Seckin Cagirgan

Hodgkin’s lymphoma following treatment with etanercept in ankylosing spondylitis

It has been well known that anti-TNF drugs might increase lymphoma risk in rheumatoid arthritis (RA), where the rate of lymphoma has already been increased. However, unlike RA, an increased rate of lymphoma has not been reported in ankylosing spondylitis (AS). Hereby, we present a case with AS developing Hodgkin’s lymphoma (HL) following 6 months of etanercept treatment. Pathological analysis revealed mixed cellular type of HL. Although we report a single case, it should be kept in mind that anti-TNF drugs might cause lymphoma development not only in RA, but also in AS.

The plasma levels of activated thrombin activatable fibrinolysis inhibitor and thrombomodulin in Behçet disease and their association with thrombosis.

OBJECTIVE To investigate the plasma levels of activated thrombin activatable fibrinolysis inhibitor (aTAFI) and thrombomodulin (TM) in Behçet disease (BD) and their relationship with thrombosis. METHODS Plasma aTAFI and TM levels were measured by ELISA in 89 patients with BD (18 having venous thrombosis) and in 86 healthy controls. RESULTS Compared with healthy controls, the BD group had significantly lower levels of aTAFI (13.49+/-8.88 microg/ml vs. 26.76+/-11.57 microg/ml, p<0.0001) and significantly higher levels of TM (3.26+/-1.85 ng/ml vs. 2.6+/-0.69 ng/ml, p=0.0003). Neither aTAFI, nor TM levels differed significantly between BD patients with and without thrombosis (p>0.05). Despite a tendency to positive correlation (r=0.37, p=0.0004) between plasma levels of aTAFI and TM in healthy controls, there was a tendency for negative correlation (r=-0.51, p<0.0001) between these two parameters in BD patients. CONCLUSION The plasma aTAFI and TM levels do not seem to be related with the presence of thrombosis observed in BD. Increased plasma TM levels in BD may simply reflect endothelial cell activation and dysfunction.

Risk factors for adverse events during collection of peripheral blood stem cells

We retrospectively reviewed peripheral blood stem cell (PBSCs) collections following 528 mobilization cycles over a 10-year period. A total of 206 (13.1%) AEs occurred in association with the 1572 procedures. One hundred and ninety-one (12.15%) of the AEs were classified as clinical AEs and 15 (0.95%) were classified as apheresis instrument related AEs. The most common clinical AE was numbness of the lips, tongue, or extremities (161 procedures, 10.2%) related to the infusion of acid citrate dextrose-A (ACD). Multivariate analysis revealed high amounts of ACD/weight (odds ratio [OR]=1.11, p=0.009), high numbers of procedures (OR=1.33, p<0.001) and female gender (OR=2.83, p<0.001) as being significantly associated with clinical AEs. Female gender was shown to be the most important risk factor for clinical AEs. Females who have a significantly increased risk of AEs would benefit from prophylactic calcium before and/or during PBSC collection.

Primary central nervous system leukemia presenting with an isolated oculomotor palsy.

We present a patient with an isolated oculomotor nerve palsy due to central nervous system leukemia with bone marrow findings consistent with myelofibrosis without any blasts. This has not been previously reported.

Immunohistochemical detection of CD 95 (Fas) & Fas ligand (Fas-L) in plasma cells of multiple myeloma and its correlation with survival

Multiple myeloma (MM) is a malignant disease resulting from an uncontrolled proliferation of a neoplastic plasma cell clone in the bone marrow, which might also be induced by the loss of control on apoptosis. Fas ligand (Fas-L), a member of the tumor necrosis factor family, induces apoptosis mediated via its transmembrane death receptor Fas (Apo-1/CD95) antigen. In the present study, immunostaining was performed on the initial diagnostic bone marrow biopsies of 36 MM patients (1 stage I, 5 stage II, 30 stage III), to evaluate the distribution of Fas receptor and Fas-L on malignant plasma cells. Both Fas and Fas-L were positive in 13 cases and negative in 3, whereas 10 cases were Fas-negative, Fas-L-positive and 10 were Fas-positive, Fas-L-negative. Although no association was found between the expression of Fas receptor or Fas-L and overall survival, Fas-L positivity was significantly associated with a shorter event-free survival (p = 0.0335). In this study, it has been shown that the expression of Fas-L, in malignant plasma cells of myeloma patients significantly shortens the event-free survival, indicating that the defect in apoptosis might be associated with disease progression in MM.

Two Consecutive Spontaneous Regressions to Chronic Phase in a Patient with Blastic Transformation of Chronic Myelogenous Leukemia

In this report, we present a patient with chronic myeloid leukemia (CML) in blastic phase who had two consecutive episodes of spontaneous regression back to chronic phase without chemotherapy. Although, spontaneous remission (SR) is well documented in acute leukemia, SR in CML blastic phase is extremely rare and to the best of our knowledge only one case has been reported in the world literature. The factors possibly related to this phenomenon are discussed.

Acquired activated protein C resistance in sarcoma patients.

Acquired activated protein C resistance (aAPCR) is seen more frequently in solid and hematological cancer patients. We aimed to investigate the presence of aAPCR and the frequency of clinically detectable thrombosis in sarcoma patients. Normalized activated protein C sensitivity ratio (nAPCSR), factor V Leiden (FVL) mutation, factor V (FV) levels and factor VIII (FVIII) levels were prospectively measured in 52 patients and in 52 healthy controls. Clinically detectable thrombosis was present in one patient (1.92%). Compared with healthy controls (106%), the sarcoma patients had significantly lower values of the nAPCSR at pre (87.25%) and post (94.35%) treatment period (P < 0.0001). aAPCR was found as 4.2, 13 and 0%, respectively. The post-treatment FV levels (178.1 U/dl) were significantly (P < 0.001) higher than the pretreatment levels (147.5 U/dl). Inverse correlation was found between post-treatment FV levels and nAPCSR values (r = −0.38, P < 0.02). We found out a slightly increased frequency of venous thromboembolism in sarcoma patients. As an original finding which has not been reported previously in the literature, we also found out a decrease in the nAPCSR, persisting even after treatment. Thirdly, we found out that the significantly higher rate of aAPCR at the time of diagnosis totally disappeared after treatment.

Primary cutaneous B-cell lymphoma: report of eight cases and review of the literature

Primary cutaneous B-cell lymphoma (PCBCL) is a heterogeneous group of lymphoproliferative diseases comprising 5– 10% of all cutaneous lymphomas reported by European groups. 1 PCBCL is defined as a lymphoma of B-cell origin with no evidence of extracutaneous involvement at presentation. 2 After the gastrointestinal tract, skin is the second most common site of extranodal involvement of non-Hodgkin’s lymphoma (NHL). 3,4 Two classification systems for PCBCL are in use currently: the European Organization for Research and Treatment of Cancer (EORTC) 5 and the recently published World Health Organization (WHO) Classification of Neoplasms of Haematopoietic and Lymphoid Tissues. 6 The EORTC classifies PCBCL into indolent and intermediate categories. The indolent group includes follicle center cell (FCC) lymphoma and immunocytoma. Large B-cell lymphoma of the leg is considered intermediate grade, and EORTC tends to use this term for a specific, aggressive clinical entity involving the leg. FCC lymphoma typically presents as solitary or grouped nodules or plaques, often localized to the scalp, forehead, or back; systemic dissemination is rare. In the WHO classification, the term follicle cell (FC) lymphoma is preferred over FCC lymphoma. Thus, clinical series that utilize the WHO classification are more likely to consider a lesion with large cells and/or diffuse histology as FCC lymphoma; many FCC lesions are classified as diffuse large B-cell lymphoma (DLBCL). 7,8 Although EORTC subdivides cutaneous B-cell lymphomas into two main clinical categories, PCBCL-leg and PCFCCL, primarily based on the site of presentation, the WHO system does not accept the prognostic importance of such distinction. 8,9 During recent consensus meetings, differences between WHO and EORTC were resolved and a consensus classification system was developed, as outlined in Table 1. 10

Recommendations for Risk Categorization and Prophylaxis of Invasive Fungal Diseases in Hematological Malignancies: A Critical Review of Evidence and Expert Opinion (TEO-4)

This is the last of a series of articles on invasive fungal infections prepared by opinion leaders in Turkey. The aim of these articles is to guide clinicians in managing invasive fungal diseases in hematological malignancies and stem cell transplantation based on the available best evidence in this field. The previous articles summarized the diagnosis and treatment of invasive fungal disease and this article aims to explain the risk categorization and guide the antifungal prophylaxis in invasive fungal disease.

The loss of CD34+ cells in peripheral hematopoietic stem cell products cryopreserved by non-controlled rate freezing and stored at −80 °C after overnight storage

Although peripheral blood stem cell (PBSC) products cryopreserved by non-controlled rate freezing and stored at -80 °C after overnight storage are used frequently, data regarding the rate of loss of CD34+ cells in these products are limited. In this prospective study, CD34+ cells were counted at three (fresh, post-overnight and post-thaw) points in 83 PBSC products from 41 patients by flow cytometry. Compared to fresh products, the mean losses of post-overnight and post-thaw total CD34+ cells are 16.3% and 38.4% (p = 0.02), and the mean losses of post-overnight and post-thaw viable CD34+ cells are 16.5% and 48.5%, respectively (p < 0.001). The numbers of fresh viable, post-thaw total and post-thaw viable CD34+ cells were inversely correlated with the durations of neutrophil and platelet engraftment. Our results indicate that the mean loss of post-thaw total and viable CD34+ cells is approximately 20% higher than that observed in standard cryopreservation methods. In addition, fresh viable, post-thaw total and especially post-thaw viable CD34+ cell levels are valuable predictors of both neutrophil and platelet engraftments.