ISSN: 1300-7777 E-ISSN: 1308-5263
Turk J Hematol: 25 (1)
Volume: 25  Issue: 1 - 2008
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REVIEW
1.Red cell glucose-6-phosphate dehydrogenase deficiency in Turkey
Çiğdem Altay, Fatma Gümrük
Pages 1 - 7 (3037 accesses)
Eritrosit glukoz-6-fosfat dehidrogenaz (G6PD) enzim eksikliği dünyada en sık görülen eritrosit enzim eksikliğidir. Eritrosit glukoz-6-fosfat dehidrogenaz enzim eksikliğinin epidemiyolojisi, biyokimyasal ve molekuler analizleri ile ilgili dünyada ve ülkemizde son 50 yılda yapılan çalışmalar özetlenmiştir.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common erythrocyte enzyme deficiency in the world. The epidemiological, biochemical and molecular studies on G6PD enzyme deficiency performed over the past 50 years are summarized herein, with special emphasis on the findings of studies related to the enzyme deficiency in Turkey.

RESEARCH ARTICLE
2.Malaria parasitemia and antimalaria prophylaxis in sickle cell anemia patients in steady staten
Omolade Augustina Awodu, Victoria Ade Wagbatsoma, Mathew Ebose Enosolease
Pages 8 - 12 (7920 accesses)
Orak hücre anemisi olduğu bilinene 37 hasta, Edo eyaleti Benin şehrinde Benin Üniversitesi Eğitim Hastanesi rutin hematoloji kliniğine başvurdukarında malarya parazitemisi açısından incelendi. Parazitemi kantitatif “buffy coat” incelemesi ile belirlendi. İncelenen hasta grubunda malarya parazitemi sıklığı %86.5 olarak bulundu. Erkek ve kadın orak hücre anemisi hastaları arasında parazitemi sıklığ açısından anlamlı bir fark yoktu (p=0.35). Malarya parazitemisi hematokrit değerinin 0.20’den daha az olması ile anlamlı olarak ilişkiliydi (p=0.03). Malarya profilaksi tipleri ile parazitemi arasında istatiksel olarak anlamlı bir farklılık yoktu. Sonuç olarak, paraziteminin önlenmesinde malarya profilaksisinin rolü önemsiz gözükmektedir. Bu nedenle, asıl olarak ilaçlı sivrisinek ağlarının kullanılması gibi diğer koruyucu önlemlere ağırlık verilmesi önerilmektedir.
Malaria parasitemia was assessed in 37 known sickle cell anemia patients attending the routine hematological clinic of the University of Benin Teaching Hospital, Benin-City, Edo State. Parasitemia was determined using the quantitative buffy coat analysis. The prevalence of malaria parasitemia among the population studied was 86.5%. There was no significant difference in the prevalence of parasitemia among the male and female sicklers (p=0.35). Malaria parasitemia was significantly associated with hematocrit <0.20 (p=0.03). There was no statistically significant difference between the type of prophylaxis used and parasitemia. In conclusion, the role of malaria prophylaxis in preventing parasitemia seems negligible. It is therefore recommended that more emphasis.

3.Hematological profile of HIV patients in relation to immune status - a hospital-based cohort from Varanasi, North India
Suresh Venkata Satya Attili, V. P. Singh, Madhukar Rai, Datla Vivekananda Varma, A. K. Gulati, Shyam Sundar
Pages 13 - 19 (5711 accesses)
OBJECTIVES: To study the spectrum of hematological manifestations and evaluate the relationship between various hematological manifestations and CD4 cell counts in a hospital-based cohort of HIV-infected adults in and around Varanasi, North India.
MATERIALS-METHODS: The clinical and hematological profiles of the patients attending the Infectious Disease Clinic, Varanasi, India were recorded. The relationship between CD4 counts and various hematological manifestations was analyzed.
RESULTS: A total of 470 HIV-infected individuals were followed for 830 person years of observation (PYO). Rate of hematological episodes was 1047 episodes per 1000 PYO. CD4 counts were significantly lower in individuals with severe anemia and neutropenia compared to those without. However, no relation could be established between thrombocytopenia and CD4 counts. In the above- mentioned population, CD4 levels were significantly lower in those with anemia/neutropenia harboring any particular disease compared to those who had the same disease without anemia/ neutropenia.
CONCLUSIONS: There is a strong negative association between CD4 counts and the severity of anemia and neutropenia in this population. They can be considered as good clinical indicators to predict and access the underlying immune status. Though fall in the CD4 levels during neutropenia is observed, it is difficult to comment since the estimations of CD4 rely on the total leukocyte counts. However, the relation between anemia and disease progression is straight forward and quite useful for the treating physician.

4.Cloning of chimerical translocations as positive control for molecular genetic diagnosis of leukemia
Duran Ustek, Sema Sırma, Aris Cakiris, Fulya Coşan, Başar Oku, Uğur Özbek
Pages 20 - 23 (2195 accesses)
Lösemiye özgü kimerik mRNA’ların ters transkriptaz PCR ile saptanmasında standart Kontrollerin olması oldukça önemlidir. Lösemi hastalarından elde edilen kimerik genlere ait pozitif örnekler veya hücre hatlarından elde edilen standartlar yaygın olarak kullanılmaktadır. Gerek hasta örnekleri, gerekse hücre hatlarından elde edilen pozitif kontroller, kısıtlı, pahalı ve bir çok sorunlar oluşturmaktadır. Daha ucuz, tekrar üretilebilir standartların üretilmesi ihtiyaç haline gelmiştir. Biz p190, t (9: 22) p210, t (4: 11), t (1: 19), t (15: 17), t (12; 21) kimerik gen bölgelerini plasmidlere klonlayarak daha ucuz ve tekrar üretilebilen standart pozitif kontrolleri ürettik. Bu plasmidler, lösemilerin moleküler genetik teşhisinde standart pozitif marker olarak PCR ve RT-PCR sırasında kullanılabilir inancını taşımaktayız.
The diagnosis of leukemia-specific mRNAs by polymerase chain reaction (PCR) and reverse transcription-PCR (RT-PCR) require well-known positive standard controls. In general, the positive controls are obtained from cell lines and leukemia patients who have been diagnosed at the molecular level by RT-PCR. These are expensive and restricted sources for standard positive controls. Thus, there is a need for less expensive and reproducible standard positive controls in this area. We have cloned the t (9: 22) p190, t (9: 22) p210, t (4: 11), t (1: 19), t (15: 17), t (12; 21) breakpoint junctions of fusion genes into the plasmids. Cloned fusion genes are suitable for testing PCR experiments of the molecular genetic diagnosis of leukemia samples. We cloned and optimized fusion gene junctions as a standard positive control to check PCR efficiency and as a standard positive marker for diagnosis.

5.T-helper-1 (Th1) and Th2 cytokines after allogeneic hematopoietic stem cell transplantation (allo-HSCT)
Pervin Topçuoğlu, Şule Mine Bakanay, Klara Dalva, Mutlu Arat, Meral Beksaç, Muhit Özcan
Pages 24 - 35 (2216 accesses)
Th1 (IFN-g, IL-2, TNF-a) ve Th2 (IL-4, IL-5, IL-10) tip sitokinler graft versus host hastalığı(GvHH) patogenezinden sorumlu olduğu düşünülmektedir. Allo-HKHT yapılan 23 hastada Th (CD4+) lenfositlerde hücre içi sitokinlerin ekspresyonu hem nötrofil engraftman haftasında (S1) hem de transplantasyon sonrası 100. günde (S2) değerlendirildi. CD4+TNF-a+ hücreler allo-periferik kan (PK) grubunda S1’de % 22’den S2’de % 26’ya arttı. Kemik iliği (K‹) grubunda CD4+IL-2+ hücreler allo-PK grubuna göre daha yüksek olma eğiliminde idi ve S2’dekinden daha yüksekti. Düzey II-IV GvHH olan hastalarda düzey 0-1 GvHH’lilere göre S1 de IL-2 ve TNF-a ekspresyonları anlamlı yüksek ve S2 de IL-2 ekspresyonlarında anlamlı oranda düşük olduğu görüldü. Kronik GvHH’li hastalarda S2’de IFN-g ekspresyonununda artış olma eğilimindeydi. Sonuçta, bu çalışmadan elde edilen veriler GvHH’da Th1 sitokinlerin rolünü desteklemektedir.
Dysregulation of T-helper (Th)1 (IFN-g, IL-2, TNF-a) and Th2 (IL-4, IL-5, IL-10) type cytokines has been suggested in the pathogenesis of graft versus host disease (GvHD). We analyzed intracellular cytokine expression in Th (CD4+) lymphocytes in 23 patients undergoing allo-hematopoietic stem cell transplantation (HSCT) both in the week of neutrophil engraftment (S1) and on the posttransplant 100th day (S2). CD4+TNF-a+ cells increased from 22% at S1 to 26% at S2 in the allo-peripheral blood (PB) group. CD4+IL-2+ cells at S1 in the bone marrow (BM) group had a tendency to be higher than in the allo-PB group and also higher than at S2. IL-2 and TNF-a expressions at S1 were significantly higher and IL-2 expression at S2 was significantly lower in patients with grade II-IV acute GvHD compared with grade 0-I GvHD. IFN-g expression tended to be higher at S2 in the chronic GvHD group compared with the patient without GvHD. Consequently, the data in this study support the role of Th1 cytokines in GvHD.

6.Use of gemtuzumab ozogamicin in the treatment of pediatric relapsed/ refractory Acute Myeloid Leukemia
Elif Ünal, Indira Sahdev
Pages 36 - 41 (3757 accesses)
Gemtuzumab ozogamisin (GO, MylotargTM) erişkin relaps akut myeloid lösemi (AML) vakalarında kemoterapötik olarak çalışılmış monoklonal bir antikordur. Çocuk hastalarda deneyim sınırlıdır. Biz, tedavisinde GO kullanılan altı pediatrik relaps/refrakter CD33+AML hastasını sunmak istedik. GO tedavisi sonrası bir hastada remisyon sağlandı, üç hastada kemik iliğinde <%5 blast ile yanıt elde edildi, iki hasta ise refrakterdi. İlaç yan etkisi olarak ateş ve titreme, hipotansiyon ve hipoksi gözlendi. Üç hastada karaciğerde veno-oklusif hastalık gelişti. Bunlardan ikisinde VOD ölüme katkıda bulundu. Bir hasta GO tedavisini hematopoetik kök hücre transplantasyonu sonrasında aldı ve VOD gelişmedi. Gemtuzumab ozogamisin pediatrik relaps/refrakter AML hastalarında VOD riski nedeniyle dikkatli kullanılmalıdır.
Gemtuzumab ozogamicin (GO, MylotargTM) is an antibody-targeted chemotherapy agent that has been studied in acute myeloid leukemia (AML) at first relapse in adults. There is limited experience in pediatric patients. We report six patients with refractory/relapsed CD33+AML who were treated with GO on compassionate-use basis. One patient attained remission. One patient is still alive following hematopoietic stem cell transplantation (HSCT), and one patient died in remission. Two patients were refractory and three patients had a response with <5% blasts in the bone marrow. Fever and chills, hypotension and hypoxia were observed as side effects. Three patients developed veno-occlusive disease (VOD) of the liver. Two of these three patients had persistence of VOD at the time of their deaths. One patient treated postSCT had bone marrow response without VOD. GO should be used cautiously in chemotherapy-refractory AML pediatric patients due to the high incidence of VOD.

CASE REPORT
7.Hemophagocytic syndrome with erythrocyte phagocytosis by the myeloid precursors in a patient with AML -M2
Mümtaz Yılmaz, Filiz Vural, Mahmut Töbü, Yeşim Ertan, Filiz Büyük
Pages 42 - 44 (2708 accesses)
Hemofagositik sendrom, ateş, halsizlik, kilo kaybı, lenfadenopati ve pansitopeni, bozulmuş karaciğer fonksiyon testi, hipertriglserdemi, hiperferritinemi ile karakterli klinik tablodur. Histopatolojik olarak, retiküloendotel dokularda histiositten zengin mononükleer hücre infiltrasyonu ve active makrofajlar tarafından eritrosit, lökosit, platelet ve bunların öncül hücrelerinin fagositozları ile karekterlidir. Hemofagositik sendromun, şiddetli enfeksiyon, malign ve otoimmün hastalıklar gibi güçlü immünolojik uyaranlar sonucu geliştiği düşünülmektedir.
Hemophagocytic syndrome is characterized by fever, fatigue, weight loss, lymphadenopathy, and laboratory abnormalities including pancytopenia, liver dysfunction, hypertriglyceridemia and hyperfibrinemia. Histopathologically, lesions are characterized by mononuclear cell infiltration with marked histiocyte proliferation and phagocytosis of erythrocytes, leukocytes, platelets and their precursors by activated macrophages in the reticuloendothelial tissues. Hemophagocytic syndrome may develop from strong immunological stimuli such as severe infection, malignancy and autoimmune diseases.
We present a 73-year-old man with acute myeloblastic leukemia FAB M2 type (AML M2) whose bone marrow histology showed unusual hemophagocytosis by myeloid cells and myeloblasts.

8.Albendazole-induced bicytopenia
Erdem Akbal, Ömer Akyürek, Esra Sarıbacak Can, Fahri Güneş, Mustafa Altınbaş
Pages 45 - 47 (3338 accesses)
Kist hidatik echinococcus granulosusa bağlı oluşan ve %55-70 karaciğerde görülen paraziter bir hastalıktır. Kist hidatik tedavisi primer cerrahi olmak üzere, medikal ve PAIR (perkütan aspirasyon injeksiyon reaspirasyon) tedavisi olmak üzere üç şekilde verilebilir. Nüks eden olgularda, cerrahi sonrası rekurrensi azaltmak ve işlemi kolaylaştırmak için PAIR ile birlikte medikal tedavi verilebilir. Medikal tedavide en sık tercih edilen ajan albendazoldur. Albendazol kullanımına bağlı en sık görülen yan etki karaciğer fonksiyon testi yüksekliğidir. Kemik iliği supresyonuna nadiren neden olur. Bu makalede albendazol kullanımına bağlı lökopeni ve trombositopeni gelişen bir vakayı literatürde daha önce görülmemesi nedeniyle sunduk.
Echinococcosis is a parasitic disease which occurs due to cyst hydatid Echinococcus granulosus and occurs in the liver in 55-70% of cases. Cyst hydatid treatment can be performed in three ways: primary surgery, medical and PAIR (Puncture Aspiration Injection Reaspiration) treatment. Medical treatment can be applied with PAIR in order to decrease post-surgery recurrence and facilitate the treatment in repeating cases. The most commonly used agent in medical treatment is albendazole. The most common albendazole-induced side effect is elevated levels of liver enzymes. It rarely causes bone marrow toxic consequences. We present herein a case with albendazole-induced leukopenia and thrombocytopenia, which to our knowledge has never been reported in the literature.

9.First observation of homozygote Hb Q-Iran (alpha 75 (EF4) Asp-His)
Hilal Özdağ, Inci Yıldız, Nejat Akar
Pages 48 - 50 (3747 accesses)
Sivas kökenli anormal hemoglobini olan bir çocuğa moleküler analizle Homozigot Hb Q-Iran (α1 75(EF4) Asp-His tanısı konulmuştur.
The first observation of homozygote Hb Q-Iran (α1 75(EF4) Asp-His) is presented in this report. The clinical and hematological data of the index case, his father and mother showed that homozygous or heterozygous Hb Q-Iran has no clinical importance.

10.First observation of Hb D-Ouled Rabah [beta19(B1)Asn>Lys] in the Turkish population
Aylin Köseler, Anzel Bahadır, Hasan Koyuncu, Ayfer Atalay, Erol Ömer Atalay
Pages 51 - 53 (3448 accesses)
Hb D-Ouled Rabah [beta19(B1)Asn>Lys] Cezayirdeki Tuareg kabilelerinde bildirilen nadir bir hemoglobin varyantıdır. Her ne kadar Çinde de bir olgu bildirilmiş olsa dab u hemoglobin varyantının sadece Berberce konuşan toplumlarda bulunabileceği konusunda bir görüş bulunmaktadır. Bu çalışmada Hb D-Ouled Rabah [beta19(B1)Asn>Lys] Türkiye'de ilk kez bildirilmektedir.
Hb D-Ouled Rabah [beta19(B1)Asn>Lys] is a rare hemoglobin (Hb) beta chain variant reported from Tuareg tribes in Algeria and once from China. It was suggested that Hb D-Ouled Rabah might be specific of Berber-speaking populations. Our report describes the first observation of this hemoglobin variant in the Turkish population.

IMAGES IN HEMATOLOGY
11.Pearson syndrome associated with hemophagocytic syndrome in a child
Fatma Gümrük, Barış Kuşkonmaz, Turgay Coşkun
Pages 54 - 55 (2257 accesses)
Abstract | Full Text PDF

LETTER TO EDITOR
12.Is it a sea-blue histiocyte?
Şinasi Özsoylu
Page 56 (1672 accesses)
Abstract | Full Text PDF

 



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