E-ISSN: 1308-5263
Turkish Journal of Hematology - Turk J Hematol: 25 (4)
Volume: 25  Issue: 4 - 2008
REVIEW
1. Therapeutic apheresis in hematopoietic cell transplantation
Nina Worel, Gerda Leitner, Andrea Wagner, Hildegard T Greinix, Paul Höcker, Peter Kalhs
Pages 164 - 171
With the introduction of hematopoietic stem cell transplant (HSCT), transplant-associated problems like graft-versus-host disease (GVHD) and transplant-associated microangiopathy (TAM) have occurred. In addition, approximately 40% of allogeneic HSCTs are performed across the ABO blood group barrier, bearing the risk for immunohematological complications like severe hemolysis and pure red cell aplasia (PRCA). All these problems can potentially require therapeutic apheresis. In this review, we address recent developments in therapeutic apheresis for patients undergoing allogeneic HSCT for prevention or treatment of hemolysis in minor ABO-incompatible transplantation, treatment of PRCA after major ABO-incompatible transplantation, and treatment of TAM and GVHD.

RESEARCH ARTICLE
2. Optimization of transfection of green fluorescent protein in pursuing mesenchymal stem cells in vivo
Yusuf Baran, Ali Uğur Ural, Ferit Avcu, Meral Sarper, Pınar Elçi, Aysel Pekel
Pages 172 - 175
OBJECTIVE: Green Fluorescent Protein (GFP) has been used as a marker of gene expression and a single cell marker in living organisms in cell biology studies. The important areas that GFP is used are expression levels of different genes in different organisms by inserting GFP in these genes and as a marker in living cells. In this study, we tried to optimize transfection of mesenchymal stem cells, (MSCs) used for regeneration of damaged tissues in animals, by GFP containing plasmid vector by which MSCs can be followed in vivo.
METHODS: To this aim, phM-GFP plasmid vector carrying GFP gene and effectene transfection reagent were used.
RESULTS: The data revealed that twice transfection of MSCs resulted in higher expression of GFP for longer times as compared to once transfected MSCs. On the other hand, leaving the chemical transfection agents in the medium induced apoptosis after a while.
CONCLUSION: As a conclusion we suggest the transfection of MSCs twice with 48 hours interval and removal of transfection agents after 8 hours which removed toxic and apoptotic effects of the chemicals.

3. Seroprevalence of hepatitis and human immuno-deficiency virus in multitransfused patients from a pediatric hematology clinic
Nazan Sarper, Emine Zengin, Birsen Mutlu, Suar Çakı Kılıç
Pages 176 - 180
OBJECTIVE: Transfusion transmitted hepatitis has been a severe problem in Turkey in pediatric cancer patients and in chronic congenital anemia. The aim of the present study was to investigate the prevalence of hepatitis B, hepatitis C and human immunodeficiency virus infections in these patients in a University Hospital.
METHODS: Multi-transfused 66 children (59 acute leukemia, 6 thalassemia major, 1 severe hereditary spherocytosis) diagnosed and followed-up between May, 2000 and December, 2006 were evaluated. Screening of all the patients for HbsAg, anti-HBs, anti-HBc, anti-HCV and anti-HIV was performed at presentation and during the last follow-up. Serologic studies of leukemic patients were also repeated at the end of the chemotherapy. Hepatitis B vaccination was administered to unvaccinated patients with anemia. All blood products were provided by Blood Bank of the Center.
RESULTS: No patient was found HBsAg, anti-HCV or anti-HIV positive at diagnosis and at the end of the therapy. There was history of hepatitis B vaccination in only 42% of the patients at diagnosis due to administration of this vaccine to newborns since 1998. At the beginning of the study, 45 % (n=27) of the leukemic patients were immune for hepatitis B, but after completion of the intensive chemotherapy seropositivity persisted in only 28.8 % (n=17).
CONCLUSION: Transmission of these viruses is no longer a real problem even in multitransfused immunosuppressed children in Pediatric Hematology Units as a result of the improvements in screening of voluntary blood donors, administration of disposable material in clinics and vaccination by hepatitis B.

4. Some hematological parameters and the prognostic values of CD4, CD8 and total lymphocyte counts and CD4/CD8 cell count ratio in healthy HIV sero-negative, healthy HIV sero-positive and AIDS subjects in Port Harcourt, Nigeria
Victor Dapper, Pedro Emem-Chioma, Blessing Didia
Pages 181 - 186
OBJECTIVE: The present study attempts to determine normal values of CD4, CD8, CD4/CD8 ratio, total WBC and differential counts, hematocrit and total lymphocyte count (TLC) in healthy HIV sero-negative and sero-positive subjects, and to assess the prognostic significance of these parameters in these subjects as compared to AIDS subjects.
METHODS: A total of 300 subjects (147 M, 153 F) aged between 17 and 71 years were recruited into the study. Subjects were separated according to sex and divided into three groups: Group A: healthy HIV sero-negative subjects; Group B: healthy HIV sero-positive newly diagnosed ART-naïve subjects; and Group C: AIDS subjects. CD4 and CD8 counts were determined by flow cytometry; hematocrit was determined using Hawksley micro-capillary tubes; total WBC and differential counts were determined manually with the improved Neubauer counting chamber; and TLC was obtained by multiplying the percentage of lymphocytes by the total WBC count.
RESULTS: For male subjects, significant differences were found in CD4 count, CD4/CD8 count ratio, hematocrit, total WBC and TLC, whereas for female subjects, significant differences were found only in CD4 and CD4/CD8 count ratio in the three groups of subjects. In both sexes, however, these parameters were found to be highest in healthy HIV sero-negative subjects and lowest in AIDS subjects, with HIV sero-positive subjects having intermediate values.
CONCLUSION: The results confirm previous reports that the CD4 count and CD4/CD8 count ratio are fairly reliable indicators of the progression of HIV infection. In addition, the results also apparently suggest that the prognostic value of CD8 count is limited and that of TLC possibly sex-dependent. The results could be of importance in our environment since previous reports have been relatively scarce.

5. The levels of nitric oxide in beta-thalassemia minor
Nihayet Bayraktar, Mehmet Ali Erkurt, İsmet Aydoğdu, Yalçın Başaran
Pages 187 - 189
OBJECTIVE: The aim of this study was to investigate the relationship between NO (nitric oxide) and beta-thalassemia minor.
METHODS: A total of 60 patients with beta-thalassemia minor (30 M, 30 F) were included in the study. The control group consisted of 60 healthy subjects (30 M, 30 F). Plasma nitrite/nitrate levels were measured using the Griess reaction method and analyzed by spectrophotometry at 545 nm.
RESULTS: Plasma direct nitrite, total nitrite and nitrate levels were 7.561±6.19, 42.548±7.37 and 34.84±6.24 in beta-thalassemia minor patients versus 36.9±19.8, 85.9±35.3 and 48.61±17.35 Ìmol/dl in controls, respectively. Plasma direct nitrite, total nitrite and nitrate levels were significantly lower in beta-thalassemia minor patients compared with the control group (p<0.001).
CONCLUSION: These findings confirm that plasma NO levels in beta-thalassemia minor patients are decreased at the time of diagnosis. This may be helpful in assessing the prognosis and follow-up evaluation of patients with beta-thalassemia minor.

6. Significance of molecular-cytogenetic aberrations for the achievement of first remission in de novo acute myeloid leukemia
Milena G. Velizarova, Evgueniy A. Hadjiev, Kamelia V. Alexandrova, Dora N. Popova, Ivanka Dimova, Boriana M. Zaharieva, Draga Toncheva
Pages 190 - 194
OBJECTIVE: The majority of adults diagnosed with acute myeloid leukemia (AML) display acquired cytogenetic aberrations at presentation. In this article, we present the major cytogenetic findings regarding AML and review their clinical significance for achievement of the first complete remission.
METHODS: We studied 71 adult patients with de novo AML, without previous myelodysplasia or alkylating therapy. Conventional cytogenetics and FISH were performed on bone marrow cells. The patients with AML were assigned to 12 subgroups according to established data for cytogenetic, molecular and general laboratory results. The selection of the analyzed parameters is consistent with internationally accepted “prognostic factors” in adult AML.
RESULTS: Complete remission upon induction therapy was achieved in 40% of cases (in a mean period of 2.3 months from therapy initiation). The patients with t(15;17) PML-RARA and inv(16)/CBFbeta-MYH11ë demonstrated the highest frequency of complete remission. Patients with hypodiploidy, t(9;22)/bcr-abl and complex karyotypes were therapy-resistant or died within the first three months after AML diagnosis.
CONCLUSION: Molecular-cytogenetic findings have an important significance for achievement of first complete remission. However, laboratory and biologic features (age, WBC and LDH) and type of AML have a large influence on the disease outcome.

CASE REPORT
7. Scleredema-associated IgA myeloma with myelofibrosis in a young adult: a case report
Seema Rao, Rakhee Kar, Hara Prasad Pati, Renu Saxena
Pages 195 - 197
Scleredema of Buschke is a rare fibromucinous connective tissue disorder of unknown etiology. It is often associated with a benign monoclonal gammopathy and rarely with myelomatosis. We report a case of scleredema-associated IgA myeloma with myelofibrosis in a 24-year-old male patient. Scleredema generally affects young adults and onset of associated monoclonal gammopathy is at a younger age than when not associated with scleredema. However, presentation at a much younger age (24 years in our case) is very unusual. Although mucin deposition in the bone marrow has been reported in scleredema, to the best of our knowledge, myelofibrosis has not been reported.

8. Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case report
Cengiz Demir, Ali Bay, Imdat Dilek, Ahmet Faik Öner
Pages 198 - 200
We present herein a 23-year-old man with acute myeloblastic leukemia (AML) associated with Davidoff-Dyke-Masson syndrome (DDMS) and Marfan syndrome (MS). The diagnosis of DDMS was based on findings including left facial asymmetry, left hemiparesis, mental retardation, right cerebral hemiatrophy, dilatation of the ipsilateral lateral ventricle and calvarial thickening. The diagnosis of MS was based on clinical findings including tall stature, myopia, retinitis pigmentosa, blue scleras, scoliosis, pectus excavatum, arachnodactyly and low ratio of upper/lower body segment. The patient developed hepatosplenomegaly, gingival hypertrophy and pancytopenia. Peripheral blood film and bone marrow examination showed that most of nucleated cells were blasts; immunophenotype of those cells showed CD11+, CD13+, CD14+, CD33+ and HLA-DR+. These findings confirmed the diagnosis of AML (FAB-M5). After induction chemotherapy, remission was obtained. To the best of our knowledge, our case is the third report of AML in MS syndrome, while AML associated with DDMS and MS has not been previously reported in the literature.

9. Gum hypertrophy - an unusual presenting feature in a case of precursor T-cell acute lymphoblastic leukemia
Chennagiri Srinivasamurthy Premalata, Davanam Satyanarayana Madhumati, Vishweshwariah Lakshmidevi, Rudramurthy Pradeep, Lingappa Appaji, Geetashree Mukherjee
Pages 201 - 204
Acute lymphoblastic leukemia/lymphoma, the malignant transformation of T-cell or B-cell precursors, is the most common diagnosis in pediatric oncology. Precursor T-cell acute lymphoblastic leukemia/lymphoma commonly affects adolescents, and is associated with mediastinal mass in over half of the cases, with early dissemination to bone marrow, gonads and the central nervous system. We present a rare case of precursor T-cell acute lymphoblastic leukemia/lymphoma with initial oral manifestation, presenting with the unusual features of gum hypertrophy and involvement of upper jaw and palate in a 10-year-old boy. This report discusses the clinical presentation, histopathologic and immunologic features, and diagnosis of this malignancy.

LETTER TO EDITOR
10. The use of high-dose acyclovir in patients with hematological malignancies who develop herpes virus infections: Is it really safe?
Ismail Sarı, Leylagül Kaynar, İsmail Koçyiğit, Fevzi Altuntas
Pages 205 - 206
Abstract |Full Text PDF

11. Failure of yttrium-90 (90Y)-ibritumomab tiuxetan radioimmunotherapy (Zevalin®) with fatal side effects in relapsed/refractory diffuse large B-cell NHL transformed from other lymphomas
Songül Şerefhanoğlu, Ebru Koca, Hakan Goker, İbrahim Celalettin Haznedaroğlu, Deniz Çetiner, Nilgün Sayınalp, Yahya Büyükaşık, Osman İlhami Özcebe
Pages 207 - 208
Abstract |Full Text PDF

12. Allogeneic stem cell transplant in a patient with aplastic anemia with bacteremia and candidemia
Sharat Damodar
Pages 209 - 210
Abstract |Full Text PDF

13. Insulin-like growth factor-1 and zinc
Nejat Akar
Page 211
Abstract |Full Text PDF

IMAGES IN HEMATOLOGY
14. A rare presentation of multiple myeloma: Tc-99m MIBI SPECT imaging of orbital involvement
İlknur Ak Sivrikoz, Zafer Gülbaş
Pages 212 - 213
Abstract |Full Text PDF