Filiz Vural

Hemophagocytic syndrome associated with inappropiate secretion of antidiuretic hormone in lymphoma and acute myeloblastic leukemia: Report of two cases

Hemophagocytic syndrome (HPS) is a rare clinicopathological disorder characterized by systemic proliferation of phagocytizing histiocytes associated with fever, cytopenias, lymphadenopathy, hepatosplenomegaly, and disseminated intravascular coagulopathy. We present the association of hemophagocytic syndrome associated with inappropriate secretion of antidiuretic hormone (SIADH) in two cases of hematological malignancies; anaplastic large cell lymphoma (ALCL) and acute myeloblastic leukemia (AML M4) In the patient with lymphoma, the diagnosis of lymphoma, HPS and SIADH were concurrent. In the patient with AML, HPS and SIADH were observed while the patient was in hematological remission. Thus it seems that patients with HPS may also carry a risk for the development of SIADH; the relationship with HPS and SIADH should be further investigated.

Gingival involvement in a patient with CD56+ chronic myelomonocytic leukemia

Leukemic infiltration of the gingiva is most commonly reported to be associated with monocytic subtypes of acute myeloblastic leukemia (AML) but rarely with myelodysplastic syndromes (MDS). Here we report a case of CD56 +  chronic myelomonocytic leukemia (CMML) who developed gingival involvement simultaneously when the leukocyte count elevated. At that time no increase in peripheral or bone marrow blasts were observed. Gingival hypertrophy regressed with the treatment of hydroxyurea. Three months later, bone marrow blast count elevated and the patient was treated with two courses of AML-like regimen and then one course of consolidation therapy. The patient is in complete hematological remission for one and a half years. Similar to other extramedullary involvements, gingival hypertrophy in CMML can be a harbinger of the disease entering a more aggressive phase requiring systemic chemotherapy.

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

Abstract 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.

EBV-associated nasal-type T/natural killer cell lymphoma presenting with polyserositis and rhabdomyolysis

Nasal-type T/natural killer (NK)-cell lymphoma, a distinct clinicopathological entity is highly associated with Epstein-Barr virus which shows an aggressive course. We present a CD56+ nasal-type T/(NK)-cell lymphoma case with systemic manifestations of rhabdomyolysis and polyserositis who died of multiorgan failure shortly after his admission to hospital in spite of adequate chemotherapy and supportive care.

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

The clearance time of infused hematopoietic stem cell from the blood circulation.

There is no detailed information about the clearance time of infused hematopoietic stem cell (HSC) from the blood circulation in humans. In this prospective study, peripheral blood CD34+ cell counts were detected during the 4days period following autologous HSC transplantation in 20 patients by means of flow cytometry. The median CD34+ cells were at the highest level in the first hour and decreased below pre-infusion values on the first day after HSC infusion. By nonparametric analysis, positive correlation was found between CD34+ cell levels at the first hour and the post-thaw CD34+ cell dose (r=0.57, p=0.01). An inverse correlation was determined between CD34+ cell levels at the first hour and neutrophil engraftment (r=-0.54, p=0.01). Compared with the patients having CD34+ cell count of ⩾2μL(-1) in the first hour following HSC infusion, the patients having CD34+ cell count of <2μL(-1) had delayed both neutrophil (20 vs. 12, p=0.008) and platelet (47 vs. 11, p=0.01) engraftments. Our results indicated that infused HSCs were removed from the blood circulation within 1day. In addition, CD34+ cell levels at the first hour may be used as an important indicator to predict the delay of neutrophil and platelet engraftments.

Chronic myelogenous leukemia exhibiting trisomy 14 due to a Robertsonian translocation with philadelphia chromosome

[15] Ulrich-Pur H, Penz M, Fiebiger WC, Schu¨ll B, Kornek GV,Scheithauer W, et al. Oxaliplatin-induced fever and release ofIL-6. Oncology 2000;59:187 9.[16] Tonini G, Santini D, Vincenzi B, Borzomati D, Dicuonzo G,La Cesa A, et al. Oxaliplatin may induce cytokine-releasesyndrome in colorectal cancer patients. J Biol Regul Home-ost Agents 2002;16:105 9.[17] Lim KH, Huang MJ, Lin HC, Su YW, Chang YF, Lin J,et al. Hypersensitivity reactions to oxaliplatin: A case reportand the success of a continuous infusional desensitizationschedule. Anticancer Drugs 2004;15:605 7.[18] Edmondson DA, Gruling BJ, Urmanski AM, Wong SJ, LevyMB. Oxaliplatin hypersensitivity: Case report and successfulrepeat desensitization. Am J Ther 2007;14:116 8.

Epidemiology and analysis of invasive fungal infections in patients withhematological malignancies: a single-center real-life experience

Background/aim: Invasive fungal infection (IFI) causes morbidity and mortality among patients with hematological malignancies. We evaluated the incidence and treatment characteristics of IFIs between October 2012 and December 2013. Materials and methods: Patients who received chemotherapy or stem cell transplantation were retrospectively evaluated. Fungal infections were classified according to EORTC criteria.Results: Prophylaxis and antifungal therapy were given in 30.5% and 23.6% of 522 chemotherapy courses, respectively. The incidence of proven/probable IFI was 6.7%. The incidence of IFI among patients who received prophylaxis was significantly higher than among those who did not receive it (11.3% vs. 4.6%, P = 0.005). There was no significant difference between patients who received mold-active and no mold-active prophylaxis (P = 0.098). The most common single agent therapy and causative pathogen was liposomal amphotericin B (57.1%) and Aspergillus (n = 5), respectively. IFI-attributable mortality rate was 14.2% in 6 weeks.Conclusion: The IFI incidence and mortality rate were similar to that reported in the literature. The IFI rate was higher in the group using prophylaxis, as this is a high-risk group. Although the IFI rate was not significantly different between groups using prophylaxis, patients should be followed closely for the effective use of posaconazole prophylaxis.

Extramedullary Relapse in a CML Patient after Allogeneic Stem Cell Transplantation

Myeloid or granulocytic sarcoma (GS) is a tumoral lesion consisting of immature granulocytic cells. It is a rare entity during the course of CML patients especially after allogeneic stem cell transplantation (SCT). Relapse without bone marrow involvement is much rarer. We report a case of CML patient who relapsed with isolated granulocytic sarcoma after allogeneic SCT during cytogenetic and molecular remission. 28-year-old male was diagnosed as CML and allogeneic SCT was performed because of refractory disease to tyrosine kinase inhibitors. Complete cytogenetic and molecular response was achieved after allogeneic SCT followed by dasatinib treatment. Approximately 5 years after the transplantation, very rapidly progressive lesion was documented and diagnosed as GS although he was at molecular and cytogenetic remission. The patient died during chemotherapy due to sepsis. GS relapse after allogeneic SCT is a very rare type of relapse in CML patients with molecular and cytogenetic remission. Since it is a very aggressive disease with a poor prognosis, combined chemoradiotherapies with other possible options like DLI or second allogeneic SCT should be considered as soon as the diagnosis is confirmed.

FAS/FASL gene polymorphisms in Turkish patients with chronic myeloproliferative disorders

Introduction Chronic myeloproliferative disorders (CMPD) are chronic myeloid hematological disorders, characterized by increased myeloid cell proliferation and fibrosis. Impaired apoptotic mechanisms, increased cell proliferation, uncontrolled hematopoietic cell proliferation and myeloaccumulation may contribute to the pathogenesis of CMPD. The aim of our study was to show the possible role of FAS/FASL gene polymorphisms in CMPD pathogenesis and investigate the association with clinical parameters and susceptibility to disease. Material and methods We included 101 (34 polycythemia vera (PV), 23 primary myelofibrosis (PMF), 44 essential thrombocythemia (ET)) CMPD patients diagnosed according to the WHO classification criteria and 95 healthy controls in this study. All the patients and the controls were investigated for FAS/FASL gene expression, allele frequencies and phenotype features, and also FAS mRNA levels were analyzed. Results Chronic myeloproliferative disorders patients showed increased FAS-670AG + GG genotype distribution compared with the control group (p < 0.05). While the A allele was more frequent in both groups, AG genotype was more frequent in CMPD patients. There was no association between FAS-670A>G gene polymorphism and some clinical parameters such as splenomegaly and thrombosis (p > 0.05). No statistically significant difference in FASL+843C>T genotype or allele frequency was found between groups (p > 0.05). Moreover, no statistically significant difference was detected in FASL and JAK2V617F mutations (p > 0.05). FAS mRNA expression was 1.5-fold reduced in patients compared to healthy subjects. Conclusions According to our findings, FAS/FASL gene expression may contribute to the molecular and immunological pathogenesis of CMPD. More investigations are needed to support these data.