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Abstract | Full Text PDF

Calcineurin`in (protein fosfataz tip 2B, PP2B) metilprednizolona baðlý lösemi hücre farklýlaþmasý ve apopitozundaki rolünü anlamak amacý ile HL-60 lösemik hücrelerin metilprednizolon altýnda apopitozisi, calcineurin spesifik protein fosfataz aktivitesi ve calcineurin`in regülatör ve katalitik alt birimlerinin ve calmodulin`in ekspresyonunu inceledik. Hücre kültür ortamýna metilprednizolon eklendikten sonraki 72 saatlik dönemde hücreler apopitoz ve farklýlaþma gösterdiler. Apopitoz oluþmadan, farklýlaþma döneminde spesifik calcineurin enzim aktivitesinde kademeli bir artýþ izlendi. Ancak bu sýrada katalitik ve regülatör calcineurin alt birimlerinin immünblotlarýnda artýþ olmazken, calmodulin ekspresyonu giderek arttý. Metilprednizolona baðlý apopitoz ve farklýlaþmada spesifik calcineurin enzim aktivitesinde belirgin artýþ olmasý calcineurin`in posttranslasyonel modifikasyonlarnda belirgin öneme sahip olabilir.

To evaluate the role of calcineurin (protein phosphatase type 2B, PP2B) in methylprednisolone-induced differentiation and apoptosis of leukaemic cells, we have investigated the induction of apoptosis, calcineurin specific protein phosphatase activity and expression of regulatory and catalytic subunits of calcineurin and calmodulin after induction of HL-60 leukaemic cells with methylprednisolone. The cells underwent differentiation and apoptosis within 72 hours time period after methylprednisolone added to cell culture media. Before apoptosis occurred, the specific calcineurin enzyme activity revealed gradual increase during the differentiation process. However, immunoblots of catalytic and regulatory subunits of calcineurin showed no amplification in the amount of these cellular signaling mediators during methylprednisolone-induced differentiation and apoptosis but calmodulin expression gradually increased during the process. Significant increase in the specific calcineurin enzyme activity during differentiation and apoptosis might be crucial to the posttranslational modifications of calcineurin during methylprednisolone-induced differentiation.

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CXCR4, CXC kemokin SDF-1’in reseptörüdür ve hematopoietik hücrelerin hominginde rol oynayabilir. Bu çalýþmada akut promiyelositik lösemili (APL) hastalarda ATRA tedavisi sýrasýnda CXCR4 gen ekspresyonunu araþtýrdýk. APL, 15 ve 17. kromozomlardaki PML ve RAR alfa genleri arasýnda özgül bir translokasyonla karakterizedir. ATRA’ya baðlý APL hücre farklýlaþmasý sýký bir þekilde PML-RAR alfa varlýðýna baðlýdýr. Bu çalýþmada beþ APL hastasý alýnmýþ ve her hastadan tanýda ve remisyonda alýnan iki örnek birbirleri ile karþýlaþtýrýlmýþtýr. Hýzlý ve duyarlý bir yöntem olan kantitatif gerçek zamanlý PCR C LightCycler kantifikasyon için kullanýlmýþtýr. Tanýda promiyelosit oranlarý %18 ve %20 olan iki olguda CXCR4 gen oranlarý az eksprese olmuþ, %50 ve %80 olan iki olguda ise orta derecede eksprese olmuþ, blast oraný %95 olan bir olguda ise aþýrý eksprese olmuþtur. Bu bulgular ATRA tedavisinin, blast oranýna baðlý olarak CXCR4 eksprese eden olgularda etkili olabileceðini ancak ATRA’nýn CXCR4’le ilgili moleküler yollarda nasýl etki ettiðinin araþtýrýlmasý gerektiðini göstermektedir.

CXCR4 is the receptor of CXC chemokine SDF-1 and may play a role in the homing of hematopoietic stem cells. We have investigated the CXCR4 gene expression during ATRA treatment in acute promyelocytic leukaemia (APL) patients. APL is a characteristic disorder with a specific translocation between PML and RAR alpha genes on chromosome 15 and 17. ATRA-induced differentiation of APL cells is strictly dependent on the presence of PML-RAR alpha. In our study, five APL patiens were involved. Two samples from each patient were compared to each other: Primary diagnostic sample and a sample taken at remission. Quantitative real-time PCR (LightCycler) has been used for quantification, which is a recently developed method for rapid and sensitive detection of gene expression. CXCR4 gene ratios were found under-expressed in cases 1 and 6 with blast counts at diagnosis 18%, and 20% but only moderately under-expressed in cases two and four where the blast count was at diagnosis 50%, and 80%. It was over-expressed only in case three where the blast count was 95%. These findings indicate that ATRA treatment might be effective in CXCR4 gene expression related to amount of the blast population in APL. Further gene expression studies would be helpful to understand how ATRA works on CXCR4 related molecular pathways in APL pathogenesis.

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Hemoglobin Pyrgos [ß83 (EF7) Gly→Asp] nadir rastlanan bir hemoglobin varyantýdýr. Bu rapor, bu varyantýn Türkiye’de ilk kez 18 yaþýnda Isparta’da yaþayan bir Türk kýzýnda görülmesini tanýmlamaktadýr.

Hemoglobin Pyrgos [ß83 (EF7) Gly→Asp] is a rare hemoglobin variant. This report describes the first observation of this variant in an 18-year-old Turkish girl living in Isparta.

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Trombotik trombositopenik purpura (TTP), tüketim trombositopenisi, immün olmayan intravasküler hemolitik anemi, böbrek yetmezliði, çeþitli nörolojik bulgular ve ateþ ile gelir. Bu klinik sendrom tiklopidin, kinin, mitomisin C, siklosporin gibi ilaçlar, allojeneik kök hücre nakli, gebelik, infeksiyon, otoimmün hastalýklar ve metastatik kanserlere eþlik edebilir. Bu yazýda TTP tablosu ile baþvuran ve ileri incelemede leomiyosarkom tanýsý konan 48 yaþýnda bir kadýn hasta sunulmaktadýr. Hasta multipl exchange transfüzyon ve bunu takip eden leomiyom çýkartýlmasý ile baþarýyla tedavi edilmiþtir. Bu olgu bilgimiz dahilinde leomiyosarkom ile seyreden ilk TTP olgusudur. TTP`li kadýn olgularda dikkatli jinekolojik muayene yapýlmasý ve pelvik ultrasonografi çekilmesi yararlý olabilir.

Thrombotic thrombocytopenic purpura (TTP) typically presents with consumptive thrombocytopenia, non-immune intravascular hemolytic anemia, renal failure, various neurologic findings and fever. It is a clinical syndrome that can be associated with drugs such as ticlopidine, quinine, mitomycin C and cyclosporine, allogeneic stem cell transplantation, pregnancy, infections, autoimmune diseases and metastatic carcinoma. Here, we describe a 48-years- old women presented with full picture of TTP and diagnosed as leiomyosarcoma with further evaluation. She was successfully treated with multiple exchange transfusions and total excision of leiomyoma thereafter. As far as we know, this is the first case of leiomyosarcoma of the uterus presented with TTP. Therefore, the women with TTP, in whom no other cause can be found, should undergo a careful gynecological examination and pelvic ultrasonography should be performed.

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Hiperkalsemi, miyelom ya da T-hücreli lösemi-lenfoma gibi bazý lenfoproliferatif hastalýklarda sýkça görülmektedir. Ancak B-hücreli kronik lenfositer lösemi (KLL) de nadiren tarif edilmiþtir. Yaygýn osteolitik lezyonlar ve çoðul patolojik kýrýklar ile baþvuran bir KLL olgusu sunulmaktadýr. Hasta 74 yaþýnda erkek olup bilateral humerus ve radius kýrýðý ve kafa, fibula, femur ve kostalarda çoðul osteolitik lezyonlar ile baþvurdu. Daha sonra yapýlan ileri incelemede KLL saptandý. Ayrýca hiperkalsemi de eþlik ediyordu. Hiperkalsemi etkeni olabilecek tüm nedenler araþtýrýldý ve dýþlandý. Açýk kemik iliði biyopsisi lenfosit infiltrasyonu olarak yorumlanýrken prolenfositlerde artýþ gözlendiði rapor edildi. KLL’li bir hastada; klinik seyir sýrasýnda hiperkalseminin ortaya çýkmasý negatif bir prognostik kriter olarak yorumlanabilir.

Hypercalcemia is common in some lymphoproliferative disorders such as myeloma or T- cell leukaemialymphoma, but is rarely described in B-cell chronic lymphocytic leukaemia (CLL). A CLL patient who have been presented with multiple pathological fractures and widespread osteolytic lesions is reported. He was a 74 year old male with fractures of his bilateral humerus and radii and multiple osteolytic lesions of skull, fibula, femur and costals. On his admission to the hospital for the fractures he has been diagnosed as CLL. Hypercalcemia has also been documented. All the disorders that could be the reason of hypercalcemia have been ruled out. The open biopsy of bone marrow showed lymphocytic infiltration in which increased number of prolymphocytes are observed. Hypercalcemia arising in a patient with CLL may indicate a negative prognosis.

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immünsüpresyon nakil sonrasý lenfoproliferatif hastalýk geliþmesi için risk faktörüdür. AML nedeni ile nakil yapýlan bir erkek hastada saðdan baþlayan ve iki jeneralize konvülziyon gözlenmesinin ardýndan çekilen BT taramada kitle lezyonlu saptanmýþ ve yapýlan stereotaksik biyopsi ile CD20 ve CD10 pozitif büyük B-hücreli lenfoma tanýsý konmuþtur. 56 Gy dozunda SSS ýþýnlamasý yapýlan hastada klinik ve radyolojik düzelme saptanmýþ, ancak hasta multiorgan yetmezliði ile kaybedilmiþtir.

Immunosuppression is a risk factor for the development of posttransplant lymphoproliferative disorder. This type of malignancy developed in an immunocompromised man. The patient presented with focal convulsion starting from right face and right arm followed by two generalized convulsions with one minute interval. Diagnosis was made by computed tomographic (CT) scan of the cranium following oral and intravenous administration of contrast dyes which revealedmass leisons in the frontotemporal lobe and by stereotaxic biopsy. Tissue sections showed a malignant tumor cell infiltration with large areas of necrosis and many mitoses. Many tumor cells were positive for CD 20 and CD 10. These findings were consistent with large B-cell lymphoma. Central nervous system radiation with a dose of 56 Gy was given with clinical and radiological improvement. The patient died due to multiorgan failure. Finally, the immunocompromised patients should be closely followed for the development of lymphoproliferative disorder.

Abstract | Full Text PDF

Abstract | Full Text PDF

Abstract | Full Text PDF