Parvovirus-B19 (PV-B19) küçük bir DNA virusu olup Parvoviridia ailesinin bir üyesidir. PV-B19 ile ilgili hastalıklar virus, konakçı cevabı ve immun sistem etkileşmesini temsil eden iyi birer örnektir. PV-B19 ilişkili hastalıklar, çocukluk çağında eritema infeksiozum, hidrops fetalis ve kronik hemolitik anemilerde geçici eritroid aplazi, kadınlarda artralji, immun yetmezlikli olgularda ise kronik eritroid aplazidir. Sitopeni (bisitopeni, monositopeni yada pansitopeni) yukarıdaki hastalıklara eşlik edebilir. Öte yandan PV-B19 ile ilişkili olabilecek nörolojik, vaskülitik, hepatik, romatoid, nefritik, otoimmun, miyokard hastalıkları ve mekanizması tam olarak açıklanamamış birçok hastalık da vardır. Virus beklenmeyen ve açıklanamayan klinik tablolara yol açabileceği için yanlış tanı konulabilmektedir. Bu nedenledir ki henüz klinik tanı konulmamış, hematolojik bulguları olan hastalarda PV-B19 infeksiyonu dikkate gelmelidir. Ancak immun yetmezlik durumunda serolojik testler tanı için yeterli olmayabilir. Virus serumda yada doku örneğinde PCR ile tespit edilebilir. Destek tedavisi, kan transfüzyonu yada immunglobulin günümüzde kullanılan tedaviler olup aşı çalışmaları araştırma aşamasındadır.

OBJECTIVE: Even though much progress has been made in defining primitive hematologic cell phenotypes by using flow cytometry and clonogenic methods, the direct method for study of marrow repopulating cells still remains to be elusive. Long Term Culture-Initiating Cells (LTC-IC) are known as the most primitive human hematopoietic cells detectable by in vitro functional assays.
METHODS: In this study, LTC-IC with limiting dilution assay was used to evaluate repopulating potential of cord blood stem cells.
RESULTS: CD34 selections from cord blood were completed succesfully with magnetic beads (73,64%±9,12). The average incidence of week 5 LTC-IC was 1: 1966 CD34+ cells (range 1261–2906).
CONCLUSION: We found that number of LTC-IC obtained from CD34+ cord blood cells were relatively low in numbers when compared to previously reported bone marrow CD34+ cells. This may be due to the lack of some transcription and growth factors along with some cytokines and chemokines released by accessory cells which are necessary for proliferation of cord blood progenitor/stem cells and it presents an area of interest for further studies.

OBJECTIVE: Heparin has been shown to be a strong inhibitor of the proliferation of several cell types. In this in vitro study, we investigated whether different heparin concentrations can affect the cell cycle of lymphoblasts in newly diagnosed acute lymphoblastic leukemia (ALL) patients.
METHODS: Lymphoblasts were incubated in different heparin concentrations (0, 10, 20 U/ml), and the percentages of lymphoblasts in each phase of the cell cycle were simultaneously measured by flow cytometry at 0, 1, and 2 hours (h).
RESULTS: The percentages of lymphoblasts at the G2/M and S phases were significantly increased in 20 U/ml heparin concentration at 1 h compared to 0 U/ml (without heparin) concentration. We demonstrated that heparin increases the percentages of lymphoblasts in the S and G2/M phases in a concentration- and time-dependent manner.
CONCLUSION: It was shown that heparin expands the proliferation of lymphoblasts by increasing the transition to G2/M and S phases and the S-phase fraction ratio. Heparin thus appears promising for its contribution to new treatment fields such as by providing a synergistic effect with chemotherapeutic drugs.

OBJECTIVE: The imbalance between neurotoxic cytokine tumor necrosis factor-α (TNF-α) and neurotrophic cytokines epidermal growth factor (EGF) and interleukin-6 (IL-6) plays a role in the pathogenesis of cobalamin (Cbl) deficiency-induced neuropathy. The aim of this study was to evaluate autonomic nervous system dysfunction and to look for any relationship between autonomic nervous system disturbances and serum cytokine levels (TNF-α, EGF, IL-6) in patients with Cbl deficiency.
METHODS: Serum levels of TNF-α, EGF and IL-6 were studied in patients with Cbl deficiency (n=41) and a healthy control group (n=17) and after 3 months in patients who underwent Cbl
replacement therapy (n=22). All patients with Cbl deficiency underwent electrophysiological studies (EPS) for the diagnosis of neuropathy. Statistical analysis was performed using SPSS for Windows 11.5 software.
RESULTS: With EPS, 29 of 41 Cbl-deficient patients (70.73%) demonstrated neurological dysfunction
[3 (7.32%), 19 (46.34%) and 7 (17.07%) patients with sensorimotor peripheral neuropathy, parasympathetic, and sympathetic autonomic dysfunction, respectively]. Although there was no significant difference in serum levels of EGF and IL-6 between patients with versus without autonomic dysfunction, levels were significantly lower in Cbl- deficient patients than healthy controls.
CONCLUSION: Presence of autonomic dysfunction seems to be a frequent neurological finding in patients
with Cbl deficiency. However, we could not find any relationship between serum cytokine levels and autonomic dysfunction by EPS.

OBJECTIVE: This study analyzes the clinical and laboratory findings of children with primary hemophagocytic lymphohistiocytosis (HLH) followed in various referral centers of Turkey.
METHODS: A simple three-page questionnaire prepared by the Turkish Histiocyte Study Group was used for documentation of patient data.
RESULTS: Age at diagnosis varied from 0.6 to 78 months (median±SD, 16.5±26.1). Sex distribution was almost equal (F/M=10/12). The frequencies of parental consanguinity and sibling death in the family history were 100% and 81.1%, respectively. The most common clinical findings were hepatomegaly (100%) and fever (95%). The most common laboratory findings were anemia (100%), hyperferritinemia (100%) and thrombocytopenia (90.9%). Triglyceride and total bilirubin levels in the deceased versus surviving group appear to be high (triglyceride: 394±183 mg/dl, 289±7 mg/dl; total bilirubin: 2.7±6.9 mg/dl, 0.5±1.2 mg/dl, respectively).
CONCLUSION: We concluded that fever, hepatosplenomegaly, anemia, thrombocytopenia, and hyperferritinemia are the most common clinical and laboratory findings in primary HLH. Increased triglyceride and total bilirubin level at the time of diagnosis might be an indicator of poor prognosis in HLH.

OBJECTIVE: Mixed lymphocyte culture (MLC) is one of the routine tests performed prior to hematopoietic stem cell transplantation (HSCT) as a predictive assay for assessing the quality of donor matching and graft-versus-host disease (GVHD). The stimulation index is one of the formulas of the MLC test, and it is used for evaluation of matching between donor and recipient. Modified MLC (mMLC) test is produced by adding various cytokines to the MLC test, and increased sensitivity has been reported with this modification.
METHODS: The importance of the stimulation index values in MLC and mMLC tests was evaluated in 59 patients who received HSCs from human leukocyte antigen-identical sibling donors. In the mMLC test, cytokines were added as interleukin (IL)-2, IL-2 + IL-4 and IL-2 + interferon (IFN)-gamma + tumor necrosis factor (TNF)-alpha. Stimulation index values in mMLC test were compared with stimulation index values in MLC test.
RESULTS: Twenty-three (39%) patients developed GVHD. When evaluated in terms of stimulation index >1 patients, in MLC, 55% of the patients developed GVHD (p=0.229), whereas these values were 75% in the IL-2 added mMLC test (p=0.035), 100% in the IL-2 + IL-4 added mMLC test (p=0.076) and 85.7% in the IL-2 + IFN-gamma + TNF-alpha added mMLC test (p=0.015).
CONCLUSION: mMLC increased the sensitivity of the test. The relation between the positive results and evidence of GVHD after transplantation was found significant.

OBJECTIVE: The aim of this study was to evaluate the effect of radioactive iodine (RAI) ablation therapy on the complete blood count (CBC) in thyroid cancer patients.
METHODS: One hundred sixty four patients undergoing RAI ablation therapy after total thyroidectomy were included. CBC results were available from the patients’ medical records at the time of ablation and at the 1st, 6th, and 12th months after RAI therapy.
RESULTS: Hemoglobin (Hb), white blood cell (WBC) and platelet (Plt) values were significantly lower than baseline at 1 month after treatment (p<0.0001). Hb and WBC values were increased at the 6th month and at the 1st year. Plt values increased at the 6th month but had decreased again at the 1st year. The values were usually in normal ranges except in the patients with low pretreatment Hb and WBC values.
CONCLUSION: RAI ablation therapy in thyroid cancer patients is a safe treatment modality without any serious or persistent hematological side effects.

OBJECTIVE: The aim of this study was to investigate the willingness of university students regarding blood donation and to compare results among residents living in the Kayseri city center.
METHODS: Admission for blood donation after donor acquisition campaigns and the rates of repeated donation over a one-year period were compared between the two groups.
RESULTS: Between November 2006 and August 2008, a total of 29614 people were included in the study. After educational campaigns, the rate of admission for blood donation was 66% among university students, while it was only 29% among the city residents. Although the deferral rate and adverse events during donation were found to be higher in the student group, they had a higher repeated donation rate and higher return rate after a short message system.
CONCLUSION: University students appear to be good candidates for long-term regular blood donation. Use of a short message system to issue reminders about blood donation may be a reasonable method to replenish the blood supply.

OBJECTIVE: Angiogenesis plays a critical role in the development and growth of solid tumors and hematologic malignancies. The system involving angiopoietin-2 [Ang-2] and its receptor Tie-2 appears to play an important role in tumor angiogenesis and in the biology of hematological and non-hematological malignancies. We evaluated the levels of soluble (s)Ang-2 and sTie-2 in acute myeloid leukemia (AML) patients and investigated the impact of their circulating levels on the overall survival in those patients.
METHODS: Ang-2 and Tie-2 were measured in plasma samples from AML patients and controls using enzyme-linked immunosorbent assay (ELISA).
RESULTS: The levels of sAng-2 and sTie-2 were significantly higher in AML patients (2382.1±1586.1 pg/ml and 6.74±3.47 ng/ml, respectively) than in controls (649.5±402.6 pg/ml and 2.63±0.57 ng/ml, respectively; p<0.01). AML patients with high levels of sAng-2 and sTie-2 (≥2500 pg/ml and ≥8 ng/ml, respectively) had significantly shorter overall survival than those patients with low levels (<2500 pg/ml and <8 ng/ml, respectively).
CONCLUSION: The results of our study demonstrated the prognostic significance of circulating sAng-2 and sTie-2 in AML patients. Modulation of the angiopoietin / Tie-2 axis may be a promising approach to improve the outcome in those patients.

Acquired pure megakaryocytic aplasia is a rare hematological disorder characterized by thrombocytopenia with absent or markedly reduced megakaryocytes in the bone marrow. We report a case of a 25-year-old male diagnosed as acquired pure megakaryocytic aplasia. Treatment with prednisone and intravenous immunoglobulin failed, but he was successfully treated with cyclosporine, with complete remission after 90 days and normal platelet count maintained thereafter.

Hepatitis-associated aplastic anemia occurs in up to 10% of all aplastic anemia cases. Syngeneic bone marrow transplantation is rare in patients with severe aplastic anemia and usually requires pre-transplant conditioning to provide engraftment. We report on a 29-year-old male patient with hepatitis-associated severe aplastic anemia who had a series of severe infectious conditions before transplantation, including tracheal inflammation. Life-threatening bleeding, which developed after bronchoscopy, was successfully treated with activated recombinant factor VII and platelet transfusions. Syngeneic peripheral blood stem cell transplantation using immunosuppressive treatment with antithymocyte globulin and cyclosporin A without high-dose pre-transplant conditioning was performed, followed by complete hematologic and hepatic recovery.

We report herein a very rare case of acute lymphoblastic leukemia having a chromosomal constitution of 48,XY,+X,+5,t(9;22)(q34;q11) in the bone marrow. A patient with additional chromosomes X and 5 with a Philadelphia chromosome has not been reported previously. However, no abnormal karyotype was obtained from the lymphocytes in our patient, and he did not have the characteristics of Klinefelter syndrome. He achieved a complete remission with IDA-FLAG and dasatinib therapy. The mechanism of trisomy 5 or any other chromosomal aneuploidy in the pathogenesis of leukemogenesis remains unclear. Further studies involving the genes affected by this karyotype and their products may lead to strategies to further increase the understanding of drug-resistant acute lymphoblastic leukemia and may represent the next frontier in the targeted therapy of those patients.

Hemophagocytic lymphohistiocytosis (HLH) is a disease characterized by phagocytosis of blood cells by macrophages within the lymphoreticular tissue. It can develop secondary to some diseases or be familial as a result of genetic mutations. Niemann-Pick disease (NPD) is a very rare lipid storage disease. A three-month-old girl presented with high fever (39°C), abdominal distension and paleness. The parents were consanguineous. The liver and spleen were palpable 10 cm and 11 cm below the costal margins, respectively. Bicytopenia (Hb: 5.5 g/dl, platelet: 77000/mm3), hypertriglyceridemia (351 mg/dl), hyperferritinemia (>1500 ng/dl) and hypofibrinogenemia (120 mg/dl) were detected. Bone marrow aspiration demonstrated foam cells and hemophagocytosis by macrophages and Niemann-Pick cells. Lysosomal sphingomyelinase activity was 0.24 nmol/h/mg/protein (normal: 0.86-2.8). Due to the parents’ refusal of further evaluation, the nature of HLH as primary or secondary could not be determined. To the best of our knowledge, this is the first case of NPD associated with HLH and the first demonstration of hemophagocytosis by Niemann-Pick cells.

Giant cell hepatitis associated with direct Coombs’ test-positive hemolytic anemia is a rare condition of childhood and the pathogenesis remains unclear. An autoimmune activation and loss of self-tolerance in these patients may be the underlying pathology related to the response of some of the patients to immunosuppressive treatment. Herein, we report the clinical presentation and course of three consecutive patients with this rare condition. We conclude that serum ferritin at diagnosis may be used for prediction of the outcome.