E-ISSN: 1308-5263
Turkish Journal of Hematology - Turk J Hematol: 26 (3)
Volume: 26  Issue: 3 - 2009
NEWS
1. Obituary Prof. Ernest Beutler (1928 - 2008)
Nejat Akar
Page 105
Abstract |Full Text PDF

REVIEW
2. Fertility preservation in females with malignant disease-1: causes, clinical needs and indications
Murat Sönmezer, Sinan Özkavukçu
Pages 106 - 113
Cancer incidence is progressively increasing in parallel with an increase in the rate of cancer survivors with the help of advanced treatment modalities. By the year 2010, it is estimated that one in every 250 persons will have survived a childhood malignancy. The increased rates of survival bring about complications related to reproductive health. Cytotoxic treatments due to chemo- and radiotherapy or bone marrow transplantation suppress or irreversibly harm not only female ovarian reserve but also male testicular sperm production. In this review, cryopreservation of gametes and gonads with fertility preservation options and indications prior to cancer treatments are discussed.

RESEARCH ARTICLE
3. Ciprofloxacin: a novel therapeutic agent for iron overload?
Mitra Elmi, Parvaneh Rahimi-moghaddam, Khosrou Abdi, Mehdi Shafiee-ardestani, Massoud Mahmoudian
Pages 114 - 117
OBJECTIVE: Major thalassemia is one of the hematological diseases requiring multiple blood transfusions, which results in iron overload in the liver, heart and other organs. Current iron chelation therapy consists of intravenous (IV) deferoxamine and oral deferasirox and deferiprone. Although these chelators are effective, many side effects are reported. In the present study, the iron-chelating effect of ciprofloxacin with good oral absorption was investigated.
METHODS: Thirty male albino Wistar rats were used for the study. Ciprofloxacin (7 or 14 mg/kg per day) was administered simultaneously with iron (0.03 g/kg per day) or after one-month administration of iron. Ciprofloxacin effect on iron absorption in the liver and heart was studied carefully using atomic absorption.
RESULTS: A significant decrease in the liver and heart iron following the ciprofloxacin (14 mg/kg per day) administration was observed, when compared with the control group. This ciprofloxacin-induced tissue iron depletion was more pronounced when it was administered simultaneously with iron, when it was administered for a longer duration (2 months rather than 1 month) and when it was given in higher doses (14 mg/kg per day).
CONCLUSION: Administration of ciprofloxacin may help to decrease the burden of parenteral administration, thereby improving compliance and also the life expectancy of thalassemic patients.

4. Acute myeloid leukemia in Turkish children with Fanconi anemia. One center experience in the period between 1964-1995
Sevgi Gözdaşoğlu, Mehmet Ertem, Zümrüt Uysal, Emel Babacan, Memnune Yüksel, Işık Bökesoy, Asuman Sunguroğlu, Ayten Arcasoy, Ayhan Çavdar
Pages 118 - 122
OBJECTIVE: Fanconi’s anemia(FA) is an autosomal recessive disorder characterized by a progressive pancytopenia,variable congenital abnormalities and an increased risk for the development of acute myeloid leukemia (AML). The objective of this study is to evaluate AML in the patients with FA diagnosed and followed-up in the Department of Pediatric Hematology at Ankara University School of Medicine in the period between 1964-1995.

METHODS: A total of 39 patients within the age range 2-14 years( mean 8.2±3.16),28 male and 11 female were diagnosed as FA on the basis of congenital abnormalities,pancytopenia, bone marrow hypoplasia and diepoxybutane induced chromosomal abnormalities that observed in all patients The hereditary and familial basis of FA was apparent in this series.Common abnormalities were growth retardation,cefe’- au- laitspots,hyperpigmentation,microcephaly, finger and thumb deformities,mental retardation and hypogenitalismus
RESULTS: Four AML (10.2%) were observed in our series.Cytogenetic analysis of these cases revealed 46/ XX,dup(3)(q22;q26) t(7;17) (p11;p11) in one where it was unsuccessful in three.Two cases could not achieve remission and died.The other two achieved complete remission and remained in remission for2 and 6 months.
CONCLUSION: Acute myelomonocytic type in three cases and acute monocytic type in one patient
were diagnosed in our series. The patients with FA should be followed with regard to AML and solid tumors. AML and solid tomors should be taken into the consideration as the first manifestation of FA.


5. Prevalence and etiological classification of thrombocytopenia among a group of pregnant women in Erbil City, Iraq
Rawand Pouls Shamoon, Nawsherwan Sadiq Muhammed, Muhammed Salih Jaff
Pages 123 - 128
OBJECTIVE: To determine prevalence and define causes of pregnancy associated thrombocytopenia
METHODS: A total of 850 pregnant women at different ages of gestation were screened for ‎thrombocytopenia. A control group of 150 age-matched nonpregnant women were tested for ‎platelet count. Neoborns of thrombocytopenic women were tested within 24 hours of ‎delivery and reassessment of women's platelets was done within 7-10 days post delivery. ‎
RESULTS: The mean platelet count in pregnant women was significantly lower than in nonpregnant ‎women (221±59.9 vs. 273±66.9). Thrombocytopenia affected 8% of cases, with peak ‎incidence during the third trimester. Gestational thrombocytopenia was found to be the ‎principal cause (73.8%); hypertensive disorders caused thrombocytopenia in 23% of cases ‎and two cases (4%) were due to immune thrombocytopenic purpura. No maternal or fetal ‎complications were noted. The mean platelet count of 51 neoborns of thrombocytopenic ‎women was (240 ± 7.1). Two neoborns (4%) had low platelet counts.‎
CONCLUSION: Majority of thrombocytopenias were mild gestational and occurred in late in pregnancy. No ‎maternal or neonatal bleeding complications were observed.‎

6. Beta globin gene cluster haplotypes of the beta thalassemia mutations observed in Denizli province of Turkey
Anzel Bahadır, Onur Öztürk, Ayfer Atalay, Erol Ömer Atalay
Pages 129 - 137
OBJECTIVE: Our aim is to identify the beta globin gene cluster haplotypes for the beta thalassemia mutations in Turkey at regional basis. Beta thalassemia mutations included in this study were IVS-I-110 (G>A), FSC 8/9 (+G), IVS-II-1 (G>A), IVS-I-5 (G>C), IVS-I-1 (G>A), IVS-I-6 (T>C) and FSC 8 (-AA).
METHODS: We studied 22 unrelated patients with β-thalassemia major and 72 unrelated healthy subjects from our Department’s DNA bank. Haplotype analysis was done by polymerase chain reaction (PCR)-based restriction enzyme digestion for the beta globin gene cluster of the following polymorphic restriction sites: Hinc II 5’ to ε, Hind III 5’ to Gγ, Hind III in the IVS-II 5’ to Aγ, Hinc II in pseudo β, Hinc II 3’ to pseudo β, Ava II in β, Hinf I 3’ to β. Associated haplotypes for the normal control samples (72 individuals, 144 chromosomes) were determined by Arlequin 3.1 software with unknown gametic phase.
RESULTS: According to the results obtained, the most frequent beta globin gene cluster haplotypes in the normal population are (+----++), (+----+-), (-+-++++), (+-----+) with the frequencies of 28.6 %, 17.2 %, 9.8 % and 8.3 % respectively. IVS-I-110 mutation is linked with the haplotypes (+----++) and (+-----+). Observed haplotypes are (+----++) for FSC 8/9 (+G), (-+-+++-) for IVS-II-1 (G>A), (-+-++-+ and -+-++++) for IVS-I-5 (G>C), (+----+- and +------) for IVS-I-1 (G>A), (-++---+) for IVS-I-6 (T>C) and (+-----+) for FSC 8 (-AA).
CONCLUSION: In conclusion, our region shows the Mediterranean character for the beta thalassemia mutations. According to the obtained results, IVS-I-110 (G>A) mutation linked with haplotype VII (+-----+), IVS-I-5 (G>C) mutation with haplotype IV (-+-++-+), codon 8/9 (+G) linked with haplotype I (+----++) were shown for the first time in Turkish population. The linkage of haplotype (+------) with the IVS-I-1 (G>A) mutation is reported for the first time in the published literature. In Denizli province of Turkey, beta globin gene cluster haplotypes of the normal population are strongly associated with the haplotypes of I (+----++), V (+----+-) and IX (-+-++++) respectively.

7. Better differential diagnosis of iron deficiencyanemia from beta-thalassemia trait
Fakher Rahim, Bijan Keikhaei
Pages 138 - 145
OBJECTIVE: Iron deficiency anemia (IDA) and ß- Thalassemia Trait (ß-TT) are the most common forms of microcytic anemia. This study was conducted to compare the validity of these discrimination indices in differentiating ß- Thalassemia Trait (β-TT) from IDA by calculating their sensitivity, specificity and Youden's index.
METHODS: Totally 323 subjects (173 children and 150 adult) with microcytic anemia were involved in this study. We calculated 10 discrimination indices in all patients with IDA and β-TT. We divided the patients into two different groups as younger and older than 10 years.
RESULTS: None of indices showed sensitivity and specificity of 100% in the patients older than 11 years, but only Shine and Lal Index showed sensitivity close to 90% and specificity of 100% in the patients younger than 10 years. The most accurate discriminative index for patients younger than 10 years was Shine & Lal and in case of patients older than 11 years it was RDWI. Youden's index for Shine & Lal & RBC Count and for RDWI & RBC Count indices has most diagnostic value for younger and older than 10 years, respectively.
CONCLUSION: None of the indices was completely sensitive and specific in differentiation between β-TT and IDA. MCHD mean and median were very close to normal values for both IDA and β-TT patients, but in case of MHDL we have found mean and median were significantly higher than normal values in β-TT and lower than those in IDA patients. In our study, Youden's index of RBC and S & L were the highest and the most reliable discrimination indices in differentiating β-TT from IDA in the patients younger than 10 years and for patients older than 11 years, the most reliable discrimination indices were RBC and RDWI.

CASE REPORT
8. Primary autoimmune myelofibrosis: a report of three cases and review of the literature
Rakhee Kar, Shyamali Dutta, Seema Tyagi
Pages 146 - 150
Myelofibrosis in association with autoimmune disorders has been consistently recognized in sporadic case reports over a number of years. Autoimmune myelofibrosis has been described most commonly in association with systemic lupus erythematosus (SLE). In addition, myelofibrosis presenting as cytopenias and showing clinical response to immunosuppressant drugs, notably steroids, has been reported with a wide range of immune-mediated disorders, including Sjögren’s syndrome, polyarteritis nodosa, rheumatoid arthritis, ulcerative colitis, and primary biliary cirrhosis. Attempts have been
made to define a syndrome of primary autoimmune myelofibrosis (PAIMF), as a distinct steroid-responsive clinicopathologic entity with excellent prognosis. Herein, we describe three cases of autoimmune myelofibrosis with a review of the literature.

9. Granulocytic sarcoma after stem cell transplantation in a child with biphenotypic leukemia
Zühre Kaya, Ülker Koçak, Meryem Albayrak, Türkiz Gürsel, Nalan Akyürek, Suna Özhan Oktar
Pages 151 - 153
Granulocytic sarcoma is an extramedullary tumor composed of leukemic blasts. Isolated granulocytic sarcoma has rarely been reported in children with leukemia undergoing allogeneic stem cell transplantation. We report a case of isolated granulocytic sarcoma arising from the pleura in an 11-year-old girl who was previously treated for biphenotypic leukemia with allogeneic stem cell transplantation. Complete resolution of the tumor was achieved after two inductions with MRC 12 protocol; however, she died of sepsis during the neutropenic period. The unusual presentation, immunophenotypic differences at diagnosis and relapse, and the management options are discussed.

10. Hypertrichosis: the possible side effect of cyclosporin in an infant with hemophagocytic lymphohistiocytosis receiving HLH-2004 chemotherapy protocol
Sinem Akgül, Yasemin Işıl Balcı, Şule Ünal, Ayfer Alikaşifoğlu, Aytemiz Gürgey
Pages 154 - 156
Hemophagocytic lymphohistiocytosis is a life-threatening condition of severe hyperinflammation that results from an uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. The immediate treatment strategies include immune suppressive therapy such as corticosteriod, etaposide and cyclosporin A. Herein, we present a 13- month-old infant, who developed severe hypertrichosis after the administration of HLH-2004 treatment protocol and discuss the various hypotheses regarding the causal relationship between cyclosporine A and hypertrichosis, emphasizing the importance of patient follow up.

IMAGES IN HEMATOLOGY
11. Severe bone marrow aplasia and Coombs-positive autoimmune hemolytic anemia in microfilariasis - coincidental or causal?
Upendra Mogalluru Srinivas
Page 157
Abstract |Full Text PDF

LETTER TO EDITOR
12. Depletion of innate immunity or bone marrow suppresion by viral infection ?
Şinasi Özsoylu, Şule Ünal
Page 158
Abstract |Full Text PDF

13. Usage of U7 snRNA in gene therapy of hemoglobin S disorder - is it feasible?
Viroj Wiwanitkit
Pages 159 - 160
OBJECTIVE: OBJECTIVE: Hemoglobin (Hb) S disorder [beta6(A3)Glu-->Val, GAG-->GTG] is an important hemoglobinopathy with the highest endemicity in Africa.
METHODS: METHODS: Here, the author performs a basic bioinformatics gene ontology study to assess the effect of co-expression between nucleic acid sequence for human Hb S beta globin chain and U7.623.
RESULTS: : According to this study, the analytical results show that fully recovery of hemoglobin in both function and biological process can be got.
CONCLUSION: CONCLUSION: Here, the author proposed that U7 snRNA might be a probable tool for treatment by gene therapy approach in Hb S disorder.