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Objective: Achieving an early molecular response (EMR) is crucial for improving the prognosis of patients with chronic myeloid leukemia (CML). The halving time (HT) and reduction ratio (RR) of BCR: : ABL1 transcript levels have recently emerged as additional prognostic indexes besides the BCR: : ABL1 International Scale (IS). We aimed to investigate the prognostic role of BCR: : ABL1 transcript levels, HT, and RR on molecular response kinetics at 3 months in patients with newly diagnosed chronic-phase (CP)-CML. Materials and Methods: Forty patients with CP-CML who received first-line imatinib treatment were included in this study. BCR: : ABL1 transcript levels and molecular responses at baseline and at 3, 6, 12, and 24 months of treatment were evaluated retrospectively. Major molecular response (MMR) at 12 months and event-free survival (EFS) were determined as primary endpoints and the effects of treatment kinetics on these parameters were examined. Results: Of the 40 patients, BCR: : ABL1 IS was ≤10% at 3 months in 72.5%, representing EMR. The rate of event occurrence was 45.5% in patients with BCR: : ABL1 IS of >10%, whereas it was 6.9% in those with BCR: : ABL1 IS of ≤10% (p=0.004). MMR was detected in 62.1% of the patients with EMR and in 9.1% of those without EMR (p=0.003). The cut-off value for achieving MMR was 24 days for HT and 0.04 for RR. Deep molecular response (DMR) at 24 months was associated with HT of ≤24 days and RR of ≤0.04. EFS was found to be significantly better in the group with BCR: : ABL1 IS of ≤10% and HT of ≤24 days (p=0.001) and in the group with BCR: : ABL1 IS of ≤10% and RR of ≤0.04 (p=0.007) compared to others. Conclusion: Our findings revealed that MMR could be predicted via EMR as well as by HT and RR. Additionally, HT of ≤24 days and RR of ≤0.04 were more important than BCR: : ABL1 IS of ≤10% in achieving DMR at 24 months, and the combination of BCR: : ABL1 IS of ≤10% with both HT of ≤24 days and RR of ≤0.04 has the best predictive value for EFS.

Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.

Background: The transmembrane receptor tyrosine kinase-like orphan receptor 1 (ROR1) has acted on the causation and sustentation of mature B cell lymphomagenesis for chronic lymphocytic leukemia (CLL) cells. The aim of this study was to show whether there is a relationship between the level of ROR1 surface expression in CLL cells and disease findings. Materials and Methods: The level of ROR1 cell surface expression was determined in accordance with the flow cytometric analysis of CLL patients at the first diagnosis time. 2 groups were formed according to the high and low ROR1 levels. The cut-off point to the ROR1 level was calculated for advanced-stage disease by using receiver operating characteristic (ROC) curves. A two-sided p-value

Castleman disease is a rare lymphoproliferative disease also known as angiofollicular lymph node hyperplasia. It is classified as hyaline vascular and plasmacytic variants histologically but characteristics of both types can coexist. Most unicentric cases of the disease are hyaline vascular while most multicentric cases are of the plasmacytic type. Although the pathogenesis is not completely understood, the role of interleukin (IL)-6 in unicentric disease and the roles of IL-6 and human herpes virus-8 in multicentric disease are well defined. Unicentric disease is typically localized and symptoms are minimal and treated locally. Multicentric disease is systemic and clinically characterized by generalized lymphadenopathy, splenomegaly, anemia, and systemic inflammatory symptoms. Systemic therapies are primarily given. Several malignant diseases including lymphomas, POEMS syndrome, follicular dendritic cell sarcomas, paraneoplastic pemphigus, Kaposi sarcoma, and amyloidosis can be associated with Castleman disease. In this paper, recent information about Castleman disease, which is a rare disease, is summarized.

Amaç: Nedeni bilinmeyen ateş (NBA) etiyolojisi çoğu zaman klinisyenleri uğraştıran, tanı için invaziv işlemler gerektiren klinik bir tablodur. Kemik iliği kültürü ve biyopsisi ile lenf nodu biyopsisi bu amaçla tercih edilen invaziv girişimlerdir. Çalışmamızda NBA etiyolojisi araştırdığımız hastalarımıza ait bu incelemelerin tanıdaki rolü ve sonuçları tartışılmıştır.Materyal ve Metot: Ankara Atatürk Eğitim ve Araştırma Hastanesi Enfeksiyon Hastalıkları Kliniği’nde NBA etiyolojisine yönelik kemik iliği (Kİ) kültürü ve biyopsisi amacı ile Hematoloji ile konsülte edilen ve lenf nodu biyopsisi değerlendirilen hastalar incelenerek, tanıda bu invaziv girişimlerin rolü değerlendirilmiştir.Bulgular: Kemik iliği kültürü-biyopsisi yapılan 18 erkek, 14 kadın, toplam 32 hastadan alınan kemik iliği kültürlerinin altısında üreme saptandı. İki hastada eş zamanlı olarak hem kan, hem de kemik iliği kültüründe üreme vardı. İki hastanın sadece kemik iliği kültüründe Brucella spp. üredi.Kemik iliği biyopsilerinden 24’ü (%75,00) normoselüler, 6’sı (%19,00) hiperselüler, biri (%3,00) lenfoma tutulumu gösteren kemik iliği ve bir diğeri ise poliklonal plazma hücre artışı gösteren hiposelüler kemik iliği şeklinde raporlandı. Fizik muayenede lenfadenopatisi saptanan dokuz hastaya lenf nodu biyopsisi yapıldı. Lenf nodu biyopsileri ile bir hastada Leishmania spp., bir hastada EBV ve iki hastada diffüz büyük B hücreli non-Hodgkin lenfoma (NHL) saptandı. Sonuç: NBA etiyolojisinde kemik iliği biyopsisi ve kültürü ve lenf nodu biyopsisi invaziv tanısal girişimler olup, kemik iliği tutulumu ile seyreden brusella gibi zoonotik enfeksiyonlar başta olmak üzere lösemi, lenfoma, tüberküloz gibi durumları ortaya koymada altın standart testtir. NBA etyolojisi araştırılırken girişimsel hematolojik işlemlerin değeri göz ardı edilmemelidir. Birinci basamak hekimleri, bu işlemlerin gerekliliği ve zamanlaması konusunda bilgi sahibi olmalıdır.

Three cases of Epstein-Barr virus (EBV)-negative post-transplant lymphoproliferative disease that occurred 6 to 8 years after renal transplantation are reported. The patients respectively had gastric mucosa-associated lymphoid tissue lymphoma, gastric diffuse large B-cell lymphoma, and atypical Burkitt lymphoma. Absence of EBV in the tissue samples was demonstrated by both in situ hybridization for EBV early RNA and polymerase chain reaction for EBV DNA. Patients were treated with reduction in immunosuppression and combined chemotherapy plus an anti-CD20 monoclonal antibody, rituximab. Despite the reduction in immunosuppression, patients had stable renal functions without loss of graft functions. The patient with atypical Burkitt lymphoma had an abnormal karyotype, did not respond to treatment completely, and died due to disease progression. The other patients are still alive and in remission 5 and 3 years after diagnosis, respectively. EBV-negative post-transplant lymphoproliferative diseases are usually late-onset and are reported to have poor prognosis. Thus, reduction in immunosuppression is usually not sufficient for treatment and more aggressive approaches like rituximab with combined chemotherapy are required.

Many cases have been established with coexisting Kaposi’s sarcoma (KS) and classical Hodgkin’s Lymphoma (C-HL) in the same lymph node. But composite presentation of KS and Nodular Lymphocyte Predominant subtype of Hodgkin’s lymphoma (NLPHL) in the same lymph node has not been described yet. KS is related to immunodeficiency most frequently due to human immunodeficiency virus (HIV) infection or immunosupression by other reasons. Our case presented here was not related to any immunodeficiency status. Besides of being the first case of composite KS and NLPHL in the same lymph node, it was also unusual with the indolent behaviour of the NLPHL without any therapy for 8 years follow up and primary lymph node presentation of KS without cutaneous involvement.

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Introduction: Pretransfusion testing is an essential serological test to protect the recipient from hemolysis and provide compatible blood product. The final step is the crossmatching test which is done by the transfusion center. Although all products are crossmatched in same cases, compatible products may not be available. Aims and Objectives: This study aimed to determine the safety and efficacy of the least incompatible crossmatched erythrocyte transfusion, through the use of biological in vivo crossmatch testing. Materials and Methods: The study included twenty patients who required transfusion and for whom appropriate red blood cell (RBC) could not be found. Totally, 69 units of least incompatible RBC transfusion from crossmatch incompatible products was administered by applying the “in vivo compatibility” test. Patients were observed during the transfusion with respect to acute hemolytic reactions that could develop. The biochemical hemolysis parameters were examined before and at 24 h after transfusion. Results: All transfusions were completed successfully with no complications or symptoms observed in any case. A statistically significant increase in hematocrit (Hct) and hemoglobin was seen post transfusion (P < 0.001). As parameters of hemolysis, lactate dehydrogenase and bilirubin levels were found to be statistically normal (not increased) (P = 0.453 and 0.946, respectively). Conclusions: Biological in vivo compatibility testing seems to be a safe, predictive, and bedside feasible test, which could be lifesaving for many patients.

Background: Complete response (CR) and very good partial response (VGPR) are targeted with pre-ASCT induction regimens in patients by diagnosed multiple myeloma (MM), who are candidates for ASCT. In this study, it was aimed to compare the response and survival evaluations of cases who underwent induction treatment by vincristine-doxorubicin-dexamethasone (VAD) protocol versus bortezomib containing regimens. Materials and Methods: The data of 96 ASCT eligible patients, retrospectively analyzed. P value> 0.05 was considered statistically significant. Results: While 66 cases had received bortezomib containing regimens as induction regimen, 30 cases had received VAD protocol. The total survival was 91.3 (st.s 6) months and 43 (st.s 7.9) months, respectively, when we compared the cases without ASCT and with ASCT (p = 0.001). The OS of patients who underwent ASCT after reaching at least VGPR was longer than the underwent ASCT without reaching VGPR (p=0.019). Post-ASCT PFS (p=0.717) and OS (p = 0.126) analyzes were performed in 74 cases undergoing ASCT treatment, there was no significant statistical difference when patients with treated by VAD protochol and treated by bortezomib containing regimens as pre-ASCT induction regimens was compared to each other. Conclusion: Whatever the type of induction regimen is, the level of response achieved before ASCT is important. The survival of the myeloma patients are much more influenced with HDT-ASCT as well as post-transplantation strategies to keep the patients in remission. Even though it is outdated, we think that the VAD protocol may be an option in patients who are not responding with the new generation of agents in the following days.

We report a young male patient who developed plasma cell myeloma/plasmacytoma 11 years after having received an allogeneic hematopoietic cell transplantation for AML. The patient received a second transplantation from the same donor without immunosuppression and developed graft-versus-host disease (GVHD). Our observation has two aspects that warrant attention: first, insufficiency of long-term tolerance to prevent GVHD in the absence of immunosuppression and second, a stromal or genetic susceptibility to develop hematologic malignancies despite of a complete donor-type chimerism.