for 3 years, median follow-up was 44 (range 5-60) months and the 3-year event-free survival (EFS) rate was 68.5%) overall, 58.6% in group A and 78.4% in group B. The EFS among patients in group B was significantly higher than in group A (p=0.05). When we compared the 3-year EFS of groups A and B in the high-risk groups and high-risk subgroups with white blood cell (WBC) counts > or = 50 x 10(9)/l and age > or = 10 years, the survival rates were 45% versus 77.2%, 33% versus 78% and 45% versus 89%, respectively. During the follow-up of 162 patients, relapses were significantly higher in group A. Bone marrow relapses in 162 patients, and also in a subgroup of patients > or = 10 years of age were significantly higher in group A. These results suggest that HDMP during remission-induction chemotherapy improves long-term EFS, particularly for high-risk patients.

PARVOVIRUS B19 INFECTION REMINISCENT OF MYELODYSPLASTIC SYNDROME IN THREE CHILDREN WITH CHRONIC HEMOLYTIC ANEMIA

The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of trueclonal MDS.

Osteopetrosis and Glanzmann's thrombasthenia in a child

Autosomal recessive osteopetrosis is a rare, fatal disease characterized by accumulation of excessive bone mass due to defective bone resorption. The pathogenesis of osteopetrosis is controversial. Osteoblast-osteoclast interaction defects, incorrect differentiation of osteoclasts, abnormal contact between osteoclast and extracellular matrix, and abolished signaling are included in this process. Recently, mutations in the gene of the vacuolar proton pump have been described in some cases of recessive osteopetrosis. Glanzmanns thrombasthenia (GT) is a rare hereditary qualitative platelet disorder characterized by a lifelong bleeding tendency due to quantitative and qualitative abnormalities of the platelet integrin αIIbβ3. Several mutations on either integrin αIIb [glycoprotein (GP) IIb] or integrin β3 (GP IIIa) were reported in GT. We report on a patient with autosomal recessive osteopetrosis concurrently diagnosed with variant type Glanzmanns thrombasthenia. To our knowledge, our patient was the first case reported in the literature in which osteopetrosis and Glanzmanns thrombasthenia were diagnosed together.

Serum levels and differential expression of CD44 in childhood leukemia and malignant lymphoma: Correlation with prognostic criteria and survival

Abstract Background : The CD44, a cell surface proteoglycan, participates in a variety of function including tumor dissemination and metastasis. However, there are no available data on the prognostic significance of CD44 expression of tumor tissue correlated with serum sCD44 level in childhood leukemias and lymphomas.

High prevelance of chronic magnesium deficiency in T cell lymphoblastic leukemia and chronic zinc deficiency in children with acute lymphoblastic leukemia and malignant lymphoma.

Magnesium and zine are the elements having essential roles in regulation of cell growth, division and differentiation. There have been some studies in the literature suggesting an association between the deliciency of these elements and the development of malignant disorders. In this study hair and serum Zine and magnesium levels were investigited in children with acute lymphoblastic leukemia (ALL) and malignant lymphoma (ML) at the time of initial diagnosis. Ten children with T-cell ALL. 10 children with B-precursor ALL. 5 children with Burkitts Lymphoma (BL). II children with Hodgkins lymphoma (HL). 10 children with non-Burkitt non-Hodgkins lymphoma (NBNHL) and 12 age and sex matched healthy children as a control group were included in the study. Mean hair magnesium levels in all of the groups of the patients were lower than the levels in the control group but the difference was statistically significant only in the children with T cell ALL comparable to the controls (28.9±3.9μg/g and 87.6±18.5μg/g respetiveley, p<0.05). Mean serum magnesium levels in all the cohorts were not significantly different than those in controls (p>0.05 in each comparison). Mean hair zine levels in the patients with T-cell. B-precursor ALL, BL, HL, NBNHL were 103.4±14.6μg/g. 100.9±7.8μg/g. 91.1±19μg/g, 72.5±9.1μg/g. 103.2±12.2μg/g respectively. Each of these levels were significantly lower than the mean hair Zine levels of the control group (141.2±9.6μg/g. p<0.05 in each comparison). Although mean serum zine levels in all of the groups were also decreased, the differences were statistically significant only in the groups with B-precursor ALL. HL and NBNHL (75.9±5.29μg/dl. 68.6±7.3mUg/dl. 85.7±5.5μg/dl respectively) when compared with controls (105.1±9.9μg/dl, p<0.05 in each comparison). Hair magnesium and zine levels showed a positive correlation with each other in all the groups (r≅0.5). No significant difference was found in the mean hair/serum magnesium and Zine levels between malnourished and nonmalnourished patients. In conclusion, regarding the results of our study and previous daia in the literature chronic magnesium and zine deficiency seems to be associated with the development of ALL and malignant lymphoma in a group of patients.

Burkitt's Lymphoma in 63 Turkish Children Diagnosed Over a 10 Year Period

Sixty-three Turkish children with Burkitts lymphoma (BL) diagnosed over a 10-year period in a single institution were retrospectively analyzed. Burkitts lymphoma included 41.7% of non-Hodgkins lymphomas and 17.2% of all childhood malignant solid tumors diagnosed in our department in this duration. The patients studied with BL were aged between 3 and 14 years (mean 5.9 years), with a male of female ratio of 2:1. While the age distribution in our patients was similar to that in African BL (endemic), the predominance of abdominal involvement and the frequency of bone marrow infiltration and pleural effusion were reminiscent of American BL (sporadic). The incidence of jaw involvement (15.9%) in our group was higher than in American BL, however, and was not a high as in African BL. Most of the patients were of a lower socioeconomic status. Significant growth retardation was found in the children with BL compared with 40 age-matched children without malignancy, nor chronic or endocrinologic disorders, who were of a similar socioeconomic status. A serological study for Epstein-Barr virus (EBV) was performed in 18 children, and the IgG-type antibody to the viral capsid antigen of EBV was found to be positive in all of them. As a result, BL seems to include a considerable proportion of all childhood malignant solid tumors in Turkey. The epidemiological and clinical presentation and course indicate that BL appears in Turkish children in a form that is between the African and American types of the disease. Further molecular and chromosomal studies in Turkish children with BL are needed.

Invasive fungal infections in children with hematologic and malignant diseases.

Background: To evaluate the clinical feature and outcome of invasive fungal infections (IFI) in children with hematologic and malign diseases. Patients and Methods: The medical records of children with hematologic and malignant diseases, who were hospitalized at our hospital between January 2010 and December 2011, were reviewed. Proven, probable, and possible IFIs were diagnosed according to the revised definitions of the European Organization for Research and Treatment of Cancer/Mycosis Study Group. The demographic, clinical, and laboratory characteristics of the patients who met the study criteria were evaluated. Results: IFI was diagnosed in 67 (7.2%) febrile episodes of 56 patients, of which 10 (1.2%) were proven, 20 (2%) probable, and 37 (4%) possible IFI. Blood culture of 10 cases with proven IFI yielded yeast and the most common isolated agent was Candida parapsilosis. Seventy percent of cases with fungemia had central venous catheter (CVC). Twenty cases with probable IFI had invasive mold infection. The cases with mold infection had higher median C-reactive protein values, lower neutrophil counts, and longer duration of neutropenia compared with the cases with yeast infection. A total of 14 patients (20.9%) died. Presence of CVC, bone marrow transplantation, total parenteral nutrition, prolonged fever, and proven/probable IFI were detected more often in patients who died, compared with patients who survived. Conclusions: IFIs are important causes of death in children with hematologic and malignant diseases. Mold infections are seen more frequently in cases with prolonged and profound neutropenia, and invasive yeast infections, especially with non-albicans Candida species, in cases with CVC. Early and effective treatment considering these findings will help to decrease the mortality.