Anna Christoforidou

Clinical features, outcome, and prognostic factors for survival and evolution to multiple myeloma of solitary plasmacytomas: a report of the Greek myeloma study group in 97 patients.

Solitary plasmacytoma (SP) is a rare plasma cell dyscrasia characterized by the presence of bone or extramedullary plasma cell tumors. The treatment of choice is local radiotherapy (R/T) ± surgical excision. The role of adjuvant chemotherapy (C/T) or novel agents (NA) is uncertain. Data related to prognostic factors are inconclusive. Herein, we describe the clinical features, survival and prognosis of 97 consecutive patients, 65 with bone SP (SBP), and 32 with extramedullary SP (SEP), diagnosed and treated in 12 Greek Myeloma Centers. Objective response rate (≥PR) and complete response (CR) was 91.8% and 61.9%, respectively, and did not differ between the 2 groups. Overall, 38 patients relapsed or progressed to multiple myeloma (MM). After a median follow‐up of 60 months, 5 and 10‐year overall survival (OS) probability was 92% and 89% in SEP and 86% and 69% in SBP, respectively (P = 0.2). The 5‐ and 10‐year MM‐free survival (MMFS) probability was 90% and 70% for patients with SEP vs. 59% and 50% for patients with SBP, respectively (P = 0.054). Overall, the 5‐ and 10‐year OS probability, plasmacytoma relapse‐free survival (PRFS), progression‐free survival and MMFS was 84% and 78%, 72% and 58%, 58% and 43%, and 70% and 59%, respectively. In the multivariate analysis, prolonged PRFS and young age were positive predictors of OS. Achievement of CR was the only positive predictor of PRFS. Immunoparesis was the only negative predictor of progression to MM. The addition of C/T or NA‐based treatment increased toxicity without offering any survival advantage over R/T. Am. J. Hematol. 89:803–808, 2014.

Hydroxyurea (HU) is effective in reducing JAK2V617F mutated clone size in the peripheral blood of essential thrombocythemia (ET) and polycythemia vera (PV) patients

Ph-negative chronic myeloproliferative disorders (PhnegcMPD) are treated according to the estimated vascular risk. The recent discovery of V617F point mutation of the JAK2 kinase, which frequently occurs in these diseases, has not changed their management so far. However, emerging data tend to support a prothrombotic role for the mutation, along with a better response of JAK2V617F mutated patients to hydroxyurea treatment. Our data further support this notion.

CD1d expression as a prognostic marker for chronic lymphocytic leukemia

Abstract We analyzed the expression of CD1d, an antigen-presenting molecule, on peripheral blood leukemic cells of cases of chronic lymphocytic leukemia (CLL) by flow cytometry. We demonstrated variable expression of CD1d on leukemic lymphocytes and an association between high expression of CD1d with shorter time to treatment and overall survival of patients. CD1d was positively associated with CD38 expression, but not with unmutated heavy chain variable (VH) mutational status or adverse cytogenetics of leukemic lymphocytes. Our findings support that CD1d expression is a prognostic marker for CLL.

Evaluation of hypochromic erythrocytes in combination with sTfR-F index for predicting response to r-HuEPO in anemic patients with multiple myeloma.

The purpose of this study was to evaluate the sTfR-F index and hypochromic erythrocytes (HYPO%) as potential predictors of response to recombinant human erythropoietin (r-HuEPO) of anemic patients with multiple myeloma (MM) before treatment, as well as early in the course of treatment. Twenty-six newly diagnosed anemic MM patients received r-HuEPO 30,000 IU/wk sc, for six weeks. The sTfR-F index and HYPO% were determined at baseline and at weeks 2 and 6. Patients were classified in 1 of 4 categories of a diagnostic plot, according to erythropoietic state (ES I-IV), defined by the combination of sTfR-F index and HYPO%. Sixteen of 20 patients in ES I and II before treatment responded to r-HuEPO, whereas none of the 6 patients in ES III and IV responded (P < .001). At week 2, 44% of patients who responded and 60% of the nonresponders were in functional iron deficiency (FID) and the proportion increased to 69% and 80%, respectively, by week 6. Seven of the patients who did not respond received in addition 200 mg iron sucrose IV weekly, for the next 4 weeks, and 6 of them responded. These results suggest that combination of sTfR-F index and HYPO% in a diagnostic plot can be used as a predictive model to recognize patients who will benefit from r-HuEPO and identify FID requiring iron supplementation, before treatment and early in the course of treatment, contributing thus to optimization of r-HuEPO therapy.

Pure red cell aplasia as first manifestation of splenic marginal zone lymphoma-successful treatment with rituximab: a case report

IntroductionAcquired pure red cell aplasia is a rare disorder, usually appearing secondary to various pathologic conditions such as thymoma, systemic autoimmune diseases or in the course of lymphomas. Conventional treatment consists of immunosuppression with corticosteroids, antithymocyte globulin or cyclosporin-A.Case presentation8 weekly courses of rituximab were administered to a patient who presented with pure red cell aplasia secondary to newly diagnosed splenic marginal zone lymphoma. Transfusion independence was achieved after the 6th course, and pure red cell aplasia receded completely with therapy.ConclusionPure red cell aplasia may ensue early in the course of splenic marginal zone lymphoma and other low grade lymphomas. Rituximab is a safe and effective alternative treatment for pure red cell aplasia secondary to lymphoproliferative disorders.

Hypercalcemia remains an adverse prognostic factor for newly diagnosed multiple myeloma patients in the era of novel anti-myeloma therapies

To evaluate the prognostic impact of hypercalcemia in newly diagnosed patients with symptomatic multiple myeloma (MM), especially after the incorporation of new agents.

Coagulation and fibrinolysis activation after single-incision versus standard laparoscopic cholecystectomy: a single-center prospective case-controlled pilot study.

Laparoscopic cholecystectomy is associated with attenuated acute-phase response and hypercoagulable state compared with the open procedure. Single-incision laparoscopic cholecystectomy is a new technique aiming to minimize the invasiveness of the procedure. By comparing the degree of coagulation and fibrinolysis activation after conventional multiport (CLC) and single-incision (SILC) laparoscopic cholecystectomy, we aimed to determine whether the reduced incision size induces a lower thrombophilic tendency. Thirty-two adult patients with noncomplicated symptomatic cholelithiasis were nonrandomly assigned to CLC or SILC. Prothrombin fragment 1 + 2 (F1 + 2), thrombin–antithrombin complexes (TAT), D-dimers, fibrinogen, and von Willebrand factor levels were measured at baseline, at 1st, and 24th hour, postoperatively. Twenty-six patients were finally included in the study. Fifteen patients underwent CLC (male/female: 5/10) and 11 underwent SILC (male/female: 1/10). There were no perioperative complications. An almost similar postoperative pattern and degree of activation of coagulation and fibrinolysis pathways was noted in both groups. No statistically significant differences were found between SILC and CLC for F1 + 2, TAT, D-dimers, fibrinogen, and von Willebrand factor levels, duration of surgery, length of hospital stay, and postoperative morbidity. A similar pattern and extent of coagulation and fibrinolysis activation is present in SILC and CLC, and therefore there is no difference in tendency for thrombosis. Thromboembolic prophylaxis should be considered in SILC as recommended for CLC, pharmacologic or mechanical, considering the hemorrhagic risk and the presence of additional thromboembolism risk factors. SILC appears to be a safe, feasible technique that can be recommended for its potential advantages in cosmesis and reduced incisional pain.

Successful treatment of chronic lymphocytic leukemia mutlifocal central nervous system involvement with ibrutinib

Central nervous system involvement (CNSi) is rare in the course of chronic lymphocytic leukemia (CLL). The frequency ranges from 0.8% to 1% [1], and it is often underreported. Diagnosis is challenging and there is no consensus on the optimal therapy or survival. CNSi manifests as either leptomeningeal infiltration or a focal parenchymal lesion, or both [1]. We describe the case of a CLL patient who progressed with parenchymal CNS involvement and was successfully treated with ibrutinib.

Diffuse connective tissue disorders in HIV-infected patients

Background: Human immunodeficiency virus (HIV) infection has been associated with various autoimmune disorders. Aim: To review the spectrum of diffuse connective tissue disorders (dCTD) in HIV-infected patients, in the context of highly active anti-retroviral therapy. Methods: Electronic search of the literature was performed using the terms HIV, AIDS, autoimmune, rheumatic/rheumatological, immune reconstitution inflammatory syndrome, Systemic Lupus Erythematosus, Diffuse Infiltrative Lymphocytosis Syndrome, Sjogren’s syndrome, vasculitis, Behçet’s disease, cryoglobulins, Henoch-Schönlein purpura, and antiphospholipid syndrome. Results: We reviewed the clinical manifestations, natural history and treatment of dCTDs, since the implementation of Highly Active Anti-Retroviral Therapy (HAART), and the emergence of new pathogenic mechanisms, such as the immune reconstitution inflammatory syndrome. Conclusions: Caution in differentiating clinical and laboratory findings of dCTDs from non-specific manifestations of acute and chronic HIV infection is warranted due to the common presentation. Patients with chronic infection and access to HAART have a normal life expectancy and dCTDs, although rare, must be correctly addressed. HAART alone or combined with immunosuppressive therapy result in favourable outcomes.

Overcoming heparin resistance in pregnant women with antithrombin deficiency: a case report and review of the literature

BackgroundThe risk of thromboembolic events during pregnancy in patients with antithrombin deficiency is increased. Preventing thromboembolic events during pregnancy in the case of antithrombin deficiency is still a matter of concern.Case presentationWe present a case of a 19-year-old primigravida Greek Pomak woman, who was diagnosed as having congenital antithrombin deficiency. She had a history of recurrent miscarriages and a family history of thrombosis. She was managed with adjusted doses of low molecular weight heparin throughout her pregnancy, with regular anti-Xa and antithrombin level monitoring. Prior to delivery and for 4 days after delivery she received human antithrombin III concentrate. She delivered a small for gestational age baby with no other complications. She required an increased dose of heparin due to heparin resistance.ConclusionsAntithrombin deficiency is associated with an increased risk of venous thromboembolic events with a 50% risk of thromboembolic events before the 50th year of life. It is a rare condition, so data concerning the optimal management during pregnancy are limited. The selection of patients who should receive low molecular weight heparin prophylaxis as well as dose intensity and monitoring are discussed. In our patient a conventional low molecular weight heparin dose proved to be inadequate at least at the laboratory level.