Mutlu Kasar

Prophylactic red blood cell exchange may be beneficial in the management of sickle cell disease in pregnancy

Sickle cell disease (SCD) is associated with chronic hemolysis and painful episodes. Pregnancy accelerates sickle cell complications, including prepartum and postpartum vasoocclusive crisis, pulmonary complications, and preeclampsia or eclampsia. Fetal complications include preterm birth and its associated risks, intrauterine growth restriction, and a high rate of perinatal mortality. The purpose of this study was to evaluate pregnancy outcomes in patients with SCD who underwent planned preventive red blood cell exchange (RBCX).

Cranial involvement in sickle cell disease

PURPOSE To evaluate cranial findings in patients with neurologically symptomatic sickle cell disease (SCD). MATERIALS AND METHODS We studied 50 consecutive patients with SCD and neurologic symptoms. All patients underwent brain MR examinations: all 50 underwent classic MR imaging; 42, diffusion-weighted MR imaging; 10, MR angiography; four, MR venography; and three patients, digital subtraction angiography. RESULTS Of the 50 SCD patients, 19 (38%) had normal MR findings, and 31 (62%) showed abnormalities on brain MR images. Of the 50 patients, 16 (32%) had ischemic lesions; two (4%), subarachnoid hemorrhage; one (2%), moya-moya pattern; one (2%), posterior reversible encephalopathy; one (2%), dural venous sinus thrombosis; 12 (24%), low marrow signal intensity and thickness of the diploic space; 12 (24%), cerebral atrophy; and two (4%), osteomyelitis. Twenty-seven patients (54%) presented with headache, which was the most common clinical finding. CONCLUSIONS The cranial involvement is one of the most devastating complications of SCD. Early and accurate diagnosis is important in the management of cranial complications of SCD.

Effectiveness of Fludarabine- and Busulfan-Based Conditioning Regimens in Patients With Acute Myeloblastic Leukemia: 8-Year Experience in a Single Center

OBJECTIVE Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative treatment for acute myeloblastic leukemia (AML). Because the conditioning regimen of busulfan plus cyclophosphamide carries significant risks of toxicity, we evaluated the factors affecting survival after fludarabine replacement instead of cyclophosphamide. METHODS The study included 55 patients who underwent allo-HSCT for AML and received busulfan, fludarabine, and antithymocyte globulin (ATG). RESULTS Forty-eight patients received a myeloablative regimen; 7 patients received a reduced-intensity conditioning regimen. The neutrophil and platelet engraftment times were 12 days (range 9 to 20) and 12 days (range 7 to 19), respectively. Graft-vs-host disease (GvHD) developed in 10% and 50% of the patients, respectively. Seven patients received donor lymphocyte infusion. Of them, 5 patients developed grade I or II GvHD, one grade IV GvHD. The median follow-up period was 20.6 months. The predicted progression-free survival (PFS) at 1 and 3 years after transplantation was 78% and 74%, respectively. The overall survival (OS) at 1, 3, and 5 years was 76%, 74%, and 62%, respectively. Treatment-related mortality (infection in 1 patient, GvHD in 2 patients) occurred in 3 patients (5.5%). Multivariate analysis revealed that OS and PFS were not influenced by age, dose of busulfan or ATG, or presence of cytomegalovirus antigenemia. Acute GvHD and pretransplantation minimal residual disease positivity negatively affected the transplant outcome. The presence of active disease at the time of transplantation was found as an independent risk factor for AML. CONCLUSIONS Busulfan- and fludarabine-based conditioning regimens are effective for AML, and have acceptable toxicity, morbidity, and mortality.

Red blood cell alloimmunization in patients with sickle cell disease in Turkey: a single center retrospective cohort study -

Amac: Orak hucre hastaliginin sik goruldugu bolgemizde eritrosit alloimmmunizasyon sikligini ve iliskili durumlari arastirmayi amacladik. Gerec ve Yontem: bu calisma tek merkezli, zamansal kesitli ve geriye donuk kohort calsimasi olarak planlanmistir. Toplamda 216 orak hucre hastaligi [Hemoglobin (Hb) SS, Hb S-β talasemi, Hb S-α talasemi] tanisi olan hasta calismaya dahil edilmistir. Hastalar transfuzyon miktarina gore iki gruba ayrilmistir. Yilda 6’dan daha az transfuzyon alan ya da eritroferez oykusu olmayan hastalar Grup 1’e, yilda 6 ve daha fazla basit transfuzyon alan ya da eritroferez islemine alinan hastalar Grup 2’ye dahil edilmistir. Bulgular: Calismamiza 216 hasta dahil edilmistir. Transfuzyon tedavisi alan toplam 216 hastanin 67 (%31.0)’sinde, Grup 1’deki 56 hastanin 17’sinde (%30.4), Grup 2’deki 160 hastanin 50’sinde (%31.3) tespit edilmistir. Hastalar alloimmunizasyon gelisimi acisindan analiz edildiginde, calismamiz ne orak hucre komplikasyonlarinin alloimmunizasyon gelisimi icin ne de alloimmunizasyonun olum icin bir risk faktoru olmadigini gostermistir. Sonuc: Calismamamizda bulunan yuksek alloimmunizasyon sikligi, tecrubeli merkezler disinda yapilan transfuzyonlarda alloimmunizasyonu onleyici politikalara yeteri kadar uyulmadigi konusunda fikir vermektedir. Bu nedenle orak hucre anemili hastalarda alloimmunizasyon, ayrintili eritrosit antijen tanimlama islemi yapilarak azaltilabilir veya onlenebilir.

Prevention of recurrence of stroke in a patient with sickle cell disease who has Moyamoya Syndrome

Moyamoya sendromu orak hucreli anemi hastalarinda serebrovaskuler olaylardan yillar sonra gelisebilir. Bu calismada yeni bir norololojik atagin oncu isaretleri gelisen ve Moyamoya sendromu olan hastada, hidroksiure ve eritrosit degisimi ile inme olusmasini onleme cabalari sunulmaktadir.