Crimean-Congo hemorrhagic fever (CCHF) is an acute tick-borne viral disease transmitted to humans by Hyalomma ticks or by direct contact with the blood of infected humans or domestic animals. In certain areas of the world, including Africa, Asia, South East Europe and Middle East, sporadic cases or outbreaks of CCHF have been reported. During the last six-year period from 2003 to 2009, CCHF has also occurred endemically in Turkey, particularly during spring and summer, with a case-fatality rate of approximately 5%. The disease is characterized by acute fever, nausea, vomiting, headache, myalgia, elevated liver enzymes and hemorrhagic manifestations ranging from mucocutaneous bleeding to life-threatening massive hemorrhage with disseminated intravascular coagulation (DIC) and hemophagocytosis. As with other viral hemorrhagic diseases, activation of lymphocytes, monocytes, macrophages and oversecretion of cytokines play a pivotal role in the pathogenesis and prognosis of CCHF. Recently an increasing number of publications on CCHF have been emerging in the literature, majority of which have been written by infection specialists. In this article, recent literature on CCHF has been reviewed, with particular emphasis on hematological manifestations, pathogenesis and therapeutic approaches in CCHF from the hematologist’s point of view. (Turk J Hematol 2009; 26: 161-6)

OBJECTIVE: Thalassemia is one of the most common genetic disorders worldwide. Cap +1 mutation which causes ‘silent beta thalassemia’ is present around all ethnic groups of Pakistan. This study was designed to detect the frequency of Cap+1 mutation in Pakistani Population.
METHODS: Molecular genetic for Cap+1 beta thalassemic mutation was done by extracting DNA from whole blood by using Genomic DNA Purification Kit (Gentra system USA). Amplification Refractory Mutation System (ARMS) primers were designed for detection of normal and mutant DNA.
Basic hematological parameters were performed by using automated analyzer (Sysmex KX-21). Cellulose acetate hemoglobin electrophoresis was done by using semi-automated technique (INTERLAB Roma Microtech Series Electrophoresis system 4.23).
RESULTS: The frequency of Cap+1 mutation was observed 5% (10/200) in targeted thalassemic families (having patients with beta-thalassemia intermedia) while its frequency was observed 2% (12/600) in total thalassemic genes in Pakistani population.
CONCLUSION: Cap+1 (A-C) is a silent mutation and it has very minimum effect on beta globin synthesis because of which it produces very less clinical severity and certain important laboratory diagnostic tests like basic hematological parameters and Hb A2 levels are also remain in normal range. Therefore individuals with Cap+1 mutation may produce children with beta-thalassemia intermedia if they marry an individual with beta-thalassemia minor. Cap+1 (A-C) mutation is an unsuspected cause of beta thalassemia transmission in Pakistani population. This mutation can identify at molecular level. As this molecular defect is difficult to diagnose in Laboratory with routine laboratory tests because of that it has become a serious hindrance for thalassemia prevention program in Pakistan. (Turk J Hematol 2009; 26: 167-70)

OBJECTIVE: Heparin-induced thrombocytopenia (HIT) is a life threatening complication of heparin therapy, causing thrombosis. The aim of our study was to find out the frequencies of HIT antibody seroconversion and clinical HIT in Turkish medical patients on different forms of heparins.
METHODS: Our study included 61 patients who were on unfractionated heparin (UFH) (n: 37) and low molecular weight heparin (LMWH) (n: 24) therapies. The frequency of HIT antibody formation was determined by means of antigenic (ELISA), and functional assays (serotonin release assay-SRA).
RESULTS: The seroconversion rates in UFH and LMWH groups were found to be 18.9% and 4.1% (ELISA), and 8.1% and 4.1% (SRA), respectively. One patient (2.1%) on UFH therapy developed deep vein thrombosis. No thromboembolic event was observed in patients taking LMWH.
CONCLUSION: Seroconversion rates by means of antigenic and functional assays and clinical HIT were more common in patients on UFH than patients on LMWH therapy. (Turk J Hematol 2009; 26: 171-5)

OBJECTIVE: Hemorrhagic cystitis (HC) is a generally self-limited complication of hematopoietic stem cell transplantation (HSCT). It may occur in the early or late posttransplant period and can promote sometimes severe morbidity. We analyzed our data regarding HC in allogeneic HSCT patients in order to establish the efficacy of hyperbaric oxygen (HBO) therapy in severe HC and to document the main problems during its use.
METHODS: Between March 1993 and August 2006, 161 patients received allogeneic HSCT. Mesna, hyperhydration and forced diuresis were used as early HC prophylaxis of cyclophosphamide-induced HC. However, HC was diagnosed in 49 of the 161 recipients and 17 of them were considered as severe HC. We analyzed their data retrospectively.
RESULTS: Forced diuresis with hyperhydration (up to 8 L/day) and transfusion support to maintain a platelet count above 30x109/L were sufficient in 10 of the 17 patients with severe HC. Alternative therapies used included intravesical irrigation with formalin and prostaglandin (PG)F2 alpha and HBO, and HBO appeared to be the most useful among them.
CONCLUSION: We conclude that HBO offers a noninvasive therapeutic alternative in the management of intractable HC in the HSCT setting. (Turk J Hematol 2009; 26: 176-80)

OBJECTIVE: The present study was carried out to investigate the potential effects of extremely low-frequency electromagnetic fields (ELF-EMF) and lead acetate on some hemato-biochemical, immune and pathologic variables in mice.
METHODS: A total of 90 female mice were equally divided into six groups. (Gp. 1) kept as control, (Gp. 2) exposed to EMF of 2 millitesla (mT) intensity and 50 Hz frequency (4h/day) for 30 days, (Gps. 3 and 4) were administered lead acetate orally at doses 1 and 5 mg/kg BW, respectively for 30 days. The last 2 groups (5, 6) were exposed to EMF- lead combination for the same period.
RESULTS: EMF exposure induced a significant increase in RBCs (p<0.001), WBCs (p<0.01) and platelets (p<0.001) counts, compared to control. However, anemia and leukopenia were recorded with oral administration of Pb acetate. The phagocytosis % and phagocytic index were significantly (p<0.05) increased in mice exposed to EMF for 30 days, but decreased (p<0.01) in the animals given the highest dose of lead. Comparing to unexposed mice, significant variation in biochemical parameters (glucose, enzymes, and protein profiles) were noticed. Combined lead and EMF treatments had antagonizing effect on some previous parameters, whereas mice given the highest dose of lead with EMF aggravated hemato-biochemical and pathological findings.
CONCLUSION: We concluded that combined exposure to ELF-EMF and Pb acetate produced significant changes in the hemato-biochemical and immune parameters which were both real and inconsistent. (Turk J Hematol 2009; 26: 181-9)

OBJECTIVE: Fungal infection is a significant problem, causing of infective deaths of leukemic patients. The situation in developing countries is not well documented. The purpose of this study was characterizing IFD by analyzing data retrospectively to determine the incidence, predisposing factors, diagnostic methods, efficacy of treatment, and the outcome in pediatric patients with hematological disorders.
METHODS: There were 160 children with leukemia (22 AML, 129 ALL) and 9 with aplastic anemia (AA). The diagnostic criteria for IFD were defined according to the EORTC/MSG, 2008. IFD was classified as proven or probable. Empiric antifungal treatment with L-AmB was commenced by day 5-7 of persistent fever. Patients with invasive aspergillosis (IA) who were refractory to primary treatment were commenced on voriconazole (VCZ). Salvage therapy as combination of VCZ and caspofungin was given to those with progressive infection.
RESULTS: The incidence of IFD was found 23 (14.3%). 19 with leukemia (14 ALL, 5 AML) and 4 with aplastic anemia were diagnosed as IFD. IA was the dominant cause of infection (n=17) and the rest (n: 6) had candidiasis. Ten children had “proven” infection and 13 children were defined as “probable”. The most frequent site of infection was lungs. In our series, the most frequently used diagnostic methods were clinical findings (100%) and radiologic methods (84%). The success rate of treatment for candidiasis and IA were found 60%, 71% respectively. IFD related death rate was found 30%.
CONCLUSION: IFD is still a major morbidity and mortality reason in children with hematologic disorders. However, the availability of new antifungal treatments and diagnostic tests will improve the survival rates in these children.

OBJECTIVE: The purpose of this study was to investigate the relationship between nitric oxide degradation products (nitrate and nitrite) levels and megaloblastic anemia which is treated with cyalocobalamin.
METHODS: A total of 30 patients with megaloblastic anemia (16 Male, 14 Female) were included in the study. Cyanocobalamin was administered (1.000 µg/day intramuscularly) until the reticulocyte crisis occurred to the normal range. The control group consisted of 30 healthy subjects (15 Male, 15 Female). Nitric oxide levels were measured before treatment and compared with the values obtained during peak reticulocyte count.
RESULTS: Plasma direct nitrite, total nitrite and nitrate levels were 24,86±3,87, 60.56±7,01 and 36,02±5,24 in before treatment versus 15,48±3,05, 38,92±6,44 and 22,77±6,04 μmol/dl in after treatment, respectively. Plasma direct nitrite, total nitrite and nitrate levels were significantly lower in after treatment compared with the before treatment (p<0.001).
CONCLUSION: Nitric oxide levels are seen to increase in megaloblastic anemia. This study suggested that abnormalities in the nitric oxide levels in megaloblastic anemia are restored by vitamin B12 replacement therapy.

Many cases have been established with coexisting Kaposi’s sarcoma (KS) and classical Hodgkin’s Lymphoma (C-HL) in the same lymph node. But composite presentation of KS and Nodular Lymphocyte Predominant subtype of Hodgkin’s lymphoma (NLPHL) in the same lymph node has not been described yet. KS is related to immunodeficiency most frequently due to human immunodeficiency virus (HIV) infection or immunosupression by other reasons. Our case presented here was not related to any immunodeficiency status. Besides of being the first case of composite KS and NLPHL in the same lymph node, it was also unusual with the indolent behaviour of the NLPHL without any therapy for 8 years follow up and primary lymph node presentation of KS without cutaneous involvement.

Acute myelofibrosis is characterized by pancytopenia of sudden onset, megakaryocytic hyperplasia, extensive bone marrow fibrosis, and the absence of organomegaly. Acute myelofibrosis in patients with acute lymphoblastic leukemia is extremely rare. We report a 4-year-old boy who was diagnosed as having acute massive myelofibrosis and acute lymphoblastic leukemia. Performing bone marrow aspiration in this patient was difficult (a “dry tap”), and the diagnosis was established by means of a bone marrow biopsy and immunohistopathologic analysis. The prognostic significance of acute myelofibrosis in patients with acute lymphoblastic leukemia is not clear.

Intestinal mucus accumulation is a very rare situation observed in some solid tumors, intestinal inflammation, mucosal hyperplasia, elevated intestinal pressure, and various other diseases. However, it has never been described in acute myeloblastic leukemia. The pathogenesis of intestinal mucus accumulation is still not clear. Here, we report a 14-year-old girl with acute myeloblastic leukemia and febrile neutropenia in addition to typhlitis. She was also immobilized due to joint contractures of the lower extremities and had intestinal mucus accumulation, which was, at first, misdiagnosed as intestinal parasitosis. We speculate that typhlitis, immobilization and decreased intestinal motility due to usage of antiemetic drugs might have been the potential etiologic factors in this case. However, its impact on prognosis of the primary disease is unknown.