Sevgi Yetgin

Hacettepe University · Department of Pediatrics Hematology

Objective: To examine granulocytic and non-granulocytic cells in children with severe congenital neutropenia (SCN) and their non-neutropenic parents. Material and methods: Fifteen patients with SCN and 21 non-neutropenic parents were evaluated for a) CD95, CD95 ligand, annexin-V, propidium iodide, cell cycle and lymphocyte subsets, by flow cytom...

Myelodysplastic morphology of blood cells can be encountered not only in myelodysplastic syndrome (MDS) but also in nonclonal disorders like viral, bacterial, parasitic infections, juvenile rheumatoid arthritis, polyarteritis nodosa, immune thrombocytopenic purpura (ITP), iron deficiency anemia, megaloblastic anemia, dysgranulopoietic neutropenia,...

Objective: Transcobalamin II deficiency is one of the rare causes of inherited vitamin B12 disorders in which the patients have characteristically normal or high vitamin B12 levels related to the transport defect of vitamin B12 into the cell, ending up with intracellular cobalamin depletion and high homocysteine and methylmalonic acid levels. Mat...

Parvovirus-B19 (PV-B19) is a member of Parvoviridae, which is one of the smallest DNA viruses. PV-B19-associated diseases usually serve as a good representation of the balance of virus, host response and the immune system. The diseases manifested with PV-B19 are erythema infectiosum, which is common in children, hydrops fetalis, transient pure red...

Fludarabine, cytarabine, granulocyte colony-stimulating factor (G-CSF), and idarubicin (FLAG-IDA) regimen has been proven to be a potentially useful chemotherapy regimen for relapsed or poor-prognosis childhood leukemia. The aim of the study was to evaluate complete remission (CR) rate, toxicity, and overall survival of children with poor-prognosis...

SUMMARY: We present herein a 2-year-old boy who suffered from chronic recurrent multifocal osteomyelitis for 6 months and was later diagnosed as acute lymphoblastic leukemia. In view of the rarity of bilateral symmetric and multifocal lesions in osteomyelitis in children, we suggest that leukemia should be investigated with bone marrow aspiration i...

Seventy-seven patients with acute lymphoblastic leukemia (ALL) who were in complete remission and whose therapies had been stopped for at least 6 months before enrollment in this study were retrospectively analyzed regarding their antibody status for measles, mumps, and rubella, with the aim to demonstrate the seropositivity rate after treatment in...

In this retrospective study, we report the results of antifungal treatments (AFTs) in febrile neutropenic episodes in patients with acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML) and aplastic anemia (AA) in our center. From January 2004 to December 2005, a total of 52 patients and 221 febrile neutropenic episodes were evaluated. A...

In this pilot study, 30 (14 male, 16 female; median age: 8 years, range: 2-18) chronic non-splenectomized idiopathic thrombocytopenic purpura (ITP) patients with Rh+ blood group and their 49 attacks were evaluated after intravenous (i.v.) anti-D (WinRho SDF, Cangene Corporation, Winnipeg, MB, Canada) treatment at a dose of 50 microg/kg x 3 days (n...

Chemotherapy-induced acral erythema (CIAE) with bullous formation is an uncommon complication in children. We describe herein a child who developed CIAE with bullous formation following high-dose methotrexate and cytarabine for relapsed acute lymphoblastic leukemia. The rash completely resolved within two weeks of the appearance without any specifi...

Pseudotumor cerebri is a syndrome of increased intracranial pressure, normal cerebrospinal fluid values, and a normal cerebral ventricles on brain imaging studies. A patient with a diagnosis of pseudotumor cerebri was admitted to the authors' hospital twice within a 2.5-year interval and treated with vitamin B12 (vit-B12). At the second admission s...

Central nervous system (CNS) relapse in childhood acute lymphoblastic leukemia (ALL) has been overcome by sensitive therapeutic approachs. This study was planned to present the development of CNS relapse and survival in newly diagnosed 190 ALL patients whose cases were followed in the authors' unit between March 1991 and May 2002. St. Jude Study XI...

L-asparaginase is a crucial chemotherapeutic agent for the treatment of acute lymphoblastic leukemia. The alternatives to L-asparaginase are not available in many parts of the world, including Turkey. We sought to evaluate the utility of premedication with or without a desensitization protocol in children with acute lymphoblastic leukemia and syste...

The incidence of mixed-lineage leukemias in the pediatric age group was previously reported as 13.8% for myeloid antigen-positive ALL and 11.1% for lymphoid antigen-positive acute myeloid leukemia (AML). Recent studies showed that extensive chemotherapy protocols overcome the risk of myeloid lineage. Our study also supports most of the previous dat...

A high tumor burden at the time of diagnosis of childhood acute lymphoblastic leukemia has an unfavorable outcome. Peripheral white blood cell count is commonly used to reflect the leukemic burden and is used as one of the most important factors during determination of the risk-based treatment. However, peripheral blood blast count may not always r...

Peripheral blood smears of 43 patients (26 males, median age 18 months, range: 6-180 months) with nutritional iron-deficiency anemia (IDA) were examined for the presence of trilineage hematological dysplasia. Twelve patients were reexamined for dysplastic findings after achieving a normal Hb and hematocrit level for age by the end of 2-3 months of...

In this study, 32 patients under the age of 17 years with acquired aplastic anemia (AAA) were evaluated. Nine patients developed AAA associatedwith viral infection in which viral hepatitis and varicella infection were nearly equal. Four of the patients were administered drugs before developing AAA. Patients were treated as follows: combined immunos...

A prospective, randomized clinical trial was conducted to compare the efficacy of piperacillin/tazobactam and amikacin combination with carbapenem monotherapy for the empirical treatment of febrile neutropenic episodes of children with acute lymphoblastic leukemia or acute myeloblastic leukemia. Patients aged 2-16 years with hematological malignanc...

Chemotherapy-induced acral erythema is an uncommon and dramatic reaction to high-dose chemotherapy. It is characterized by painful erythema of both palms and soles with symmetrically well-defined borders, which may progress to bullae formation and desquamation. The bullous variant of this reaction has been reported with methotrexate and more freque...

Homozygous mutations in HAX1 cause an autosomal recessive form of severe congenital neutropenia (CN). By screening 88 patients with CN, we identified 6 additional patients with HAX1 mutations carrying 4 novel mutations. Of these, 2 affect both published transcript variants of HAX1; the other 2 mutations affect only transcript variant 1. Analysis of...

Leukemia is one of the leading journals in hematology and oncology. It is published monthly and covers all aspects of the research and treatment of leukemia and allied diseases. Studies of normal hemopoiesis are covered because of their comparative relevance.

A girl with congenital dysgranulopoietic neutropenia (CDN) and her non-neutropenic mother with aphthae (A) were investigated. Apoptosis in lymphocytes and granulocytes of both patients (mother A+) were documented by high annexin and electron microscopic morphology. CD11b/CD18 of the daughter's granulocytes ranged between low to normal while that of...

In pediatric acute immune thrombocytopenic purpura (ITP) cases, it is usually possible to determine an underlying pathology; among them, viral infections are the leading causative agents. In this report, we describe two cases of acute ITP complicated secondary to parvovirus B19 or Epstein-Barr virus (EBV) infections who were unresponsive to initial...

We investigated a 15-year-old female with congenital dysgranulopoietic neutropenia (CDN) and her non-neutropenic mother who had recurrent stomatitis. In both patients, cells of the neutrophilic, eosinophilic, monocytic, megakaryocytic, and basophilic series were dysmorphic. Plasmacytoid lymphocytes and mild megaloblastic erythroid precursors were p...

The data of allogeneic HSCT in nine children with acquired AA between June 1998 and July 2006 were analyzed retrospectively. The median duration of time to neutrophil and platelet engraftment was 18 and 25 days, respectively. None of the patients had primary graft failure. Two (22.2%) patients developed acute GVHD and of these, one (11.1%) was Grad...

Varicella zoster virus is very rarely associated with aplastic anemia. Bone marrow transplantation from an HLA-identical sibling is the treatment of choice. A seven-year-old boy presented with aplastic anemia (AA) following chicken pox infection. No clinical improvement was observed with pharmaceutical therapy and peripheral blood stem cell transpl...

Here, a case is presented with two rare genetic disorders, biotinidase deficiency and juvenile myelomonocytic leukemia, in a Turkish infant. This case may serve as a reminder that the diagnosis of a genetic disorder does not exclude the possibility of a second congenital but acquired disease.

Aim: The aim of the study was to evaluate the rate of hepatitis B and C virus infection and emphasize the importance of hepatitis B virus (HBV) vaccination in leukemic children. Methods: One hundred and sixty children who were treated for acute lymphoblastic leukemia (ALL) at Hacettepe University Faculty of Medicine, Pediatric Hematology Unit were...

Glucocorticoids (GCs) are known for their clinically useful effects in immunologic and inflammatory disorders. Although there is a huge volume of knowledge concerning the cellular and molecular effects of GCs, statements regarding their effects in multiple diseases at variable doses are not clear-cut owing to pharmacogenetic differences. The main a...

Nonimmune hydrops fetalis may occur as a result of different etiological conditions and in about one-third of cases no cause could be identified. Here, we report two cases of nonimmune hydrops fetalis associated with hereditary spherocytosis and hemophagocytic hystiocytosis. We think that babies with hydrops fetalis born of consanguineous parents s...

The protective power of two booster dose vaccination against hepatitis B virus (HBV) infection has not been previously studied in patients with acute lymphoblastic leukemia (ALL) who remained unresponsive to immunization. The aim of this study was to determine the HBV infection rate in vaccinated and unvaccinated patients with or without seroconver...

The diagnosis of acute leukemia is based on a combination of clinical, hematological, morphological, cytogenetic, and immunophenotypic data. The authors report a case of reactive lymphocytosis with extremely elevated lymphocytic and lymphoblastic leukocytosis that mimicked acute lymphoblastic leukemia, not only morphologically, but also in immunoph...

PALB2 was recently identified as a nuclear binding partner of BRCA2. Biallelic BRCA2 mutations cause Fanconi anemia subtype FA-D1 and predispose to childhood malignancies. We identified pathogenic mutations in PALB2 (also known as FANCN) in seven families affected with Fanconi anemia and cancer in early childhood, demonstrating that biallelic PALB2...

In clinical practice, when neutropenic-fever patients present with no microbiologically and clinically defined infection, the risk of underestimating an occult infection is of major concern, the clinicians have to make a decision on when to modify antibiotic therapy. Hence, a reliable, specific, and sensitive marker, which is regulated independentl...

Out of 334 children with acute lymphoblastic leukemia who were treated with St Jude Total XI and Total XIII chemotherapy protocols were investigated and 21 (6.3%) were hypertensive. The incidence of tumor lysis syndrome was higher in the hypertensive group than in the nonhypertensive group (28.6% vs. 11.5%) (P = 0.035). There were no differences be...

Hypercalcemia is a rare complication of malignancy in children. We present two children, one of whom is the youngest reported, with CALLA+ B-cell acute lymphoblastic leukemia (ALL) who developed hypercalcemia at the time of diagnosis and were treated with relatively low-dose (0.5 mg/kg) intravenous pamidronate successive to conventional therapy. No...

We reviewed the pattern of acute and long-term (during and after treatment period) neurologic complications in children with acute lymphoblastic leukemia (ALL). Among 265 patients, 40 developed neurotoxicity. Twenty-one and 19 of the patients were treated with St. Jude Total XI and St. Jude Total XIII chemotherapy protocols, respectively. There was...

Leukemia constitutes 25–30% of all pediatric malignancy cases. The epidemiologic and demographic characteristics of this group of patients are important not only for determination of the prognostic factors, but also the risk factors. In this study, 683 patients under 16 years of age who were diagnosed with acute lymphoblastic leukemia (ALL) and acu...

During recent decades, the survival rate after childhood acute lymphoblastic leukemia (ALL) has improved substantially; consequently, the long-term side effects of ALL and its treatment have gained attention, of which osteoporosis is one of the most important. The purpose of the present study was to compare the influence of different treatment prot...

Autoimmune thrombocytopenic purpura is estimated to be one of the most common acquired bleeding disorders of children. The pathogenesis involves the generation of autoantibodies against the normally expressed glycoproteins on the platelet membranes. These antibody-coated platelets in turn are destroyed by the spleen and other reticuloendothelial or...

Immune thrombocytopenia (ITP) is a frequently encountered disease in childhood. Recent reports pointed to the benefit of high-dose steroid in ITP treatment since it resulted in a better outcome in a shorter time than IV immunoglobulin therapy. In the authors' clinic, mainly after 1984, megadose methyl prednisolone (MDMP) has been used for ITP treat...

Hashimoto's thyroiditis is the most frequent autoimmune thyroid disease. L-thyroxine therapy can reduce the incidence and alleviate the symptoms of this disease. The aim of this study was to evaluate the effects of prophylactic L-thyroxine treatment on clinical and laboratory findings of patients who were euthyroid at the time of diagnosis. Thirty-...

The objective of this study was to determine the frequency of electrolyte perturbations and their relationship with leukemic status before and after chemotherapy in patients with acute lymphocytic leukemia. Blood biochemistry, liver and renal function tests, and renal sonograms were examined at diagnosis and during induction therapy in 334 patients...

Parvovirus B19 is the causative agent of various forms of hematologic diseases such as aplastic crisis in patients with hemolytic anemia, aplastic anemia, hypoplastic anemia, and idiopathic thrombocytopenic purpura. In addition, parvovirus B19 infection may precede or be associated with acute lymphoblastic leukemia (ALL). The authors present two ca...

In order to evaluate potential long-term kidney damage of childhood leukemia and risk factors affecting renal damage, we studied 116 children treated for acute lymphoblastic leukemia (ALL) using the St. Jude Total XI and XIII protocols in 1991-1998. The median follow-up period after the completion of treatment was 35 months. The following parameter...

Although human parvovirus B19 (PVB19) is mainly known to be the causative agent of fifth disease, it is also important in the etiopathogenesis of various hematologic disorders. Recently, its role in the occurrence of chronic anemia and pancytopenia in immunocompromised patients has been revealed. In this study, the authors searched for the presence...

The authors treated a girl with severe aplastic anemia that developed following urinary tract infection. She was referred to our hospital for bone marrow transplantation. The detailed medical history of the patient revealed iatrogenic metamizole sodium overdose that was given during her infection. She responded to a 3-day, high-dose methylprednisol...

Between January 1978 and August 1999, 29 infants with newly diagnosed acute lymphoblastic leukemia (ALL) were treated on three consecutive protocols. Eighteen patients with infant ALL diagnosed between 1978-1991 were included in Group 1. In this group, treatment comprised a two- or three-drug induction with prednisolone, vincristine and L-asparagin...

Vitamin D metabolites have multiple functions not only in calcium homeostasis, but also in hematopoiesis. To detect the effect of vitamin D on hematopoiesis with a surface glycoprotein marker, the proportions of the CD34+ cells were measured in bone marrow, peripheral blood and spleen prior to and after vitamin D3 treatment in an infant with severe...

Human parvovirus B19 (PVB19) infection may cause mild pancytopenia characterized by transient and spontaneous recovery in healthy subjects. Severe aplastic anemia associated with PVB19 infection in patients without an underlying disease has been described in a number of reports. Here, a previous healthy, 10-year-old girl with severe aplastic anemia...

This study was initiated to evaluate the status of selenium in Turkish residents. Serum selenium level of 76 healthy children, living in Ankara, aged 2 mo-13 y was determined by a spectrofluorometric method. Average selenium level was found to be 88.1±12.4 μg/L (mean±SD). Selenium levels showed a tendency to increase with age and mean selenium lev...

Second malignant neoplasm in childhood is increasing due to advances in therapy modalities. Acute lymphoblastic leukemia as a second malignancy following the treatment of medulloblastoma is a very rare condition. A 13-year-old boy was diagnosed as acute lymphoblastic leukemia following radiotherapy and chemotherapy for treatment of medulloblastoma.

Diagnosis of diaphragmatic injury is difficult. A case of iatrogenic diaphragmatic rupture is reported in which perforation of a herniated stomach occurred following left lobectomy and partial resection of the diaphragm for lung cancer. On the second postoperative day, bile-stained fluid coming out from the chest tube revealed gastrointestinal leak...

Nineteen children with hemophagocytic lymphohistiocytosis (HLH) were studied in the Department of Pediatric Hematology, Hacettepe University. Patients were divided into two groups. Group 1: Thirteen patients were classified as having a genetic etiology (7 familial, 6 presumed familial) on the basis of an affected sibling and consanguinity. There wa...

We examined the effect of high-dose methylprednisolone (HDMP) on blast reduction rate and compared it to conventional dose steroid treatment, administered during the first 7+ days of the induction remission period in patients with acute lymphoblastic leukemia (ALL). In our previous randomized study, the event free survival (EFS) was found to be hig...

Eight-year event-free survival (EFS) was evaluated in 205 patients with acute lymphoblastic leukemia (ALL), to consider the efficacy of high-dose methylprednisolone (HDMP) given during remission induction chemotherapy between 1 and 29 days. The St Jude Total XI Study protocol was used after some minor modifications in this trial. Patients were rand...

Rapid leukemic cell kill at initial diagnosis of patients with acute lymphoblastic leukemia (ALL) has been shown to be associated with a favorable outcome. The aim of the present study was to investigate the effect of high dose methylprednisolone (HDMP) on in vivo blast cell apoptosis in children with ALL. Annexin V-binding and Fas (CD95), Fas liga...

Minimal residual disease (MRD) was investigated in 52 children with acute lymphoblastic leukaemia (ALL), using antigen receptor gene rearrangements and reverse transcription polymerase chain reaction for fusion transcripts as molecular targets. Patients [treated according to the Medical Research Council United Kingdom ALL (MRC UKALL) XI protocol or...

Angiogenesis is a complex biologic process that occurs normally in development and in turnover and remodeling of mature vascular networks. Pathological angiogenesis and neovascularization occur in association with tumor growth and metastasis, developmental disorders, and ischemic diseases. The role of angiogenesis in hematological diseases remains...

We observed less severe symptoms in patients with chronic idiopathic thrombocytopenic purpura (ITP) than in patients with acute ITP with similar platelet counts. Thrombopoietin (TPO), soluble P-selectin, soluble P-selectin per platelet, and interleukin 6 (IL-6) were evaluated in children with ITP before treatment in 16 acute and 22 chronic cases an...

Idiopathic thrombocytopenic purpura (ITP) in children is usually a self-limiting disorder. It may follow a viral infection or immunization and is caused by an inappropriate response of the immune system. Many viruses, such as human immunodeficiency virus, cytomegalovirus (CMV), Epstein-Barr virus (EBV), varicella, rubeola, mumps, and parvovirus, ha...

Leukemic skin infiltration is quite uncommon in certain types of leukemia. Here, a child with hairy cell leukemia who developed isolated skin infiltration during remission is reported. The failure to diagnose the leukemic infiltration until the nodule reached a diameter of 2 cm is emphasized.

The aim of this study was to interpret the antibody response to hepatitis B (HB) vaccination following a two booster dose schedule in 94 acute lymphoblastic leukemia (ALL) patients. All patients were between 1-16 years of age with negative hepatitis B virus (HBV) serology and normal hepatic function. Fifty patients were vaccinated with Engerix B va...

The term emperipolesis defines the temporary presence of one cell within another's cytoplasm. In clinical use, megakaryocyte emperipolesis is the penetration of hematopoietic cells into the cytoplasm of megakaryocytes. The pathophysiological significance of megakaryocyte emperipolesis is uncertain. It has been described in association with neoplast...

Acute tumour lysis syndrome (ATLS) is a well recognised complication of treatment of a variety of malignant disorders. It commonly occurs in patients with non-Hodgkin's lymphoma (NHL) and acute lymphoblastic leukaemia (ALL) with the administration of combined cytotoxic chemotherapy. It is rarely reported after single-agent corticosteroid therapy. W...

The aim of this study is to evaluate, for the first time serum levels of vascular endothelial growth factor (s-VEGF), and basic fibroblast growth factor (s-b FGF) in children with acute lymphoblastic leukemia (ALL), and its relation to clinical manifestations of the disease. Although VEGF and b FGF have been suggested to be reliable prognostic indi...

A girl with chronic idiopathic neutropenia who developed gingival enlargement at seven years of age is presented. Intraoral examination revealed generalized gingival inflammation with a tendency to bleeding and inflammatory gingival enlargement localized to the anterior region. A considerable amount of bacterial plaque was noted on the teeth. There...

An 11-month-old patient with parvovirus infection mimicking juvenile myelomonocytic leukemia (JMML) is presented. The patient's history, presenting physical and laboratory features, was suggestive of JMML and consisted of fever, hepatosplenomegaly, lymphadenopathy, desquamation of the skin, anemia, leukocytosis with monocytosis and trilineage dyspl...

This study evaluates the dysplastic hematological changes in nine patients with idiopathic thrombocytopenic purpura (ITP) in 11 attacks, before and after corticosteroid treatment. The pretreatment blood smears of patients with ITP, displayed more neutrophils with bizarre nuclei (P < 0.001), Döhle or Döhle-like inclusions (P < 0. 01), irregular dist...

The presented case is a boy with T-cell acute lymphoblastic leukemia (ALL) with hairy cell (HC) features and monoclonal gammopathy. The disease process had an acute onset and followed a rapid, progressive course. The patient had minimal splenomegaly and bicytopenia, but the bone marrow displayed increased numbers of reticulin fibers. The blasts wer...

The presented case is a boy with T-cell acute lymphoblastic leukemia (ALL) with hairy cell (HC) features and monoclonal gammopathy. The disease process had an acute onset and followed a rapid, progressive course. The patient had minimal splenomegaly and bicytopenia, but the bone marrow displayed increased numbers of reticulin fibers. The blasts wer...

Wilson disease is an autosomal recessively inherited disease of copper metabolism and is characterized by liver and central nervous system dysfunction. The heterozygote carrier state rate is about one in 90 persons and the incidence of the disease is about 30 in 1,000,000. Although leukemia is the most common form of childhood malignancies, the pro...

CD117 protein is expressed by the primitive CD34 positive haemopoietic stem cells and also demonstrated on the blasts of 30-100% of AML cases, but rarely on lymphoblasts. Therefore several investigators have used CD117 expression to exclude lymphoblastic origin of blasts. However, conflicting results exist in the literature. We investigated CD34 an...

Several agents, used either alone or in combination, have been shown to boost absolute numbers of PMLs and CD34+ stem cells in PB. In this study, we compared the effects of three different treatments, G-CSF, HDMP, and G-CSF + HDMP, for neutropenic patients (absolute PML count < 0.5 x 10(9)/l) who were on maintenance therapy for ALL with a control g...

In this paper, research based on 51 children with a positive antiglobulin test is presented. Eighteen of the children had acute anemia and 33 had chronic anemia. Two clinical patterns were distinguished: an acute transient type and a prolonged chronic type. Corticosteroid therapy was effective in all acute cases but its results were variable in the...

To demonstrate the importance of bone marrow iron stores, we examined the complete hemogram, serum iron (SI), serum iron-binding capacity (SIBC), transferrin saturation (TS), serum ferritin and bone-marrow-stored iron in 31 children with iron deficiency (ID). The ages of the patients ranged from one to 14 years (mean 3.7 +/- 3.9). Laboratory findin...

Three patients with acute lymphoblastic leukemia (ALL) developed mononuclear cells in the cerebrospinal fluid (CSF) after a flu-like history during maintenance treatment. None of the patients showed evidence of central nervous system (CNS) involvement by either clinical or laboratory follow-up. Although the presence of > 5 mononuclear cells/microl...