Akut Promyelositik Lösemili (APL) çocuklarda, lösemik hücrenin normal hücreye dönüþünü (farklýlaþma) saðlayan all-trans retinioik asidin (ATRA) kullanýlmasý ile tedavi sonuçlarýnda önemli ilerlemeler elde edilmiþtir. Bu nedenle APL dýþýnda diðer akut myeloblastik lösemi (AML) subtiplerinde lösemik hücrelerin farklýlaþmasýný saðlayan ilaçlarýn kullanýlmsý, bu hastalar için de önemli ve ümit verici bir tedavi yaklaþýmý olacaktýr. Fare myeloid lösemik hücrelerin steroid ile farklýlaþmasýnýn saðlanabileceðini gösteren in vitro çalýþmalara dayanarak, biz de, literatürde ilk defa olmak üzere, AML’li çocuklarýn farklý subtiplerinde (AML M1,-M2,-M3,-M4,-M7), kýsa süreli yüksek doz metilprednizolon (YDMP) tedavisi ile lösemik hücrelerin normal hücrelere dönebileceðini gösterdik. Ayrýca YDMP tedavisi, lösemik hücreden dönüþen veya direkt olarak myeloid lösemik hücrelerin apoptotik ölümüne neden olmaktadýr.
Diðer lösemi ilaçlarý kullanýlmaksýzýn YDMP, hastalara verildikten kýsa süre sonra, kilinik bulgularda düzelme, periferik kan ve kemik iliði lösemi hücrelerinde önemli bir azalma ve kemik iliði dýþýndaki lösemi hücre kitlesinin hýzla küçülmesine neden olmaktadýr. YDMP’ nun sitotoksik tedavi protokollarýna ilavesi ile hastalarýn remisyon yüzdeleri artmýþ ve hastalýksýz yaþam süreleri uzamýþtýr. Bu nedenlerle, YDMP ile yapýlacak kilinik çalýþmalarýn, AML ve myelodisplastik sendromlu çocuklarýn tedavilerinden alýnacak sonuçlara önemli katkýsý olacaðý kanýsýndayýz.

Differentiation-inducing therapy with all-trans retinoic acid significantly improved the outcome in children with acute promyelocytic leukemia (APL). Therefore, use of agents that induce differentiation of leukemic cells in non-APL children appears to be a highly promising therapeutic approach. Based on the experimental studies in mice, we have shown that short-course high-dose methylprednisolone (HDMP) treatment can induce terminal differentiation of leukemic cells in children with various subtypes of acute myeloblastic leukemia (AML-M1,-M2,-M3,-M4,-M7). It has also been shown to induce apoptosis of myeloid leukemic cells with or without differentiation. Administration of HDMP as a single agent resulted in a rapid clinical improvement, a marked decrease in blast cells in both peripheral blood and bone marrow and dramatic decreases in the size of extramedullary leukemic mass in children with AML and myelodysplastic syndrome (MDS). Addition of HDMP to cytotoxic chemotherapy regimens increased the remission rate and improved the outcome in these children. Future clinical trials with HDMP would contribute to further improvements in the treatment results in these children.

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AMAÇ: Talasemi major (TM) hastalarýnda hematopoetik kök hücre transplantasyonu (HKHT) sonrasýnda stabil karýþýk-tip kimerizm ile kür mümkündür fakat rejeksiyonla sonuçlanabilir. TM tanýsýyla yapýlmýþ 28 HKHT karýþýk-tip kimerizm seyri, risk faktörleri ve sonuçlarýný deðerlendirmek üzere retrospektif olarak gözden geçirildi.
YÖNTEMLER: Ýzlem süresi ortanca 1669 gündü (811-3576 gün). Kimerizm alýcý-verici cinsiyet uyumuna göre FISH veya multipleks PCR yöntemiyle bakýldý.
BULGULAR: Primer rejeksiyon, stabil karýþýk tip kimerizm ve tam verici tip kimerizm sýrasýyla % 3.6, % 17.8 ve % 78.6 idi. 4/5 hastada % 14 kadar düþük verici kimerizmine raðmen talasemi taþýyýcýlýðý kliniði vardý. Sadece 1 hastada HKHT sonrasý 2.5 yýl sonra eritrosit ihtiyacý oldu.
SONUÇ: Stabil karýþýk-tip kimerizm, talasemi major hastalarýnda kür saðlayabilir ve bu hastalar için rejeksiyonu önceden gösteren bir belirteç bulunana kadar hastalarýn kliniði, herhangi bir müdahale için belirleyici olmalýdýr.

OBJECTIVE: Stable mixed chimerism (MC) may result in cure for thalassemia major patients following hematopoietic stem cell transplantation (HSCT), but rejection can occur. Twenty-eight HSCTs for thalassemia major were reviewed retrospectively to evaluate the clinical course of MC with possible risk factors and predictors of outcome, with a median follow-up of 1669 days (811-3576 days).
METHODS: Chimerism was detected by fluorescence in situ hybridization (FISH) or multiplex polymerase chain reaction depending on the sex match between the donor and the recipient.
RESULTS: Primary rejection, stable MC and full donor chimerism was detected in 3.6%, 17.8% and 78.6% of patients, respectively. Clinically, 4/5 patients with stable MC had thalassemia trait with donor chimerism as low as 14%. One patient was started on pRBC transfusions at 2.5 years postHSCT.
CONCLUSION: Stable MC can result in cure for thalassemia major patients. The clinical picture remains as the best guide for intervention until a more reliable predictor is available.

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AMAÇ: Thrombotic diseases are caused by genetic and environmental factors. There are a number of well-characterized genetic defects that lead to increased risk of thrombosis. Results from previous studies have indicated that FXII is involved in the pathogenesis of thrombophilic diseases. However, the results in this regard are highly controversial. One of the most important determinants of Plasma FXII level is 46CgT polymorphism in the FXII gene. In the present study, the risk of thrombophilic diseases related to this polymorphism was investigated in a case-control study.
YÖNTEMLER: Yüz altmýþ denek incelenmiþtir: 120 hastaya trombofili (96’sý tromboembolizm, 24’ü arteriyel tromboz) tanýsý konmuþ olup 40 hasta yaþ ve cinsiyet açýsýndan eþleþtirilmiþtir. Her bir denek için, FXII aktivite düzeyi, FXII’den yoksun plazma ile tek adýmlý pýhtýlaþma testi kullanarak ölçülmüþ ve 46CγT polimorfizmi, (RFLP) yöntemi ile genotiplenmiþtir.
BULGULAR: 46 CγT polimorfizmi için farklý genotipleri olan bireylerin FXII aktivite düzeylerinde anlamlý farklýlýklar sergilediðine dair önceki gözlem, bu çalýþmada doðrulanmýþtýr. Daha da önemlisi, ≤%68 olan FXII aktivitesi, 4.7 ayarlanmýþ risk oraný (OR) ile venöz tromboza yönelik yüksek risk ile iliþkilendirilmiþtir (%95 güven aralýðý [CI]: 1.03-21.1, p=0.04). Ancak bu, 5 ayarlanmýþ OR ile arteriyel tromboza yönelik bir risk faktörü deðildir (%95 CI: 0.91-27.1, p=0.09). Ct ve TT genotipte, ayarlanmýþ OR deðerleri, kontrollere kýyasla venöz trombozlu hastalar için sýrasýyla 2 (%95 CI: 0.9-4.4, p=0.11) ve 2.3 (%95 CI: 0.45-11, p=0.48) idi. Benzer þekilde, arteriyel trombozda ayarlanmýþ OR deðerleri CT genotip için 1.2 (%95 CI: 0.4 - 3.6, p=0.76) ve TT genotip için 1.8 (%95 CI: 0.2-14.9, p=0.59) idi. Böylelikle, heterozigot veya homozigot halde mutasyona uðramýþ T aleli ile venöz ya da arteriyel tromboza iliþkin yüksek risk arasýnda herhangi bir iliþki tespit edilmemiþtir.
SONUÇ: Düþük FXII aktivitesi bir risk faktörü olmamakla birlikte, yalnýzca tromboza yönelik bir risk göstergesini temsil etmektedir.

OBJECTIVE: Thrombotic diseases are caused by genetic and environmental factors. There are a number of well-characterized genetic defects that lead to increased risk of thrombosis. Results from previous studies have indicated that FXII is involved in pathogenesis of thrombophilic diseases. However, the results in this regard are highly controversial. Plasma FXII activity levels are strongly determined by a 46CγT polymorphism in the FXII gene. In the present study, the risk of thrombophilic diseases related to this polymorphism was investigated in a case-control study.
METHODS: One hundred and sixty subjects were studied: 120 patients diagnosed with thrombophilia (96 venous thromboembolism, 24 arterial thrombosis), and 40 age-gender-matched controls. For each subject, FXII activity level was measured by a one-step clotting assay with FXII-deficient plasma, and 46CγT polymorphism was genotyped using a restriction fragment length polymorphism (RFLP) method.
RESULTS: In this study, the previous observation that individuals with different genotypes for the 46 CγT polymorphism showed significant differences in Factor XII activity levels was confirmed. Most importantly, FXII activity ≤ 68% was associated with an increased risk of venous thrombosis with an adjusted OR of 4.7 (95%CI= 1.03-21.1, P=0.04). However, it was not a risk factor for arterial thrombosis with adjusted OR of 5 (95%CI= 0.91-27.1, P=0.09). In CT and TT genotype the adjusted ORs were respectively 2 (95%CI=0.9-4.4, P=0.11) and 2.3 (95%CI=0.45-11, P=0.48) for patients with venous thrombosis compared with the controls. Similarly, the adjusted ORs in arterial thrombosis were 1.2 (95%CI=0.4-3.6, P=0.76) for CT and 1.8 (95%CI=0.2-14.9, P=0.59) for TT genotype. Thus, we did not find any association of the mutated T allele in the heterozygous or homozygous state with an increased risk of both venous and arterial thrombosis.
CONCLUSION: Lower FXII activity is not a risk factor; rather, it simply represents a risk marker for thrombosis.

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AMAÇ: Çalýþmamýzda immunsupresif tedavi alýrken hastane kökenli pnömoni (HKP) geliþen hastalarda klinik baþarý oranlarýný, nötropeninin tedavi baþarýsýna olan etkilerini, mortalite ile iliþkili risk faktörlerini ve survi oranlarýný saptamayý amaçladýk.
YÖNTEMLER: Ýmmunsupresif tedavi alýrken HKP geliþen 45 eriþkin hasta prospektif olarak çalýþmaya alýndý. Transplant hastalarý ve human immunodeficiency virus (HIV)-pozitif olan hastalar çalýþmaya alýnmadý.Antibiyotik tedavisi multidisipliner olarak yönetildi. Survi analizlerinde Kaplan Meier, mortaliteyle iliþkili baðýmsýz risk faktörlerini saptamak için Cox regresyon uygulandý. Nötropeninin klinik baþarý oranlarý ile iliþkisi Chi Square yöntemiyle karþýlaþtýrýldý.
BULGULAR: Ampirik tedavi olarak 43 hastanýn 40’da (%93) antipseudomonal tedavi baþlanmasýna raðmen en sýk izole edilen etkenler Acinetobacter spp ve Escherichia coli idi. Tedavi sonu klinik baþarý oraný %65.1 idi. Sürvi oranlarý 3.,14., 42. ve 365. gün sýrasýyla % 97, 86, 58 ve 19 olarak bulundu. Üre yüksekliði [Hazard Ratio=1.01 (%95 GA: 1.00-1.02)] ve kan þekeri yüksekliði [HR=1.01 (%95 GA: 1.00-1.02)] surviyi olumsuz etkileyen baðýmsýz risk faktörleri olarak bulundu. Nötropenik olmayan (n=23) hastalarda klinik baþarý oranlarý nötropenik (n=20) olanlara göre daha yüksek bulundu (p=0.05).
SONUÇ: Ýmmunsupresif tedavi alan hastalarda geliþen HKP’lerde tedavi baþarý oranlarý düþüktür.

OBJECTIVE: The aims of this study were to determine the clinical success rates, effect of neutropenia on treatment success rates, risk factors related to mortality, and survival in patients who developed hospital-acquired pneumonia (HAP) while receiving immunosuppressive therapy.
METHODS: Forty-three adult patients receiving immunosuppressive therapy who developed HAP were included in this prospective study. Transplantation patients and human immunodeficiency virus (HIV)-positive patients were not included. Antibiotic treatment was managed by a multidisciplinary team. The Kaplan Meier method was used for the survival analysis and Cox regression was used for the identification of mortality-related independent risk factors. The relationship between neutropenia and the clinical success rate was determined using the chi-square test.
RESULTS: Although anti-pseudomonal antibiotics were started empirically in 40 of the 43 patients (93%) at the beginning of the treatment, the most frequently isolated pathogens were Acinetobacter spp. and Escherichia coli. The success rate at the end of the treatment was 65.1%. The survival rates for the 3rd, 14th, 42nd, and 365th days were 97%, 86%, 58%, and 19%, respectively. Elevated levels of urea [Hazard Ratio=1.01 (95% CI: 1.00–1.02)] and blood glucose [HR=1.01 (95% CI: 1.00–1.02)] were found to be independent risk factors affecting survival. The treatment success rate was higher in patients without neutropenia (n=23) than in those with neutropenia (n=20) (p=0.05).
CONCLUSION: The treatment success rate was low in patients who developed HAP while receiving immunosuppressive therapy.

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AMAÇ: Dalak boyutu hematolojik hastalýklarda önemli bir parametredir. Rutin klinik pratikte dalak uzunluðu gerçek dalak boyutunun en iyi göstergesidir. Dalak boyutlarýndaki bölgesel farklýlýklar doðru olmayan dalak ölçümü deðerlendirmelerine ve en sýk karþýlaþýlan dalak patolojisi olan splenomegalinin yanlýþ yorumlanmasýna neden olabilir. Dalak boyutunun normal aralýðýnýn bilinmesi bu durumda önþarttýr. Prospektif çalýþmamýzýn amacý saðlýklý genç eriþkin Türk erkeklerinde ortalama dalak uzunluðunun belirlenmesidir.
YÖNTEMLER: Mart 1993 ve Aðustos 2006 yýllarý arasýnda 161 hastaya allojeneik hematopoetik kök hücre nakli uygulandý. Siklofosfamide baðlý hemorajik sistit geliþimi engelleyebilmek amacýyla profilaktik olarak Mesna, hiperhidrasyon ve zorlu diürez uygulandý. Buna raðmen 161 olgunun 17’si aðýr olmak üzere 49’unda hemorajik sistit geliþti. Tedavi yaklaþýmý ve bulgular geriye dönük olarak deðerlendirildi. Yaþlarý 17-42 arasýnda 2179 saðlýklý genç eriþkin asker (Manisa er eðitim merkezi) tarama ultrasongrafisi ile incelendi. Olgularýn yaþ, boy, kilolarý, dalak uzunluðu ve aksesuar dalak varlýðý kaydedildi. Lineer regresyon analizi ile olgularýn dalak boyutu ile kilo, boy ve BMI (vücut kitle indeksi)'leri iliþkisi deðerlendirildi.
BULGULAR: Olgularda ortalama boy 173,1 cm±(SD) 6,5 cm, ortalama aðýrlýk 69,0 kg±(SD) 9,7 kg, ortalama BMI 22,62± (SD) 2,8. ortalama dalak boyu 10,76 cm±(SD) 1,84 cm bulundu. Dalak uzunluðu ile boy, aðýrlýk ve BMI arasýnda istatistiki anlamlý bir iliþki saptanmadý. (p < 0.01). Çalýþma grubumuzdaki olgularýn % 2.5'inde ultrasonografi ile aksesuar dalak saptandý.
SONUÇ: US tarama ile saðlýklý genç eriþkin Türk popülasyonu için dalak boyutu ortalama 10,76 cm bulundu. Türk toplumunda splenomegali deðerlendirilirken bu deðer göz önünde bulundurulmalýdýr.

OBJECTIVE: It is known that the measurement of splenic length in routine clinical practice is a very good indicator of actual splenic size. Knowledge of the normal range of spleen size in the population being examined is a prerequisite. Racial differences in splenic length could result in incorrect interpretation of splenic measurements. The purpose of this study was to establish the range of spleen length in a young male Turkish population.
METHODS: A total of 2179 volunteers, healthy men aged 17-42 years, from the annual Army Reserve Officer Training Corps training camp at Manisa were included in the study. Sonographic measurements of spleen length were performed on 2179 military personnel. Presence of accessory spleen was also determined. In addition, the height, weight, and age of each volunteer were recorded. Using linear regression analysis, the relation of spleen length and body height, weight and body mass index (BMI) was evaluated. Additionally, the prevalence of accessory spleen detected on ultrasound was calculated.
RESULTS: The mean±SD height was 173,1±6,5 cm, mean weight 69,1±9,7 kg, and mean BMI 22,62±2,87. Mean spleen length was 10,76±1,8 cm. The length of the spleen was below 12,80 cm in 95% of the subjects. No statistically significant correlation (p<0.01) between spleen length and body height, weight and BMI was found. The prevalence of accessory spleen was determined as 2.5% on ultrasound screening.
CONCLUSION: It was found that in healthy Turkish men, mean spleen length was 10,76±1,8 cm. This data should be taken into consideration when the diagnosis of splenomegaly is established in Turkish males.

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AMAÇ: Kemik lenfomalarý oldukça nadirdir ve en sýk görülen histolojik alt tip diffüz büyük B-hücreli (DBBHL) non-Hodgkin lenfomadýr (NHL). Hastalar çoðunlukla kemik ya da yumuþak dokuda þiþlik, kemik aðrýsý ve patolojik kýrýk yakýnmalarý ile baþvururlar. Tedavi genellikle cerrahi, radyoterapi, kemoterapi veya bunlarýn kombinasyonu þeklindedir.
YÖNTEMLER: Bu çalýþmada, merkezimizde 1995 ve 2005 yýllarý arasýnda taný alan 18 primer kemik NHL’lý (11 kadýn, 7 erkek) hasta retrospektif olarak analiz edilmiþtir. Hastalarýn taný anýndaki medyan yaþý 56.5 (27-78) yýl olup, medyan 4.5 (1-36) aylýk semptom süresinden sonra kliniðe baþvurmuþlardýr. Tüm hastalarda aðrý semptomu birinci sýrada yer almakla birlikte 3 hastada (%16.7) B-semptomlarý da saptanmýþtýr. Taný yöntemi olarak çoðunlukla “tru-cut” biyopsi (%66.7) uygulanýrken, hastalarýn %27.8’inde açýk operasyonla taný konulmuþtur.
BULGULAR: DBBHL % 77.8 (14 hasta) oranýnda en sýk görülen histolojik alt tip olup, %11.1’i anaplastik büyük hücreli lenfoma, %5.6’ý Burkitt-benzeri, %5.6’ý marjinal zon lenfoma olarak belirlenmiþtir. Ann Arbor klinik evreleme sistemine göre hastalarýn %44.4’ü Evre-I, %11,1’i Evre-II, %44.4’ü Evre-IV olarak deðerlendirilmiþ, kemik iliði tutulumu %22.2 hastada saptanmýþtýr. Bir hasta dýþýnda tümüne CHOP veya benzeri antrasiklin içeren kemoterapi protokolleri uygulanmýþ, 8 hastada (%44.4) Rituximab tedaviye eklenmiþtir ve bu hastalarýn hiçbirinde relaps izlenmemiþtir. %50 hastada radyoterapi birinci sýra tedavisi olarak uygulanmýþtýr. Medyan 37 (2-124) aylýk izlemde, tedavilerini tamamlayan ve tam remisyon saðlanan 17 hastanýn 5’inde (%27.8) relaps geliþmiþ olup halen tüm hastalar saðdýr. 5 yýllýk hastalýksýz sað kalým oraný %73.5 olarak belirlenmiþtir.
SONUÇ: Kemik lenfomalarýn tedavisi hastalýðýn evresi ve lokalizasyonuna göre planlanmalýdýr. Radyoterapi uygulansýn veya uygulanmasýn, özellikle rituximab ile birlikte sistemik kemoterapi yaþam süresini olumlu yönde etkiler görünmektedir.

OBJECTIVE: Non-Hodgkin’s lymphoma (NHL) of bone is a rare entity. The most common histological subtype is diffuse large B cell lymphoma (DLBCL). The major presenting symptoms are soft tissue swelling, bone pain and pathological fracture. Treatment options are chemotherapy, radiotherapy, surgery, or a combination of these modalities.
METHODS: We retrospectively analyzed the 18 patients (11 females, 7 males) with NHL of bone who were diagnosed and treated between 1995-2005. The median age was 56.5 years. The median duration of symptoms was 4.5 months. The bone pain was the first symptom in all patients. Tru-cut biopsy was performed for diagnosis in most of the cases. Diagnosis in five patients (27.8%) required open biopsy.
RESULTS: DLBCL (77.8%) was the most common histological type among all patients. Other histological subtypes were anaplastic large cell lymphoma (11.1%), Burkitt-like lymphoma (5.6%) and marginal zone lymphoma (5.6%). According to Ann Arbor staging system, 44.4% of patients were Stage I, 11.1% were Stage II and 44.4% were Stage IV. Bone marrow involvement was determined in four patients (22.2%). All patients except one were treated with anthracycline-containing regimens and eight patients (44.4%) received rituximab combination with chemotherapy. Radiation therapy was performed as the first-line therapy in 9 (50%) patients. The median follow-up was 37 months (range, 2-124 months). Among the 17 patients who achieved complete remission, five (27.8%) relapsed. All patients were still alive. The five-year relapse-free survival was 73.5%.
CONCLUSION: The treatment of bone lymphoma can be planned according to the stage and location of the disease. Although we had a relatively low number of patients, it could be concluded that whether or not radiation therapy is performed, rituximab in combination with systemic chemotherapy has been proven beneficial on survival.

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Göðüs aðrýsý yakýnmasý ile baþvuran dört pediatrik akut lenfoblastik lösemi olgusu pulmoner fungal enfeksiyon tanýsý almýþtýr. Düz grafilerinde pozitif bulgu olmayan bu hastalarýn, bilgisayarlý tomografi incelemelerinde pulmoner noduler opasifikasyonlar ve bazý hastalarda kavitasyonlar pulmoner enfeksiyon yönünden uyarýcý olmuþtur. Bu deneyimimiz lösemili hastalarda pulmoner invasif enfeksiyonun ilk yakýnmasýnýn göðüs aðrýsý olabilecegine, düz grafiler normal olsa bile bu hastalarda bilgisayarlý tomografinin erken yapýlmasý ile fungal enfeksiyonun erken taný ve tedavi edilmesinin önemine dikkat cekmektedir.

We describe herein four children with acute lymphoblastic leukemia who were diagnosed as pulmonary fungal infection after presenting with chest pain. The plain radiologic evaluations failed to reveal any positive findings, whereas computerized tomography (CT) scanning showed nodular opacification with or without cavitation. This experience suggests that chest pain may be an initial symptom of an invasive fungal infection in patients with leukemia, and CT scan of the lungs should be performed urgently for the early diagnosis and treatment, despite normal plain X-rays.

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Bu makalede hemofagositik sendrom (HLH) tanýsý alýp daha sonra aplastik anemi (AA) geliþen 2 hasta sunulmuþtur. Her 2 hastada da HLH tanýsýndan önce etyolojisi net olarak konulmamýþ akut hepatit öyküsü vardý ve bu enfeksiyon nedeni ile HLH’leri enfeksiyon iliþkili HLH olarak deðerlendirilmiþti. Ýki olguda da ateþ ve pansitopeni vardý. Hemofagositoz iki olguda da kemik iliði aspirasyon materyalinde gösterilmiþti. Bir olguda steroid diðerinde steroide ek olarak etoposid ve siklosporin (CyA) ile yüksek ateþ ve karaciðer fonksiyonlarýnda düzelme saptanmaya baþlanmýþtý. Tedaviye raðmen aðýr sitopeninin devam etmesi üzerine kemik iliðinin tekrar deðerlendirilmesi sonucunda hastalara aplastik anemi tanýsý konuldu. Hemofagositik sendrom uzun süreli tedavi edilmediðinde kemik iliðinde aplaziye neden olabilir, bu nedenle hepatite baðlý geliþen aplastik anemilerin geliþim sürecinde HLH’ da olabilir.

Two unusual cases of hemophagocytic lymphohistiocytosis (HLH) complicating aplastic anemia (AA) are described. Each patient had a history of preexisting acute hepatitis of unknown cause at the time of HLH diagnosis and infection-associated secondary HLH. They developed high fever and pancytopenia. Hemophagocytes were seen in the bone marrow. With steroid (in combination with etoposide and CyA in 1 patient), high fever disappeared and the patients’ liver function gradually recovered. As severe pancytopenia persisted, bone marrow became acellular and AA was diagnosed. Since HLH is known to be able to cause an aplastic bone marrow if untreated for a prolonged time, it is therefore in line that hepatitis-associated AA may also be associated with HLH.
Aplastic anemia-associated HLH has been reported rarely, and problems in the diagnostic procedure are discussed.

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Blastlarýn azurofilik granülasyonu, akut miyeloid löseminin (AML) bir özelliðidir. Granüler akut lenfoblastik lösemi (ALL), sitoplazmik granüllerin lenfoblastlardaki varlýðýna baðlý olarak AML’ye benzeyebilir, ancak histokimya ve immünofenotipleme doðru tanýnýn konulmasýnda yardýmcýdýr. Toluidin mavisi (TB) bir metakromatik boya olup, bazofilik diferansiyasyon sergileyen bazofiller ve miyeloid blastlarý boyar. TB'ye yönelik reaktivite, lenfoblastlarda tanýmlanmamýþtýr. Bu yayýnda, taný ikilemine neden olan TB’ye yönelik reaktivite sergileyen blastlar ile granüler ALL’ye yönelik iki vaka rapor edilmektedir. Ýmmünofenotipleme ve sitogenetik çalýþmalar, doðru tanýnýn konulmasýný saðlamýþtýr. Bu iki nadir vaka raporu ile þimdiye dek tanýmlanmamýþ TB’li lenfoblastlarýn reaktivitesinin ve akut lösemide ayrýntýlý tanýsal tetkikin öneminin altý çizilmektedir.

Azurophilic granulation of blasts is a feature of acute myeloid leukemia (AML). Granular acute lymphoblastic leukemia (ALL) may mimic AML due to the presence of cytoplasmic granules in lymphoblasts, but cytochemistry and immunophenotyping are helpful in making the correct diagnosis. Toluidine blue (TB) is a metachromatic dye, which stains basophils and myeloid blasts that exhibit basophilic differentiation. Reactivity for TB has not been described in lymphoblasts. We herein report two cases of granular ALL with blasts exhibiting reactivity for TB that caused diagnostic dilemma. Immunophenotyping and cytogenetic studies were helpful in making a correct diagnosis. This report of two rare case highlight the reactivity of lymphoblasts with TB not hitherto described and the importance of a detailed diagnostic work-up in acute leukemia.

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