Hematolojik malignitelerin tedavisinde önemli sorunlardan birisi de invazif fungal infeksiyonlarýn tanýsýdýr. Mikolojik tanýdaki zorluklar ve hýzlý müdahele etme gerekliliði, küf mantarlarýnýn tanýsýnda dolaylý iþaretleyicilerin geliþtirilmesine yol açmýþtýr. En sýk kullanýlan taný testleri galaktomannan, beta-glukan ve moleküler yöntemlerdir. Her ne kadar bu taný yöntemleri ile ilgili çok sayýda araþtýrma yapýlmakta ise de, hiçbiri HIV ya da CMV’de olduðu gibi doðrudan yarar saðlamamaktadýr. Taný ile ilgili bu yazý klinisyene eldeki araçlarý kullanma konusunda yol göstermeyi amaçlamaktadýr. Moleküler testlere henüz standardize edilmediði ve klinik kullanýma girmediði için bu yazýda deðinilmeyecektir.

One of the most problematic issues in hematological malignancies is the diagnosis of invasive fungal diseases. Especially, the difficulty of mycological diagnosis and the necessity of immediate intervention in molds have led to the adoption of “surrogate markers” which does not verify, but strongly suggests fungal infection. The markers commonly used are galactomannan (GM), beta-glucan and imaging methods. Although there are numerous studies on these diagnostic approaches, none of these markers serve as a support for the clinician, as is in human immunodeficiency virus (HIV) or cytomegalovirus (CMV) infections. This paper has been prepared to explain the diagnostic tests and show the clinician how the available resources can be used. As moleculer tests have not been standardized and not used routinely in the clinics, they will not be mentioned here.

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AMAÇ: Hücre adezyon molekülü olan selektinler, lökositlerle, aktive plateletler ya da endotel hücreleri arasýndaki etkileþime aracýlýk eder. Bu çalýþmada antifosfolipid sendromu (AFS) hastalarýnda tromboz riski ile P-selektin polimorfizmleri arasýndaki iliþkinin araþtýrýlmasý amaçlanmaktadýr.
YÖNTEMLER: AFS tanýsý “International Workshop” taný ve sýnýflandýrma kriterleri ile konulmuþtur. Hastalarýn periferik kan örneklerinden DNA elde edilmiþtir. P-selektin gen bölgesiyle iliþkili üç tane tek nükleotid polimorfizmi (S290N, c.1087G>A; N562D, c.1902G>A; T715P, c.2363A>C) araþtýrýlarak genotipleri belirlenmiþtir.
BULGULAR: Hasta grubunda 26 trombozlu (%65) AFS olgusu ve 14 (%14) trombozu olmayan AFS olgusu yer almaktadýr. Hasta ve kontrol grubu kýyaslandýðýnda incelenen polimorfizlerden N562D-DN genotipi hasta grubunda anlamlý olarak yüksek bulunmuþtur (p: 0,003). Yine hasta grubuna bakýldýðýnda trombozlu AFS grubunda trombozu olmayan AFS grubuna kýyasla N562D-DN genotipine anlamlý olarak sýk rastlanmýþtýr (p: 0,03). N562D NN genotipi ise kontrol grubunda hasta grubuna oranla daha yüksek sýklýktadýr (p: 0,004).
SONUÇ: Sonuç olarak P-selektin N562D polimorfizmi DN genotipi primer AFS hastalarýnda tromboz riski ile iliþkili olduðu söylenebilir.

OBJECTIVE: The selectins are cell adhesion molecules that mediate the interactions among leukocytes, activated platelets, and endothelial cells. We aimed to investigate whether P-selectin polymorphisms are associated with thrombosis in patients with antiphospholipid syndrome (APS).
METHODS: The diagnosis and classification of APS were based on the report of an international workshop. Genomic DNA was extracted from citrated blood samples of all subjects. Three single nucleotide polymorphisms associated with the P-selectin coding region (S290N, c.1087G>A; N562D, c.1902G>A; T715P, c.2363A>C) were assessed.
RESULTS: There were 26 APS (65%) patients with thrombosis. The number of patients without thrombosis was 14 (35%). The frequency of the N562D-DN genotype was significantly higher in patients with APS than in healthy controls (p=0.003). The frequency of this genotype was significantly higher in patients with APS with thrombosis compared with patients with no thrombosis (p=0.03). The N562D-NN genotype was found at a higher frequency in patients with APS than in healthy controls (p=0.004).
CONCLUSION: Our results suggest that the N562D polymorphism of the DN genotype of P-selectin is associated with an increased risk of thrombosis in patients with APS.

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AMAÇ: Bcl-2 ve Bcl-xL gibi antiapoptotik proteinler eritroid progenitör hücrelerin yaþam süresinde rol oynayabilir. β-talasemi minör hastalarda bu proteinler ile ilgili bilgiler sýnýrlýdýr. Biz, β-talasemi minörlü hastalarda serum Bcl-2 düzeylerinin belirlenmesi amaçladýk.
YÖNTEMLER: β-talasemi minör tanýlý doksanyedi hasta (60 kadýn ve 37 erkek, yaþ ortalamalarý 29±21 yýl) bu çalýþmaya dahil edildi. β-talasemi minör tanýsý tam kan sayýmý, aile öyküsü, ve yüksek performanslý sývý kromatografisine dayanan HbA2 düzeyleri ile konulmuþtur. Kontrol grubu anemisi olmayan 23 saðlýklý yetiþkin (17 kadýn ve 6 erkek, yaþ ortalamasý 58±9 yýl) idi. Serum Bcl-2 düzeyleri ELISA yöntemi ile ölçüldü. Ýstatistiksel deðerlendirmede Mann–Whitney U testi kullanýldý ve p<0.05 istatistiksel olarak anlamlý kabul edildi.
BULGULAR: Serum Bcl-2 düzeyinde β-talasemi minörlü hastalar ile kontrol grubu arasýnda istatistiksel olarak anlamlý farklýlýk olmamasýna raðmen (p>0.05), β-talasemi minörlü hastalarda bu seviyeler kontrol grubuna göre daha yüksekti.
SONUÇ: β-talasemi minorde hasarlý beta zincirleri bulunmaktadýr. Bu nedenle, kýrmýzý kan hücrelerinin erken ölümünün apoptoz nedeniyle olmasý beklenmektedir. Kontrol grubunun yaþ ortalamasý talasemi taþýyýcýlarýndan yüksektir, bundan dolayý Bcl-2 düzeyleri β-talasemi minörde yüksek olabilir. Ýleri yaþýn artmýþ apoptoz için bir risk oluþturtuðu bilinmektedir. Apoptoz ile iliþkili diðer protein (Bad, Bax, vb) ve yolaklar [CD95 (Fas) ligand] hasta sayýsýnýn daha fazla olduðu çalýþmalarda deðerlendirilmelidir.

OBJECTIVE: Anti-apoptotic proteins such as Bcl-2 and Bcl-xL may play a role in the survival of erythroid progenitor cells. Information about these proteins in patients with β-thalassemia minor is limited. We aimed to determine the levels of serum Bcl-2 in patients with β-thalassemia minor.
METHODS: Ninety-seven patients (60 females and 37 males with mean age of 29±21 years) with β-thalassemia minor were enrolled in this study. The diagnosis of β-thalassemia minor was based on whole blood counts, family history, and HbA2 levels estimated by high-performance liquid chromatography. The control group compromised 23 healthy adults (17 females and 6 males with mean age of 58±9 years) without anemia. The levels of serum Bcl-2 were measured by enzyme-linked immunosorbent assay. Mann–Whitney U tests were used in statistical evaluation and p< 0.05 was accepted as statistically significant.
RESULTS: Although there was no statistically significant difference between patients with β-thalassemia minor and the control group for the level of serum Bcl-2 (p>0.05), these levels were higher in β-thalassemia minor patients than controls.
CONCLUSION: There are damaged beta chains in β-thalassemia minor. Therefore, it is expected that premature death of red blood cells may occur due to apoptosis. The mean age of the control group was higher than that of the β-thalassemia minor group; this may be why Bcl-2 levels were higher in the β-thalassemia minor group. It is known that older age constitutes a risk for increased apoptosis. Other proteins (Bad, Bax, etc.) and pathways [CD95 (Fas) ligand] associated with apoptosis should be evaluated in future studies including more patients.

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AMAÇ: Eritrositlerin ABO ve RhD sistemleri dýþýndaki konvansiyonel serolojik tiplendirmesini son zamanlarda sýk kan transfüzyonu yapýlmýþ olan kiþilerde yorumlamak yanlýþ ve zor olabilir. Bazý moleküler incelemeler henüz rutin olarak kullanýlmamasýna raðmen, genotiplendirmenin yararý bu iþlemden fayda görecek hastalarý belirlemek amacýyla incelenmiþtir.
YÖNTEMLER: Hemato-onkoloijk, kronik böbrek hastalýðý veya gastrointestinal hastalýðý olan 101 hastadan ve kontrol grubu olan 50 kiþiden karþýlaþtýrmak üzere kan örnekleri alýndý. Numuneler serolojik ve genetik yöntemler kullanýlarak Fya ve Fyb için test edildi. Bütün hastalara son 3 ay içinde 3 ve daha fazla ünite eritrosit süspansiyonu transfüzyonu yapýlmýþtý. Her hasta için ortalama transfüzyon 6,1 ünite olarak hesaplandý. Transfüzyondan örneklerin alýnmasýna kadar geçen ortalama süre 24,4 gündü.Hemaglütinasyon testi serolojik analiz için uygulandý, ve uzunluðu kýsýtlanmýþ polimorfizm testi genotipleme için kullanýldý.
BULGULAR: Genetik olarak negatif olan, toplam 33 (%32,7) hastada anti-Fya veya anti-Fyb ile pozitif reaksiyon elde edildi. Yirmi üç örnekte yanlýþ pozitif Fya sonucu, 10 örnekte yanlýþ pozitif Fyb sonucu elde edildi. Son 3 ayda fenotiplendirme/genotiplendirme sonuçlarý tutarsýz olanlarda uyumlu olanlara göre anlamlý olarak daha fazla eritrosit süspansiyonu transfüzyon yapýlmýþtý: sýrasýyla ortanca 5 (ortalama ± SE: 6,85±0,69) ve ortanca 4 (ortalama ± SE: 5,71±0,51) (p=0,025). Fenotiplendirme/genotiplendirmesi sonuçlarý uyumlu olan grupta transfüzyondan test aþamasýna kadar geçen ortanca süre 25 gün (ortalama: 28,72±2,23) iken, sonuçlarý uyumsuz çýkan grupta bu süre ortanca 14 (ortalama: 15,52±1,95) gündü. Tüm donör örneklerinde fenotip ve genotipler tutarlýydý.
SONUÇ: Örnek alýnmasýndan 3 veya 4 hafta öncesinde transfüzyon alan kiþilerde Duffy sistemi için genotiplendirme yapýlmasý uygun olabilir. Eðer eritrosit süspansiyonu transfüzyonundan sonra geçen süre 6 haftadan fazla ise, Duffy fenotiplendirmeyle uygun ve güvenilir sonuçlar sunabilir.

OBJECTIVE: Conventional serologic typing of red blood cell systems other than ABO and RhD can be inaccurate and difficult to interpret in patients who have recently undergone blood transfusion. While molecular-based assays are not used routinely, the usefulness of genotyping was investigated in order to determine patients who may benefit from this procedure.
METHODS: Blood samples were taken from 101 patients with haemato-oncological, chronic renal, or gastroenterological diseases and from 50 donor controls; the samples were tested for Fya and Fyb by applying serologic and genetic methods. All patients had received 3 or more units of RBCs during the last 3 months. An average of 6.1 RBC units were transfused per patient. The average length of time from transfusion until blood sampling was 24.4 days. The haemagglutination test was applied for serological analysis, and the restriction length polymorphism assay was used for genotyping.
RESULTS: In total, 33 (32.7%) patients showed positive reactions with anti-Fya or anti-Fyb while being negative genetically. False-positive Fya results were found in 23 samples, and false-positive Fyb in 10 specimens. During the last 3 months, significantly more RBC units were transfused to patients with discrepant results than to those with accurate phenotyping/genotyping results: median of 5 (mean±SE: 6.85±0.69) versus median of 4 (mean: 5.71±0.51), respectively (p=0.025). The median length of time after the last transfusion was 25 days (mean: 28.72±2.23 days) in the group with accurate phenotyping/genotyping results versus a median of 14 days (mean: 15.52±1.95 days) in the group with discrepant results (p=0.001). Phenotypes and genotypes coincided in all donor samples.
CONCLUSION: Genotyping assays for the Duffy system should be considered if the patient underwent blood transfusion less than 3 or 4 weeks before the sample collection. If the time frame from RBC transfusion exceeds 6 weeks, Duffy phenotyping can provide accurate results.

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AMAÇ: Bu çalýþmanýn amacý; multipl myelomda (MM) kullanýlan farklý tedavi rejimlerinin kemik-spesifik alkalen fosfataz (BALP) düzeyleri üzerine etkisini incelemektir.
YÖNTEMLER: Çalýþmaya 113 MM hastasý dahil edildi. Hastalar aldýklarý tedavi rejimlerine göre; 1. grup, mefelan ve prednizolon (MP); 2. grup, vinkristin, adriablastin ve deksametazon (VAD); 3. grup talidomid artý deksametazon; 4. grup, bortezomib artý deksametazon olarak gruplara ayrýldý. BALP düzeyleri tedaviden önce, tedavinin üçüncü ve altýncý aylarýnda ölçüldü. Çalýþmanýn baþýnda, tedavi sonrasý remisyon dönemindeki hastalardan beþinci bir grup oluþturuldu (tedavi almayan grup).
BULGULAR: Bortezomib grubu ve tedavi almayan grupta tedavinin üçüncü ve altýncý aylarýnda bakýlan BALP düzeyleri, tedavi öncesine göre anlamlý olarak yüksek bulunurken; MP, VAD, ve talidomid gruplarýnda anlamlý fark saptanmadý.
SONUÇ: BALP’ýn kemik formasyonunun bir belirteci olduðu göz önünde tutulduðunda, bu çalýþma ile diðer tedavi seçeneklerine göre bortezomib tedavisinin miyelomda kemik hastalýðýnýn iyileþmesine daha etkin bir þekilde yol açtýðý gösterilmiþtir.

OBJECTIVE: This study aimed to investigate the impact of the different therapy regimens used in multiple myeloma (MM) on bone-specific alkaline phosphatase (BALP) levels.
METHODS: One hundred and thirteen patients with MM were included in the study. Patients were grouped according to the regimens they received, as follows: group 1, melphalan and prednisolone (MP); group 2, vincristine, adriablastin, and dexamethasone (VAD); group 3, thalidomide plus dexamethasone; and group 4, bortezomib plus dexamethasone. BALP levels were measured before treatment and at the third and sixth months of treatment. A fifth group consisted of patients in the post-treatment remission period at study entry (no-treatment group).
RESULTS: The BALP levels at the third and sixth months of the treatment were significantly higher than the pre-treatment levels in the bortezomib and the no-treatment groups, whereas no significant difference was observed in the MP, VAD, and thalidomide groups.
CONCLUSION: Considering that BALP is a surrogate marker of bone formation, our study suggests that bortezomib more efficiently leads to the improvement of bone disease in myeloma than other treatment options.

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AMAÇ: Non-Hodgkin lenfoma (NHL) riski enfeksiyonlar ve çevresel ajanlarla artmýþ olarak iliþkilendirilmiþtir. Hipotezimize göre bu faktörlere kronik olarak maruz kalmak T helper-2 (Th2) yolaðýný aktifler ve lenfomaya sebep olur. Bu amaçla Th2 yolaðý komponentleri olan IL-10, IL-4, IgE, ve eozinofille, NHL’nin prognostik belirteçleri arasýndaki iliþkiye baktýk.
YÖNTEMLER: Otuz bir NHL hastasý ve 27 saðlýklý kontrolde IL-10, IL-4, IgE ve eozinofil deðerlerine bakýldý. IL-4 ve IL-10 EASIA metodu ile ölçüldü.
BULGULAR: Yüksek IL-10 düzeyleri pek çok kötü prognostik özellikle, çok kýsa yaþam süresi ve lenfopeni ile iliþkiliydi. Albümin açýsýndan IL-10 ile negatif iliþki, IL-4 ile pozitif iliþki bulundu. Eozinofil ve IgE ile ilgili herhangi bir iliþki kurulamadý. Bazý hastalarda gözlenen artmýþ IL-4 geri planda kalmýþ Th2 yolaðý aktivasyonunun bir ip ucu olabilir.
SONUÇ: Th2 yolaðýnýn NHL patogenezindeki yerini tespit etmek için farklý dizaynlarda çalýþmalara ihtiyaç vardýr.

OBJECTIVE: Increased risk for non-Hodgkin lymphoma (NHL) is associated with infections and environmental agents. We hypothesized that these factors chronically trigger the T helper-2 (Th2) pathway and result in lymphoma. We investigated the role of the Th2 pathway by exploring the relationships between components of the Th2 pathway, interleukin (IL)-10, IL-4, immunoglobulin E (IgE), and eosinophils, and prognostic markers of NHL.
METHODS: Thirty-one NHL patients and 27 healthy controls were enrolled. IL-10, IL-4, IgE, and eosinophils were measured. IL-4 and IL-10 were analyzed with the enzyme amplified sensitivity immunoassay method.
RESULTS: High IL-10 levels were correlated with several poor prognostic features, short early survival, and lymphopenia. There was a positive correlation between albumin and IL-4 levels and a negative correlation between IL-10 and albumin. There was no relationship related with eosinophils and IgE. We found remnant increased IL-4, which could be a clue for the triggering of the Th2 pathway in the background.
CONCLUSION: There is a need for differently designed studies to detect the place of the Th2 pathway in NHL.

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AMAÇ: Multiple myeloma (MM), malign plazma hürelerinin klonal çoðalmasý ile karakterize bir B hücre neoplazisidir. Çeþitli çalýþmalarýn sonuçlarýna göre, bazý sýnýf 1 ve 2 HLA genlerinin hastalýða yatkýnlýk dair görüþler ortaya atýlmýþtýr.Farklý popülasyonlarda yapýlan çalýþmalarda, farklý HLA sýnýf 1 ve 2 allellerinin MM üzerine etkisi olduðu bildirilmiþtir. Bu çalýþmada, Ýranlý MM hastalarýnda HLA sýnýf 1 ve sýnýf 2 antijenlerinin birlikteliðini deðerlendirdik.
YÖNTEMLER: HLA-Ready Gene ABDR kitleriyle tekli spesifik primer polimeraz zincir reaksiyonu yönteminin kullanýldýðý bu olgu-kontrol genetiplendirme çalýþmasýnda, hasta grubuna Taleghani Hastanesi kemik iliði nakli bölümünden seçilen 105 Ýranlý MM hastasý ve 150 de kontrol olgusu dahil edilmiþtir.
BULGULAR: Çalýþma sonucunda, HLA-A*03 hasta grubunda %21 ve kontrol grubunda %12 bulunurken, HLA-B*18 ise hasta grubunda %11 ve kontrol grubunda %3 olarak saptanmýþtýr. MM hastalarýnýn HLA-A*03 ve HLA-B*18 allele sahip olma oraný kontrol olgularýyla karþýlaþtýrýldýðýnda istatistiki olarak anlamlý olacak þekilde yüksek bulunmuþtur (p=0,039, OR=2,057 ve p=0,013, OR=3,567, sýrasýyla).
SONUÇ: Bizim bulgularýmýz, Ýran toplumunda HLA-A*03 ve HLA-B*18 allel varlýðýnýn istatistiki olarak anlamlý olacak þekilde MM’ye yatkýnlýk yarattýðýný ortaya koymaktadýr. Bununla birlikte, diðer toplumlara bakýldýðýnda adý geçen allellerin ayný sonucu doðurmadýklarý görülmektedir. Farklý etnik gruplar arasýndaki bu birlikteliði deðerlendiren fazla sayýda çalýþma olmadýðý için, gelecek dönemlerde MM’li hastalarda HLA genlerinin birlikteliðinin sonuçlarýný izah edebilecek daha ayrýntýlý çalýþmalara gereksinim vardýr.

OBJECTIVE: Multiple myeloma (MM) is a B-cell malignancy characterized by the clonal proliferation of malignant plasma cells.
According to results of some studies, it has been suggested that the HLA class 1 and 2 genes have susceptibility effects on MM. Studies of different populations have reported different HLA class 1 and 2 alleles that affect MM. In this study, we assessed the association of HLA class 1 and class 2 antigens with MM in Iranian patients
METHODS: We performed a case-control genotyping study with 105 Iranian MM patients that were selected from the bone marrow transplantation department of Taleghani Hospital and 150 controls using single specific primerpolymerase chain reaction with the HLA-Ready Gene ABDR Kit.
RESULTS: Our results demonstrated that 21% of patients versus 12% of controls and 11% of patients versus 3% of controls carried HLA-A*03 and HLA-B*18, respectively. The MM patients had a significant increase in the frequency of HLA-A*03 and HLA-B*18 alleles in comparison to control subjects (p=0.039, OR=2.057 and p=0.013, OR=3.567, respectively).
CONCLUSION: Our findings suggested that the HLA-A*03 and HLA-B*18 alleles have significant susceptibility effects on MM in the Iranian population. However, compared to other populations, the above-mentioned alleles had different statuses. Since there are not many studies evaluating and calculating this association among ethnic groups, further studies among other populations are needed to explain the exact association of the HLA genes with MM.

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AMAÇ: Hepsidin demir dengesinde önemli bir rol oynar. Temel olarak hepatositler tarafýndan üretilir ve intestinal epitel hücrelerinden demir alýmýný ve makrofajlardan demir salýnýmýný inhibe eder. Kompetitif ELÝZA en düþük deðeri tespit edebilmesi, düþük maliyeti ve yüksek iþ gücünden dolayý kantitatif serum hepsidin miktari ölçümü için tercih edilen mevcut bir yöntemdir. Bu çalýþmanýn amacý son zamanlarda myelodisplazi (MDS) tanýsý almýþ olgularda demir yükünün patogenezinde hepsidinin rolünü tartýþmaktýr.
YÖNTEMLER: Bu çalýþmaya 21 yeni taný MDS hastasý ve 13 saðlýklý kontrol alýndý. Ferritin, hepsidin ve soluble transferrin reseptörü (sTFR) tüm olgularda ölçüldü.
BULGULAR: Hiposellüler MDS li 7 olgu, multilineage displazili refrakter sitopenili 8 olgu ve artmýþ blastlý refrakter anemili 6 olgu vardý. Bu 3 MDS alt tipleri arasýnda hepsidin, sTFR ve ferritin düzeyleri açýsýndan fark gözlenmedi (p>0,05). Ortalama hepsidin seviyesi sýrasýyla MDS grubunda 55,8±21,5 ng/mL ve kontrol grubunda ise 19,9±2,6 ng/mL idi. Ortalama sTFR, MDS grubunda 45,7±8,8 nmol/L ve kontrol grubunda ise 31,1±5,6 nmol/L idi. Ortalama ferritin seviyeleri ise MDS grubunda kontrolden anlamlý olarak yüksekti (539,14±83,5 ng/mL vs. 104,6±42,9 ng/mL, p<0,005). Hepsidin ve sTFR arasýnda istatistiksel anlamlý bir iliþki vardý (r=0,45, p=0,039). Hepsidin düzeyleri erkeklerde kadýnlarla karþýlaþtýrýldýðýnda daha düþük deðerlerde bulunmasýna karþýlýk erkek ve kadýnlar arasýnda istatistiksel anlamlý fark görülmedi (47,9±27,6 vs. 66,7±35,7, p>0,05).
SONUÇ: Hepsidin MDS de aþýrý demir yükünün ana nedeni olmayabilir. MDS li olgularda hepsidine periferal yanýtsýzlýk veya üretiminin yetersizliðini test edecek baþka çalýþmalara ihtiyaç vardýr.

OBJECTIVE: Hepcidin plays a pivotal role in iron homeostasis. It is predominantly produced by hepatocytes and inhibits iron release from macrophages and iron uptake by intestinal epithelial cells. Competitive ELISA is the current method of choice for the quantification of serum hepcidin because of its lower detection limit, low costs, and high throughput. This study aims to discuss the role of hepcidin in the pathogenesis of iron overload in recently diagnosed myelodysplasia (MDS) cases.
METHODS: The study included 21 recently diagnosed MDS patients and 13 healthy controls. Ferritin, hepcidin, and soluble transferrin receptor (sTFR) were measured in all subjects.
RESULTS: There were 7 cases of hypocellular MDS, 8 cases of refractory cytopenia with multilineage dysplasia, and 6 cases of refractory anemia with excess blasts. No difference was observed among the 3 MDS subtypes in terms of hepcidin, sTFR, and ferritin levels (p>0.05). Mean hepcidin levels in the MDS and control groups were 55.8±21.5 ng/mL and 19.9±2.6 ng/ mL, respectively. Mean sTFR was 45.7±8.8 nmol/L in MDS patients and 31.1±5.6 nmol/L in the controls. Mean ferritin levels were significantly higher in MDS patients than in controls (539.14±83.5 ng/mL vs. 104.6±42.9 ng/mL, p<0.005). There was a statistically significant correlation between hepcidin and sTFR (r=0.45, p=0.039). No difference in hepcidin levels between males and females was observed, although it was lower in males in comparison to females (47.9±27.6 vs. 66.7±35.7, p>0.05).
CONCLUSION: Hepcidin may not be the main cause of iron overload in MDS. Further studies are required to test failure of production or peripheral unresponsiveness to hepcidin in MDS cases.

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Miyelosupresyon, akut lenfoblastik lösemi tedavisinde görülen ciddi bir komplikasyon olup, miyelosupresyon süresi kemik iliði yetmezlik sendromlarýndan veya genetik polimorfizmin yol açtýðý ilaç farmakogenetiðinden etkilenmektedir. Merkaptopürin metabolizasýnda yer alan Thiopurin s-metil transferaz (TPMT) enziminin azalmýþ aktivitesine neden olan polimorfizmleri artmýþ kemik iliði toksisitesine yol açar. Burada, 15 yaþýnda TPMT *3A/*3C ve MTHFR polimorfizmleri saptanan ve konsolidasyon/ idame tedavisi boyunca MP’nin standart dozun %5’inde yoðun miyelosupresyon görülen ve steroid tedavisi ile geçici düzelme saptanan bir T-ALL olgusu sunulmaktadýr.

Myelosuppression is a serious complication during treatment of acute lymphoblastic leukemia and the duration of myelosuppression is affected by underlying bone marrow failure syndromes and drug pharmacogenetics caused by genetic polymorphisms. Mutations in the thiopurine S-methyltransferase (TPMT) gene causing excessive myelosuppression during 6-mercaptopurine (MP) therapy may cause excessive bone marrow toxicity. We report the case of a 15-year-old girl with T-ALL who developed severe pancytopenia during consolidation and maintenance therapy despite reduction of the dose of MP to 5% of the standard dose. Prednisolone therapy produced a remarkable but transient bone marrow recovery. Analysis of common TPMT polymorphisms revealed TPMT *3A/*3C.

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Ýntravasküler büyük B hücreli lenfoma (ÝVBBHL), non-Hodgkin lenfomalarýn nadir bir tipidir, genellikle ileri yaþta görülür, ve neoplastik hücrelerin damar lümeni içerisinde seçici infiltrasyonu ile karakterlidir. Prostat tutulumu ile taný konan ÝVBBHL olgularý ise çok nadirdir. 65 yaþýnda, daha çok nörolojik þikayetleri olan, ancak transüretral prostat rezeksiyon materyalinin histopatolojik incelemesinde, tamamen küçük damar lümenleri içerisinde sýnýrlý neoplastik büyük hücre infiltrasyonunun görülmesi ile taný konan bir olguyu sunuyoruz. Neoplastik hücre infiltrasyonu immunhistokimyasal incelemede MUM1, bcl-6 ve bcl-2 ile pozitif, ALK1, CD10, CD30 ile negatif saptandý, Ki67 proliferasyon indeksi yüksekti. CD34 ve CD31 endotelial hücrelerde pozitif olup, neoplastik infiltrasyonun damar içi yerleþimini belirgin olarak ortaya koydu. Hasta, ne yazýk ki tedavi almayý kabul etmedi ve sekiz ay içerisinde hastalýk nedeniyle kaybedildi. IVLBCL, az görülmekle birlikte, intravasküler infiltrasyon içeren tüm organ biopsilerinde ayýrýcý taný içerisinde yer almalýdýr. Bu nadir lenfoma tipinin patogenez ve biyolojisinin aydýnlatýlmasýna ihtiyaç duyulmaktadýr.

Intravascular large B-cell lymphoma (IVLBCL) is a very rare type of non-Hodgkin lymphoma, usually affecting elderly patients and characterized by selective infiltration of neoplastic cells within blood vessels’ lumina. IVLBCL diagnosed with prostatic involvement is extremely rare. We report a patient of 65 years old, having mostly neurological complaints but diagnosed with IVLBCL upon histopathological examination of transurethral prostate resection material, which revealed large neoplastic cell infiltration totally limited within the lumens of small vessels. By immunohistochemistry, neoplastic cell infiltration was positive with MUM1, bcl-6, and bcl-2 and negative with ALK1, CD10, and CD30, with a high Ki-67 proliferation index. CD34 and CD31 staining showed expression in endothelial cells, highlighting the intravascular nature of neoplastic infiltrate. The patient unfortunately refused to receive treatment and died of the disease 8 months after the diagnosis. IVLBCL, though very rare, should be considered in differential diagnosis of all organ biopsies with intravascular infiltration. Further improvements in the understanding of the pathogenesis and biology of this rare type of lymphoma are mandatory.

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Primer splenik anjiosarkom dalaðýn hematolojik olmayan tümörlerinden en sýk karþýlaþýlanýdýr. Yayýnlanmýþ serilerde metastaz %69 olarak belirtilmiþse de kemik iliði metastazýnýn sýklýðý tam bilinmemektedir. Biz Faslý bir kadýn hastada kemik iliði metastazý ile açýða çýkan daha önceden þüphelenilmemiþ bir primer splenik anjiosarkom vakasýný rapor etmek istiyoruz. Yirmi beþ yaþýndaki hastanýn ciddi anemi ve splenomegalisi mevcuttu. Kemik iliði biyopsisinde kemik iliði alanlarýnýn yerini prolifere olmuþ iðsi hücrelere býraktýðý gözlendi. Bilgisayarlý Tomografide büyümüþ dalak içinde heterojen lezyonlar görüldü. Splenektomi yapýldý ve dalak üzerinde yapýlan histolojik çalýþma primer splenik anjiosarkom tanýsýný doðruladý. Hasta kemoterapi aldý fakat splenektomiden 1 yýl sonra metastazdan öldü.

Primary splenic angiosarcomas are the most common malignant non-hematopoietic tumors of the spleen. Metastatic diseases were found in 69% of patients in a reported series but the incidence of bone marrow involvement is unclear. We report a rare case of a 25-years-old Moroccan woman with unsuspected primary splenic angiosarcoma revealed by bone marrow metastasis. She presented with serious anemia and splenomegaly. Bone marrow biopsy revealed proliferating spindle cells. Computed tomography scanning showed an enlarged spleen with heterogeneous lesions. Splenectomy was performed and retrospective histological study of the spleen confirmed the diagnosis. She died 1 year after splenectomy.

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Onikomikoz (OM) el ve ayak týrnaklarýnýn sýk görülen fungal enfeksiyonudur. Genel toplumda prevalansý yüksek bir hastalýk olmasý nedeniyle önemli morbiditeye yol açmaktadýr. OM dermatofitler, nondermatofitik küf mantarlarý veya mayalar ile ortaya çýkan hastalýklardýr. Günümüzde onikomikozun tedavisinde sistemik antifungal ajanlar tüm OM tiplerinde altýn standart tedavi olarak kabul edilmektedir. Biz burada, oral terbinafin kullanýmý sýrasýnda geliþen bir aplastik anemi olgumuzu ve literatürde terbinafine ile iliþkilendirilmiþ olan hematolojik toksisitelerin derlemesini sunuyoruz.

Onychomycosis (OM) is a common fungal infection of the toenails and/or fingernails that is highly prevalent in the general population and also responsible for significant morbidity. OM is caused by dermatophytes, nondermatophytic molds, or yeast. Today systemic antifungal agents are considered as the gold standard for all types of OM. Here we report a case of aplastic anemia associated with oral terbinafine use and a review of the literature on hematological toxicities associated with terbinafine.

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