The Turkish experience with therapeutic plasma exchange: A national survey.

Therapeutic plasma exchange (TPE) is used to treat more than 60 diseases worldwide and has drawn growing interest. Little is known about the current situation of TPE activity in Turkey, so we developed a survey to obtain information about this timely topic. We collected data on TPE from 28 apheresis units throughout Turkey. We performed a total of 24,912 TPE procedures with 3203 patients over the past decade. Twenty years ago, the majority of procedures were performed for neurological and hematological disorders, and today, most TPE procedures are done for the same reasons. The only historical change has been an increase in TPE procedures in renal conditions. Currently, renal conditions were more frequently an indication for TPE than rheumatic conditions. Fresh frozen plasma was the most frequently used replacement fluid, followed by 5% albumin, used in 57.9% and 34.6% of procedures, respectively. The most frequently used anticoagulants in TPE were ACD-A and heparin/ACD-A, used with 1671 (52.2%) and 1164 (36.4%) patients, respectively. The frequency of adverse events (AEs) was 12.6%. The most common AEs were hypocalcemia-related symptoms, hypotension, and urticaria. We encountered no severe AEs that led to severe morbidity and mortality. Overall, more than two thirds of the patients showed improvement in the underlying disease. Here, we report on a nationwide survey on TPE activity in Turkey. We conclude that there has been a great increase in apheresis science, and the number of TPE procedures conducted in Turkey has increased steadily over time. Finally, we would like to point out that our past experiences and published international guidelines were the most important tools in gaining expertise regarding TPE.

Age does not matter: Mobilization and harvesting are safe and effective for elderly allogeneic peripheral hematopoietic stem cell donors.

It took several years to succeed safe hematopoietic stem cell transplantations. HLA-matched unrelated donors have become the most common donor source for allogeneic hematopoietic stem cell transplants worldwide. The sibling donor may have more comorbidity and decreased regenerative potential of stem and immune cells. The purpose of this retrospective study was to examine whether aging had any negative effect on aging donor or patient. 27 patients who received a hematopoietic stem cell transplantation (HSCT) from February 2013 to May 2016 and their donors were analyzed. We showed that transplantation from older relative donor was feasible. Adverse event rate was low. Donors tolerated the procedure very well. Good CD34+ cell harvest was possible.

Autologous stem cell transplantation and stem cell mobilization kinetics in elderly patients with B cell non-Hodgkin lymphoma.

As known, the world population is aging and as the life span increases the number of advanced-age lymphomas also shows an upward trend. Autologous hematopoietic stem cell transplantation (HSCT) is the standard treatment modality in chemotherapy-sensitive relapsed or refractory aggressive lymphomas. Increased morbidity and mortality related to both the transplant itself and comorbid diseases can be observed in elderly lymphoma patients. Patients who are 65 years or older and underwent autologous HSCT with B-cell non-Hodgkin lymphoma were retrospectively included in our study. In terms of survival analysis, median follow-up was 34.5 months (8-159) while the overall survival (OS) was 58%. In the univariate analysis of prognostic data in OS, patients who were referred to transplantation with complete response had a statistically significant survival advantage (p=0.043). In terms of the effect of pre-transplant conditioning regimens on survival, BEAM regimen yielded better results, though not statistically significant. Age, number of chemotherapy cycles received before mobilization and radiation therapy had no significant effect on the CD34 (+) cell count in the final product (p=0.492, 0.746 and 0.078 respectively). In conclusion, autologous HSCT is a practicable treatment modality that provides survival advantage in suitable advanced-age patients with a diagnosis of B-cell non-Hodgkin lymphoma.

A new hemoglobin variant: Hb Izmir [ß86(F2)Ala→Val, GCC>GTC; HBB:c.260C>T].

We report a new hemoglobin (Hb) variant [ß86(F2)Ala→Val; HBB:c.260C>T] that we have named Hb Izmir. We have identified Hb Izmir in a Turkish woman by ion exchange high performance liquid chromatography (HPLC) during a premarital screening program in the Aegean region of Turkey. The mother and sister of the proband also carried the same variant. Using direct sequencing, we have characterized this variant as resulting from a GCC>GTC replacement at codon 86 of the ß-globin chain, corresponding to an Ala→Val amino acid substitution. In the heterozygote, the level of Hb Izmir ranged from 41.38 to 45.6%. All heterozygotes had a Hb A(2) level of less than 3.5%. Total blood count values were normal and there were no other clinical findings. Although its clinical significance is thus far unclear, Hb Izmir may be important in hemoglobinopathy screening programs.

Non-Hodgkin's lymphoma with bone involvement: a single center experience with 18 patients.

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.

Rapid transformation of atypical myeloproliferative disorder with consistent t(8;13) to B-cell acute lymphoblastic leukemia: a case report.

8p11 myeloproliferative syndrome (EMS; also known as the stem cell leukemia syndrome-SCLL) is a rare atypical myeloproliferative disorder associated with chromosomal abnormalities involving the 8p11 chromosomal band. Translocations associated with this syndrome result in the fusion of the fibroblast growth factor receptor 1 (FGFR 1) gene with various partners, resulting in ligand independent FGFR activity. The most commonly observed translocation of this syndrome is t(8;13), which results in the expression of a chimeric ZNF198-FGFR1 tyrosine kinase. Disease phenotype associated with this translocation has some typical features such as poor prognosis, and transformation to mainly acute leukemia and non-Hodgkin lymphoma; commonly with a T-cell phenotype in which obtaining and maintenance of remission is difficult by conventional chemotherapy. We hereby present a case diagnosed as atypical chronic myeloproliferative disease with consistent t(8;13)(p12;q12) and transformed rapidly to pre-B-cell acute lymphoblastic leukemia which is a rare clinical presentation.

Gastric and non-gastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue: A single-center experience.

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is a distinct lymphoma with specific clinical and pathological features that occurs in diverse anatomic locations. We conducted this retrospective study to demonstrate our experience in patients with MALT lymphomas and compare our results with the literature. We studied 23 patients with histologically confirmed diagnosis of MALT lymphomas (12 with gastric, 11 with non-gastric localization) treated during the past 13 years. The female/male ratio of patients was 15/8 with a median age at presentation of 56 years (range 27-88 years). 16 patients (70%) with stage I and II, 7 patients (30%) with stage III and IV were admitted. At presentation 93% of patients had good performance status (ECOG<2) and 5 (22%) had B-symptoms. There was no difference between gastric and non-gastric MALT lymphomas when compared with sex, age, ECOG performance status, stage of the disease. Patients were treated with different treatment modality; H. Pylori eradication only (8.6%), radiotherapy only (21.7%), surgery alone (4.%) or followed by radiotherapy (26%) or chemotherapy (21.7%). All the patients are alive with a median 33 months (range 8-153 months) of follow-up and the 5- year PFS in gastric lymphoma and non-gastric lymphoma were 86% and 84% respectively with no statistical difference (p=0.5). Because of the indolent course the prognosis of MALT lymphoma was good regardless of the treatment modalities. The treatment choice should be patient-tailored, taking into account the site, stage, age and other clinical characteristic of patient.

Retrospective analysis of results of short-term low dose interferon-α-2b combined with PUVA in the treatment of early stage mycosis fungoides.

Early-stage mycosis fungoides (MF) can be treated with different regimens such as oral photo-chemotherapy (Psoralen and UVA-PUVA). There have been some studies showing the effectiveness of combination of interferon (IFN) with PUVA. In this study, we aimed to evaluate retrospectively the patients with early-stage MF treated with IFN + PUVA. Six patients with the diagnosis of early stage (Ia-IIa) MF between June 2003 and May 2005 were enrolled in this study. IFN combined with PUVA was started and followed by IFN maintenance in complete response (CR) patients. Patients achieving CR were followed up at monthly intervals until relapse. Interferon-α-2b was administered at a dose of 3 MU 3 times a week and PUVA was applied 3 times a week. There were 4 female and 2 male patients, aged 32-75 years (mean 54.3 years). Four patients were at stage Ia, one patient at Ib and one patient at stage IIa according to TNM staging. Four of 6 patients (66%) achieved CR and 2 of 6 (33%) achieved partial response (PR). No grade 3-4 side effects due to IFN were detected, and no progression was observed during the treatment. All patients have been under treatment as planned. Low dose of IFN-α-2b plus PUVA was found to be successful in achieving excellent clinical responses in patients with early-stage MF. This treatment modality was very well tolerated.