Plazma hücre malignitesi klasik olarak dört gruptur: multiple myelom (MM), plazma hücre lösemisi, kemik yerleþimli soliter plazmasitom (SPB) ve ekstrameduller yerleþimli plazmasitom (EMP). Lokalize plazma hücre malignitesini iskelet sistemi yerleþimli SP ve EMP oluþturur. Plazmasitoma birçok farklý þekilde tanýmlanabilir. Çoðunlukla kemik iliðinde veya yumuþak dokuda plazma hücrelerinden oluþan bir kitle þeklinde görülür. Henüz, soliter plazmositomlarýn MM’a dönüþümü arasindaki iliþki tam olarak tanýmlanmamýþtýr. Soliter plazmositomlar, hem SPB hem de EMP için ana tedavi radyoterapidir. Ancak, SP için uygun tedavi dozu için ortak bir data yoktur. Lokal kontrol için optimal doz hastalýðýn yerine ve boyutuna baðlý olarak 30 ve 50 Gy arasýnda deðiþmektedir. Bu derlemede lokal kontrol için minimal RT dozu tanýmlanmaya çalýþýlmýþtýr.

Plasma-cell neoplasms are classically categorized into four groups as: multiple myeloma (MM), plasma-cell leukemias, solitary plasmacytomas (SP) of the bone (SPB), and extramedullary plasmacytomas (EMP). These tumors may be described as localized or diffuse in presentation. Localized plasma-cell neoplasms are rare, and include SP of the skeletal system, accounting for 2-5% of all plasma-cell neoplasms, and EMP of soft tissue, accounting for approximately 3% of all such neoplasms. SP is defined as a solitary mass of neoplastic plasma cells either in the bone marrow or in various soft tissue sites. There appears to be a continuum in which SP often progresses to MM. The main treatment modality for SP is radiation therapy (RT). However, there are no conclusive data in the literature on the optimal RT dose for SP. This review describes the interrelationship of plasma-cell neoplasms, and attempts to determine the minimal RT dose required to obtain local control.

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AMAÇ: Amifostin (AMI) doksorubisinin yol açtýðý kardiyotoksisiteden korunmada çeþitli deneysel ve bir kaç klinik çalýþmada kullanýlmýþtýr. Bu çalýþmanýn amacý Sýçan kalbindeki lipid peroksidasyonu, koruyucu enzimler ve mitoksantronun (MITO) yol açtýðý akut kardiyotoksisite üzerinde AMI’nin etkilerini biyokimyasal ve histopatolojik incelemeler ile araþtýrmaktý.
YÖNTEMLER: Her bir grupta 6 sýçan olmak üzere 36 sýçan 6 gruba bölündü. Ýntraperitoneal (ip) olarak kontrol grubuna serum fizyolojik ve AMI grubuna 200 mg/kg AMI verildi. MITO 2.5 ve 5 gruplarýndaki sýçanlar ip MITO 2.5 ve 5 mg/kg aldý. MITO 2.5+AMI ve MITO 5+AMI gruplarýnda ayný dozlarda MITO’dan 30 dk önce 200 mg/kg AMI uygulandý.
BULGULAR: Kretainin fosfokinaz-miyokardial bant ve kardiyak troponin T gibi kardiyak enzimlerin düzeyi deðiþiklik göstermedi. MITO gruplarýndaki malondialdehid (MDA) düzeyleri kontrollere kýyasla yüksekti. MITO ve MITO+AMI gruplarýndaki katalaz ve glutatyon düzeyleri kontrollerden yüksekti. MITO+AMI ve kontroller arasýnda süperoksit dismutaz ve glutatyon peroksidaz düzeyleri bakýmýndan fark yoktu. Kalsiyum birikimi saptanmadý. Fibrozis, dejenerasyon ve inflamasyon skorlarý MITO+AMI gruplarýnda daha düþüktü.
SONUÇ: MITO lipid peroksidasyonu ve miyokardiyal zaralanmaya neden olurken miyokardiyum bu zaralanmadan korunmak için katalaz ve GSH düzeylerini arttýrmaktadýr. AMI miyokardiyal zararlanma ve lipid peroksidasyonu azaltarak MITO’nun yol açtýðý akut kardiyotoksisiteye karþý koruyucu olabilmektedir.

OBJECTIVE: Amifostine (AMI) has been used for the prevention of doxorubicin-induced cardiotoxicity in several experimental and a few clinical studies. The aim of this study was to investigate the effects of AMI on lipid peroxidation, protective enzymes, and mitoxantrone (MITO)-induced acute cardiotoxicity in the rat heart using biochemical tests and histopathological examinations.
METHODS: Thirty-six rats were divided into six groups (n=6 in each). Control rats were given intraperitoneal (i.p.) serum saline and AMI group rats were given 200 mg/kg AMI i.p. Rats received MITO-2.5 and 5 mg/kg i.p. in the MITO-2.5 and MITO-5 groups. AMI 200 mg/kg i.p. was administered 30 min. before the same doses of MITO in the MITO-2.5+AMI and MITO-5+AMI groups.
RESULTS: The levels of cardiac enzymes such as creatinine phosphokinase-myocardial band and cardiac troponin T did not change. Malondialdehyde (MDA) levels increased in MITO groups compared to controls. Catalase and glutathione (GSH) levels in the MITO and MITO+AMI groups were higher than in controls. Superoxide dismutase and glutathione peroxidase levels were not different between MITO groups and controls. There was no difference in MDA levels between MITO+AMI groups and controls. Calcium deposition was not detected. The scores of fibrosis, apoptosis, inflammation, and degeneration in MITO groups were higher than in controls. The scores of fibrosis, degeneration and inflammation in MITO+AMI groups were lower.
CONCLUSION: MITO caused lipid peroxidation and myocardial damage, and the myocardium increased catalase and GSH levels to prevent this damage. AMI can protect against MITO-induced acute cardiotoxicity, decreasing myocardial damage and lipid peroxidation.

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AMAÇ: Kanama durdurucu olarak bilinen Ankaferd (AKD); Thymus vulgaris, Glycyrrhiza glabra, Vitis vinifera, Alpinia officinarum ve Urtica dioica bitki özlerinin özel bir karýþýmýdýr. AKD nin temel etki mekanizmasý, hayati eritrosit agregasyon odaklarý olan enkapsüle protein aðý formasyonunu saðlayarak gerçekleþmektedir. AKD kan hücreleri ve özellikle eritrositler ile oluþumunu indüklediði protein aðý sayesinde birincil ve ikincil haemostatik sistem üzerine etkisini koagülasyon faktörlerini birebir hasarlamadan gerçekleþtirir.

YÖNTEMLER: ABS nin hemostaz üzerindeki etki mekanizmasýný anlamak için 2D jel elektroforez ve kütle spektrometre yöntemleri kullanýlarak proteomik analizleri yapýldý.
BULGULAR: Ankaferd kanama durdurucu içeriðinde tanýmlanan bitkisel proteinler: NADP-baðýmlý malik enzim, Ribuloz bisfosfatkarboksilaz büyük zinciri, MturazK, ATPsentaz altünitesi beta, ATPsentaz altünitesi-alfa, Chalcon flavonon isomeraz-1, Chalcon-flavonon izomeraz 2 ve Aktin-depolimerizasyon faktördür. Ayrýca Ankaferd kapsamýnda koagülasyon için oldukça önemli farklý insan proteinleri benzerleri de tanýmlanmýþtýr, bu proteinler arasýnda; ATP sentaz, musin16 (CD164-sialomucin-benzer-2 protein), helezonal kangal taþýyan protein-141, hypotetik protein LOC283638 izoform 1, hypothetik protein LOC283638 izoform 2, dinaktin 5, Kompleks 1 intermadia iliþikli protein 30, mitokondrial protein, NADH dehidrogenaz (Ubiquinone) 1 alpha altkompleks, TP sentaz H+ taþýyýcý protein, mitokondrial aktin baðlayýcý protein 1, LIM kangal ve aktin baðlayýcý alt ünite 1 izoform a, LIM kangal ve aktin baðlayýcý alt ünite 1 izoform b, Spectrin alpha non eritrotik 1, Prolactin releasing hormone reseptör, Utrophin, tet onkogen aile üyesi 2 izoform b, Protein fosfotaz 1 regulatory altünit 12A, NIMA -iliþkili kinaz, ATP-baðlayýcý protein C12, malik enzim 1, Mitochondrial NADP(+) baðýmlý malik enzim 3, ME2 protein, Nuclear faktör 1B tipi, Abihidrolaz kangal taþýyýcý protein 12B, E3 SUMO-protein ligaz PIAS2, Alpha-1,2-glucosyltransferase ALG10-A, Cofilin, 18 kDa fosfoprotein, p18, Aktin-depolymerizing faktör, ADF, Twinfilin-1, Ankirin tekrarlayan ve FYVE kangalý içeren protein 1, Usherin öncü proteini, Urotensin II reseptör yer almaktadýr.
SONUÇ: Proteomik analizler sonucu elde edilen proteinler Ankaferdin hemostatik, yara iyileþtirme ve anti-inflamatuvar etkilerinin araþtýrýlmasýna ýþýk tutacak ve açýklayýcý olacak niteliktedir.

OBJECTIVE: Ankaferd® Blood Stopper (ABS) comprises a standardized mixture of the plants Thymus vulgaris, Glycyrrhiza glabra, Vitis vinifera, Alpinia officinarum, and Urtica dioica. The basic mechanism of action for ABS is the formation of an encapsulated protein network that provides focal points for vital erythrocyte aggregation. ABS–induced protein network formation with blood cells, particularly erythrocytes, covers the primary and secondary hemostatic system without disturbing individual coagulation factors.
METHODS: To understand the effect mechanisms of ABS on hemostasis, a proteomic analysis using 2D gel electrophoresis and mass spectrometer was performed.
RESULTS: Proteins of plant origin in Ankaferd® were NADP-dependent-malic enzyme, ribulose bisphosphate-carboxylase-large chain, maturase K, ATP synthase subunit-beta, ATP synthase subunit-alpha, chalcone-flavanone isomerase-1, chalcone-flavanone isomerase-2, and actin-depolymerizing factor. Furthermore, functional proteomic studies revealed that proteins resembling human peptides have been detected within Ankaferd®, including ATP synthase, mucin-16 (CD164 sialomucin-like 2 protein), coiled-coil domain containing 141 hypothetical protein LOC283638 isoform 1, hypothetical protein LOC283638 isoform 2, dynactin 5, complex I intermediate-associated protein 30, mitochondrial, NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, TP synthase, H+ transporting, mitochondrial actin binding 1 isoform, LIM domain and actin binding 1 isoform a, LIM domain and actin binding 1 isoform b, spectrin alpha non erythrocytic 1, prolactin releasing hormone receptor, utrophin, tet oncogene family member 2 isoform b, protein phosphatase 1 regulatory subunit 12A, NIMA (never in mitosis gene a)-related kinase, ATP-binding cassette protein C12, Homo sapiens malic enzyme 1, mitochondrial NADP(+)-dependent malic enzyme 3, ME2 protein, nuclear factor 1 B-type, abhydrolase domain-containing protein 12B, E3 SUMO-protein ligase PIAS2, alpha-1, 2-glucosyltransferase ALG10-A, cofilin, non-muscle isoform, 18 kDa phosphoprotein, p18, actin-depolymerizing factor (ADF), twinfilin-1, ankyrin repeat and FYVE domain-containing protein 1, usherin precursor, urotensin II receptor, interleukin 4, and midkine.
CONCLUSION: Proteomic analysis of Ankaferd® represents a true basis for the upcoming Ankaferd® studies focusing on its wound healing, hemostatic, anti-infective, antineoplastic, and preservative biological actions.

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AMAÇ: Sitotoksik T lenfosit antijen-4 (CTLA-4) ifadesi T lenfositlerde gerçekleþir ve T hücre cevabýný engeller. Hayvan modellerinde CTLA-4 yokluðunun, dokularýn poliklonal çoðalan lenfositler tarafýndan yoðun infiltrasyonu nedeni ile, ölümcül olduðu gösterilmiþtir. Farklý otoimmün hastalýklar ile iliþkilendirilmiþ CTLA-4 A49G polimorfizminin, CTLA-4 molekülünün inhibitör fonksiyonunu azalttýðý düþünülmektedir.
YÖNTEMLER: Bu çalýþmada otoimmun hemolitik anemi (OIHA) hastasý 46 kiþi, immun trombositopenik purpura (ITP) hastasý 62 kiþi ve 150 saðlýklý kontrol bireyinde CTLA-4 A49G polimorfizmi çalýþýlmýþtýr.
BULGULAR: ITP ve OIHA hastalarýndan oluþan iki grupta da saðlýklý kontrol bireyleri ile karþýlaþtýrýldýðýnda benzer allel frekanslarý ve genotip daðýlýmlarý saptanmýþtýr. Alt grup analizi gerçekleþtirildiðinde ise hem OIHA hem de KLL hastalýðýna sahip 4 bireyin hepsinin polimorfizme sahip olduðu gösterilmiþtir (3 AG, 1 GG). Risk alleli olan G OIHA, ÝTP ve kontrol grubunda incelendiðinde istatistik olarak anlamlý bir farklýlýk saptanmamýþtýr.
SONUÇ: Bu verilerin ýþýðýnda CTLA-4 A49G polimorfizminin lenfoproliferatif hastalýklarýn patogenezine bir katkýsýnýn olmadýðý veya lenfoproliferatif hastalýða sahip bireylerde otoimmün komplikasyonlarýn geliþmesi açýsýndan risk oluþturmadýðý sonucuna varýlmýþtýr.

OBJECTIVE: The cytotoxic T lymphocyte associated antigen-4 (CTLA-4) is expressed on T lymphocytes, and inhibits the T-cell responses. In animal models, it has been shown that complete CTLA-4 deficiency was lethal due to massive infiltration of tissues by polyclonally proliferating lymphocytes. CTLA-4 A49G polymorphism, which has been suggested to reduce the inhibitory function of the CTLA-4 molecule, was found to be associated with various autoimmune diseases in recent studies.
METHODS: In this study, we evaluated the frequency of CTLA-4 A49G polymorphism in 46 patients with autoimmune hemolytic anemia (AIHA), 62 patients with immune thrombocytopenic purpura (ITP), and 150 healthy individuals.
RESULTS: Allele frequencies and genotype distributions were similar in both ITP and AIHA patients compared to healthy individuals. In subgroup analysis, however, we found that in chronic lymphocytic leukemia (CLL) patients with AIHA (n=4), all patients had CTLA-4 A49G polymorphism (3 had AG, 1 had GG). There was no significant statistical association between G allele and systemic lupus erythematosus (SLE) or AIHA.
CONCLUSION: These data suggest that CTLA-4 A49G polymorphism does not contribute to the pathogenesis of lymphoproliferative diseases itself, nor does it increase the risk of autoimmune complications in patients with lymphoproliferative disease.

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AMAÇ: Esansiyel trombositemi (ET) platelet sayýsýnýn artmasý ve yüksek tromboz riski ile karakterize kronik bir myeloproliferatif bozukluktur. Ex vivo veriler tromboz riskine uygun olarak artan platelet reaktivitesini öne sürerken in vitro testler sýklýkla platelet aktivitesinde azalma tespit etmektedir. Bu çalýþmanýn amacý ET-hastalarýnda az sayýda çalýþmaya dahil edilmiþ bir platelet fonksiyonu konusu olan ET-plateletleri adezyonunun in vitro incelenmesidir.
YÖNTEMLER: Çalýþmaya 30 ET hastasý ile 14 saðlýklý kontrol dahil edilmiþtir. Statik platelet adezyonu tayini ile platelet adezyonu ölçülmüþtür.
BULGULAR: Temel bulgu ET plateletlerinin, in vitro adezyon indüklenmiþ uyaranlar ile kontrol plateletlerinden daha kolay aktive olduðu olmuþtur. Bu durum özellikle yaþlý hastalarda ve adenosin 5-difosfat ya da ristosetin eklenerek kombine edilmiþ kolajen ya da fibrinojen yüzeyler gibi çoklu uyaran kullanýldýðýnda barizdir. Bu gibi çoklu uyaran hastalarýn yaklaþýk %50’sinde kontrol deðeri + 2 standart sapmanýn üzerinde adezyon sonucunu vermiþtir.
SONUÇ: Bulgular ET’de artan platelet aktivitesi konseptine uygun olsa da diðer in vitro çalýþmalarýn tersinedir. Adezyon tayininde koþullarýn kronik platelet aktivasyonu varlýðý ile ilgili olarak in vitro durumu taklit edebileceðini ileri sürüyoruz.

OBJECTIVE: Essential thrombocythemia (ET) is a chronic myeloproliferative disorder characterized by elevated platelet counts and increased risk of thrombosis. Ex vivo data suggest increased platelet reactivity in agreement with the increased thrombosis risk, while in vitro tests often detect decreased platelet activity. The present study aimed to investigate adhesion of ET-platelets in vitro, which is an aspect of platelet function that has been addressed in only a few studies on ET patients.
METHODS: The study included 30 ET patients and 14 healthy controls. Platelet adhesion was measured with a static platelet adhesion assay.
RESULTS: The main finding was that ET-platelets were more readily activated by adhesion-inducing stimuli in vitro than control platelets. This was particularly evident in elderly patients and when using multiple stimuli, such as surfaces of collagen or fibrinogen combined with addition of adenosine 5’-diphosphate or ristocetin. Such multiple stimuli resulted in adhesion above the control mean +2 standard deviations for approximately 50% of the patients.
CONCLUSION: The results are in accordance with the concept of increased platelet activity in ET, but opposite to most other in vitro studies. We suggest that the conditions in the adhesion assay might mimic the in vivo situation regarding the presence of chronic platelet activation.

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AMAÇ: Allogeneik hematopoietik kök hücre nakli (Allo-HKHN) sonrasý geliþen sklerodermatoz graft-versus-host hastalýðýnýn (GVHH) klinik özelliklerinin deðerlendirilmesi amaçlanmýþtýr.
YÖNTEMLER: Allo-HKHN yapýlan 423 hasta retrospektif olarak analiz edilmiþtir. Olgularýn yaþ, cinsiyet, transplantasyon öncesi tanýlarý, hazýrlýk rejimleri, GVHH proflaksileri, akut GVHH ve/veya kronik likenoid ve kronik sistemik GVHH mevcudiyeti ve geliþen sklerodermatoz GVHH’nýn klinik özellikleri deðerlendirilmiþtir.
BULGULAR: Sklerotik lezyonlar 22 hastada ortalama 752±647 gün (ortanca 480) sonra geliþmiþtir. Akut GVHH 17 hastada geliþirken, bunlarýn 2’sinde karaciðer, 2’sinde gastrointestinal sistem ve 14’ünde deri tutulumu gözlenmiþtir. Yaygýn kronik GVHH (karaciðer, akciðer, deri ve oral mukoza) 12 hastada geliþmiþtir. Skleroz 19 hastada (%86.4) jeneralize, 3 hastada (%13.6) lokalizeydi. Jeneralize sklerodermatoz deðiþiklikler izlenen 19 hastanýn 8’inde (%36.4) leopar derisi görünümü mevcuttu. Olgularýn çoðunda sklerotik lezyonlar gövdede yerleþirken, ekstremitelerin distali etkilenmemiþtir. Olgularýn 8’i likenoid GVHH’dan sklerodermatoz GVHH’ye dönüþürken, 2’sinde her iki faz bir arada ve 12’sinde sklerodermatoz GVHH denovo olarak geliþmiþtir. Beþ olgu transplantasyonla iliþkili geç komplikasyonlar nedeniyle vefat etmiþtir.
SONUÇ: Sklerodermatoz GVHH geç baþlangýçlý ve hastalar için oldukça sýkýntý oluþturabilen bir tablodur. Sklerodermanýn aksine akral tutulum nadiren görülür. Lezyonlar hastalýk seyri boyunca kaybolmamakla birlikte pek çok olguda prognoz iyidir.

OBJECTIVE: We aimed to evaluate the clinical features of sclerodermatous chronic graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (AHSCT).
METHODS: We retrospectively analyzed 423 patients who underwent AHSCT. We assessed age, sex, pre-transplant diagnosis, conditioning regimen, GVHD prophylaxis, and occurrence of acute GVHD (aGVHD), chronic lichenoid and chronic systemic GVHD, and clinical properties of sclerodermatous GVHD.
RESULTS: Sclerotic skin lesions developed in 22 patients after a mean of 752±647 days (median 480). aGVHD appeared in 17 patients, with hepatic involvement in 2, gastrointestinal tract involvement in 2 and skin involvement in 13 of these patients. Extensive chronic GVHD (liver, pulmonary, skin and oral mucosa) developed in 12 patients. Sclerosis was generalized in 19 patients (86.4%) and localized in 3 patients (13.6%). Leopard skin eruption appeared in 8 (36.4%) of the 19 patients with generalized sclerodermatous changes. In most cases, sclerotic lesions appeared on the trunk, and distal parts of the extremities were spared. Eight patients (36.4%) progressed from lichenoid to sclerodermatous lesions, 2 (9.1%) with lichenoid and sclerodermatous phases together and 12 (55.5%) with de novo sclerodermatous lesions. Five patients died because of late transplant-related complications.
CONCLUSION: Sclerodermatous GVHD has a late onset and may be quite disabling. Unlike scleroderma, acral involvement is seen rarely. Although most lesions do not disappear in the course of the disease, most patients have a good prognosis.

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AMAÇ: Klopidogrelin trombositler üzerinde yeni in vitro etkilerini tayin etmek: Malondialdehit, glutatyon, nitrit, aggregasyon cevabý, P-selektin, fibrinojen, apolipoprotein A1, apolipoprotein B ve fosfatidilserin ekspresyonlarý.
YÖNTEMLER: Saðlýklý (n: 9) ve hiperlipidemik (n: 9) olgulardan trombositler elde edildi. Klopidogrelli ve klopidogrelsiz trombositlerde P-selektin, fibrinojen, apolipoprotein A1, apolipoprotein B ve fosfatidilserin ekspresyonlarý flow sitometre ile tayin edildi. Malondialdehit, glutatyon, aggregasyon ve nitrit seviyeleri de tayin edildi.
BULGULAR: Klopidogrel yokluðunda, hiperlipidemililerde kontrollere göre trombosit agregasyonu, malondialdehit, P-Selektin, fibrinojen ve fosfatidilserin ekspresyonunun baþlangýç deðerleri yüksek; bununla birlikte nitrit, apolipoprotein A1 ve apolipoprotein B ekspresyonlarýnýnki ise daha düþüktü. Her iki grupta, klopidogrel anlamlý düzeyde aggregasyonu ve fibrinojen ekspresyonunu azalttý, fakat nitrit seviyelerini artýrdý. Klopidogrel sadece hiperlipidemililerde P-selektin ve fosfatidilserin ekspresyonunu ve malondialdehiti azalttý ancak apolipoprotein A1 ve apolipoprotein B ekspresyonlarýný artýrdý.
SONUÇ: Görüldüðü üzere klopidogrel bazý yeni in vitro antiplatelet etkilere sahiptir. Bu çalýþma, trombosit fonksiyonlarý üzerinde klopidogrelin etkilerine yeni bir bakýþ açýsý saðlayan temel in vitro bir çalýþmadýr.

OBJECTIVE: We aimed to detect novel in vitro effects of clopidogrel on platelets by assessment of the following parameters: malondialdehyde, glutathione, nitrite, aggregation response, and expressions of P-selectin, fibrinogen, apolipoprotein A1, apolipoprotein B, and phosphatidylserine.
METHODS: Platelets were obtained from healthy (n: 9) and hyperlipidemic (n: 9) volunteers. Expressions of P-selectin, fibrinogen, apolipoproteins A1/B and phosphatidylserine with and without clopidogrel were assayed by flow cytometry. Malondialdehyde, glutathione, aggregation and nitrite levels were also assayed.
RESULTS: Without clopidogrel, the baseline values of platelet aggregation, malondialdehyde, and expressions of P-selectin, fibrinogen and phosphatidylserine were significantly higher, whereas nitrite and expression of apolipoproteins A1/B were significantly lower in hyperlipidemics than in the healthy group. In both groups, clopidogrel significantly reduced aggregation and expression of fibrinogen, but it elevated nitrite levels. Clopidogrel significantly decreased P-selectin and phosphatidylserine expression and malondialdehyde but increased expressions of apolipoproteins A1/B only in hyperlipidemics.
CONCLUSION: It seems that clopidogrel has some new in vitro antiplatelet effects. The present study is a basic in vitro study to suggest new insights into the effects of clopidogrel on platelet functions.

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Multipl miyelomda (MM) akciðer enfeksiyonlarý ve göðüs kafesi kemiklerinde tutuluma baðlý geliþen pulmoner komplikasyonlar sýkça görülmesine karþýn, akciðer parankim tutulumu oldukça nadirdir. Yapýlan klinik çalýþmalarda akciðer parankim tutulumunun kötü prognoz ile iliþkisi bulunmuþtur. Burada akciðer parankim tutulumuna olumsuz prognostik sitogenetik belirleyicilerin eþlik ettiði bir MM olgusu sunulmaktadýr.
Öksürük þikayeti ile baþvuran 62 yaþýnda erkek olguya çekilen toraks tomografisinde; sað orta lob düzeyinde heterojen hipodens kitle görünümü saptandý. Bronkoskopik biyopsi yapýldý. Patolojik incelemede immünhistokimyasal olarak kappa ile diffüz boyanma gösteren plazma hücre infiltrasyonu saptandý. Kemik iliði biyopsisinde plazma hücre infiltrasyonu görüldü. Konvansiyonel sitogenetik incelemede hipodiploidi saptandý. Fluorescence in situ hybridization ile yapýlan sitogenetik incelemede delesyon (13q) tespit edildi. Sonuç olarak, akciðerde kitle lezyonu ile baþvuran ve MM tanýsý alan hastalarda ayýrýcý tanýda MM a baðlý plazma hücre infiltrasyonu da düþünülmelidir. Bu olgular toplam sað kalýmlarý düþük olduðundan, yüksek doz tedavi gibi daha agresif tedavi yaklaþýmlarýna hýzla yönlendirilmelidir.

Although pulmonary complications developing secondary to lung infections and involvement in ribs occur frequently in multiple myeloma (MM), involvement of the lung parenchyma is quite rare. In clinical studies, the involvement of lung parenchyma has been found to be associated with unfavorable prognosis. Here, a MM case in whom involvement of lung parenchyma was accompanied by unfavorable prognostic cytogenetic markers is presented.
A 62-year-old male presented with complaint of cough, and heterogeneous hypodense mass was detected in thorax computerized tomography. The patient underwent bronchoscopic biopsy. Pathological examination revealed diffuse plasma cell infiltration staining with kappa immunohistochemically. In bone marrow biopsy, plasma cell infiltration was observed. In conventional cytogenetic examination, hypodiploidy was established. In cytogenetic examination carried out with fluorescence in situ hybridization, deletion (13q) was determined. In conclusion, in patients diagnosed with MM and presenting with pulmonary mass lesion, lung involvement associated with plasma cell infiltration should also be considered in the differential diagnosis. As overall survival is low in these cases, more aggressive treatment approaches such as high-dose treatment should be immediately considered.

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Kronik idiopatik myelofibrozis, kronik myeloproliferatif hastalýklardandýr ve splenomegali, myeloid metaplazi ve reaktif kemik iliði fibrozisi ile karakterizedir. Bu hastalýklarýn tanýsýnda, özellikle de reaktif durumlarý ekarte etmek için kemik iliðinden kromozom analizi oldukça önemli bir araçtýr. Ýdiopatik myelofibrozis, bu grup içerisinde en yüksek klonal sitogenetik anomali sýklýðýna sahiptir (%30-75). Trizomi 1q, 20q-, 13q- ve +8 en sýk gözlenen anomalilerdendir. Bu yazýmýzda masif splenomegali ile baþvuran 66 yaþýnda erkek olgunun bulgularý sunulmaktadýr. Olgunun kemik iliði biyopsisinde kronik myeloproliferatif deðiþiklikler ve dismegakaryopoez saptandý. Hidroksiüre tedavisi, splenik radyoterapi ve çok sayýda transfüzyon alan hastanýn kliniði ilerleyen aylarda kötüleþti. Yapýlan ikinci kemik iliði biyopsisisinde myelofibrozis gözlendi. Bu örneðin sitogenetik incelemesi sonucu daha önce tanýmlanmamýþ üç yollu bir translokasyon ve 9. kromozomda delesyon saptandý. Karyotipi 46,XY,del(9)(q22q34),t(8;17;21)(q22;q21;q22)[23]/46,XY[2] olarak belirlendi. Olgu kýsa süre sonra kaybedildi.

Chronic idiopathic myelofibrosis is a myeloproliferative disorder characterized by splenomegaly, myeloid metaplasia and reactive bone marrow fibrosis. Karyotype analysis of the bone marrow is an integral part of the diagnosis, especially as a discriminative tool in ruling out reactive conditions. The frequency of clonal cytogenetic anomalies in this disease is the highest among its group, varying between 30 and 75%. Among these, trisomy 1q, 20q-, 13q- and +8 are the most common aberrations. Here we report a 66-year-old male patient whose bone marrow biopsy revealed signs of chronic myeloproliferative changes and dysmegakaryopoiesis. He was administered hydroxyurea treatment, splenic radiotherapy and multiple transfusions. The patient worsened in the following months and the second bone marrow biopsy revealed myelofibrosis. Cytogenetic analysis of this bone marrow sample revealed a complex karyotype reported to be 46,XY,del(9)(q22q34),t(8;17;21)(q22;q21;q22)[23]/46,XY[2], with a previously undefined three-way translocation and deletion in chromosome 9. The patient died shortly thereafter.

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Çocukluk çaðý akut lenfoblastik lösemi (ALL) tedavisi boyunca geçirilebilecek kabakulak enfeksiyonunun, virusun düþük sitopatolojik etkileri nedeniyle, kýzamýk ve su çiçeði enfeksiyonlarýnýn aksine daha hafif seyrettiðine dair raporlar bulunmaktadýr. Daha önce kabakulak aþýsý yapýlmýþ olan üç yaþýndaki kýz hasta iki taraflý parotid bezinde þiþlik ve daktilit nedeniyle baþvurduðunda kabakulak IgM pozitifliði saptanmýþ ve ileri incelemesinde bilateral parotid infiltrasyonunun eþlik ettiði ALL tanýsý konulmuþtur. Çocukluk çaðýnýn atipik seyirli tüm hastalýklarýnda akut lösemi akýlda tutulmalýdýr. Ayný zamanda tedavi sýrasýnda geçirilen kabakulak enfeksyonlarýna benzer þekilde, ALL tanýsý anýnda eþlik eden kabakulak enfeksiyonu da hafif seyretmektedir.

Mumps infection during the course of childhood acute lymphoblastic leukemia (ALL) treatment has been reported to have a mild course and this was related to the intrinsic low cytopathological effect of the virus, contrasting with the severe course of measles and Varicella zoster virus infections in immunocompromised patients. Herein, we present a three-year-old girl, who was previously vaccinated against mumps infection, admitted with bilateral parotid swelling, dactylitis and serum immunoglobulin M positivity for mumps infection and diagnosed to have ALL with bilateral persistent parotid involvement, inconsistent with mumps infection. Acute leukemia should be suspected during the atypical course of any disease during childhood. Besides, mumps infection at presentation of ALL, as similar to infection emerging during the period of the leukemia treatment, has a mild course.

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Hb Tunis [beta124(H2)Pro>Ser] ilk kez 1988 yýlýnda Tunus’da bildirilmiþtir. Bu hemoglobin varyantý alkali ve asit ortamda yapýlan standart elektroforez yöntemleri ile ayrýlamamaktadýr. Ýzoelektrik odaklama elektroforezinde (IEF-isoelectric focusing) ise Hb A’dan biraz daha hýzlý bir davranýþ ortaya koymaktadýr. Hb Tunis normal stabilite ve normal oksijen afinitesine sahip olup herhangi bir klinik belirti vermemektedir. Premarital tarama çalýþmasýnda saptanan heterozigot Hb Tunis olgumuz, Türkiye ilk kez bildirilmektedir. Bu hemoglobin varyantý HPLC yöntemi ile ayrýlabilmiþ ve DNA dizi analizi ile doðrulanmýþtýr. Bu çalýþmamýzda ayrýca, hemoglobinopati kontrol programlarýnýn baþarýlý biçimde uygulanabilmesi için yöresel düzeyde disiplinler arasý iþbirliðinin önemini vurgulamaktayýz.

Hb Tunis [beta124(H2)Pro>Ser] was reported from Tunisia in 1988. This hemoglobin variant was detected by isoelectric focusing moving just ahead of Hb A. It cannot be identified by standard hemoglobin electrophoresis due to its similar mobility to Hb A. It has normal stability and oxygen affinity and does not produce any clinical symptoms. Here, we report a heterozygous Hb Tunis [beta124(H2)Pro>Ser] case discovered for the first time in Turkey in a premarital screening program. This hemoglobin variant can be identified with high performance liquid chromatography analysis confirmed with DNA sequencing. We emphasize in our study the importance of an interdisciplinary collaborative study at the provincial basis for the success of the hemoglobinopathy control program.

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