Hematology Reports 2019-01-19T09:28:44+01:00 Emanuela Fusinato Open Journal Systems <p><strong>Hematology Reports</strong> is an Open Access, online-only, peer-reviewed journal that considers mainly original articles, case reports and reviews on all aspects of prevention, diagnosis and management of disorders of the blood, as well as related molecular and cell biology, genetics, pathophysiology, epidemiology and controlled trials. Manuscripts on laboratory and clinical aspects of blood-related conditions are welcome, as well as research on the treatment of blood diseases. Occasional issues and supplements publish an in-depth clinical and biological analysis of particular types of blood diseases. The journal also reviews important recent developments in the biology and treatment of malignant diseases, and highlights promising new directions.</p> Morphologic and immunophenotypic features of a case of acute monoblastic leukemia with unusual positivity for Glycophorin-A 2019-01-19T09:28:38+01:00 Giovanni Carulli Paola Sammuri Cristiana Domenichini Martina Rousseau Virginia Ottaviano Maria Immacolata Ferreri Antonio AzzarĂ Francesco Caracciolo Mario Petrini <p>Acute monoblastic leukemia (AMoL) is characterized by cells with highly undifferentiated morphology. Cytochemistry with non-specific esterases is negative in up to 20% of cases. Immunophenotyping by flow cytometry has an essential role in diagnosing such a subtype of leukemia and a multiparametric approach with a wide monoclonal antibody panel is necessary. We describe a case of AMoL with morphology resembling either plasma blasts or very immature erythroblasts. Diagnosis was made by alpha-naphtyl-acetate esterase staining and with immunophenotyping, which was made with a wide monoclonal antibody panel. Blasts were positive for monocytic markers. Most of leukemic cells, however, were positive for Glycophorin-A. The presence of Glycophorin-A, which is considered as a specific marker of the erythroid lineage, has never been reported previously in cases of AMoL. This peculiar immunophenotype might be interpreted as deriving from a common myelo-erythroid precursor undergone leukemic transformation.</p> 2018-12-12T08:29:49+01:00 ##submission.copyrightStatement## Pulmonary embolism in a patient with neuroleptic malignant syndrome 2019-01-19T09:28:40+01:00 Mosaad Almegren <p>Venous thromboembolism is one of the complications in patients prescribed antipsychotic medications. Neuroleptic malignant syndrome (NMS) is a rare side effect of antipsychotic medications in this population. In this case, a young patient, who presented with NMS after a recent start of antipsychotic medications, had developed a pulmonary embolism despite standard of care measures of venous thromboprophylaxis and early mobilization. A low threshold of VTE suspicion and effective preventive measures are both required in order to avoid this preventable complication in this population.</p> 2018-12-12T08:20:50+01:00 ##submission.copyrightStatement## Immunosuppression-associated posterior reversible encephalopathy syndrome in an acute leukemia case 2019-01-19T09:28:41+01:00 Umit Y. Malkan Gursel Gunes Haluk Demiroglu Hakan Goker <p>Posterior reversible encephalopathy syndrome (PRES) was described in 1996. Herein, we aimed to report an immunosuppression- related PRES case. A 34-year-old woman was diagnosed as t-cell acute lymphoblastic leukemia and allogeneic hematopoietic stem cell transplantation (HSCT) was performed. Cyclosporine was given for GVHD prophylaxis in addition to the other routine medications of HSCT. She was hospitalized for acute renal failure and due to the possible contribution of acute renal failure cyclosporine was stopped. Tacrolimus was started for GVHD prophylaxis at a dose of 1 mg/day. However, fifteen days after the initiation of tacrolimus, blurred vision occurred in our patient. Petechial bleeding sites were detected in bilateral cerebral and cerebellar hemisphere by MR imaging. Tacrolimus dosage was reduced to 0.5 mg/day. She had hypertension which was difficult to control and followed-up in the intensive care unit. She had seizures. Control cranial MR resulted as diffusion limitation in bilateral cerebellar hemisphere, bilateral occipital and frontal-parietal regions with vasogenic edema findings; contrast involvement in left frontal-parietal and right cerebellar regions. She had vision loss and lethargy. Control cranial MR favored PRES syndrome secondary to immunosuppression. Hypertensive state was taken under control with antihypertensive treatment and all immunosuppressive agents were stopped. Two weeks later her clinical condition was slightly improved. MR test which was conducted 2 weeks after the diagnosis revealed the regression of PRES lesions. The characteristic signs on neuroimaging are the symmetrical white matter edema in the posterior cerebral hemispheres, particularly the parietal- occipital regions. In conclusion, PRES rarely develops secondary to the immunosuppressive agents and the clinicians should suspect and promptly diagnose PRES which might cause otherwise serious irreversible clinical complications.</p> 2018-11-06T13:41:18+01:00 ##submission.copyrightStatement## Transfusion-associated graft-versus-host disease: A concise review 2019-01-19T09:28:43+01:00 Palma Manduzio <p>Transfusion-associated graft-versushost disease (TA-GVHD) represents a rare fatal event observed in immunocompromised patients and immunocompetent individuals. The main clinical features of this transfusion reaction are pancitopenia and multiorgan failure (skin, liver, gut). The possible pathogenesis includes donor T lymphocyte proliferation in blood, their engraftment and host tissue attack. The purpose of this narrative review was analyzing the international guidelines for irradiation of cellular blood components to prevent TA-GVHD. A literature search was conducted using PubMed articles published between January 2000 to July 2018. American, Australian, British and Japanese transfusion guidelines have been compared regarding clinical indications. The contribution of manuscripts has been focused on recipients of Haematopoietic Stem Cell Transplantation, severe cellular immunodeficient patients, fetuses and neonates, immunocompentent individuals. Furthermore, 348 cases of TA-GVHD in the last five decades have been documented according to a recent systematic review. The standard of care to prevent this complication is gamma or x irradiation of cellular blood products. New treatments with pathogen inactivation appear safe and effective against proliferating white blood cells and T cells. Further clinical and biological studies are necessary to better characterize immunocompetence of T cells and select alternative preventive strategies.</p> 2018-11-06T13:15:13+01:00 ##submission.copyrightStatement## NOTCH activation promotes glycosyltransferase expression in human myeloid leukemia cells 2019-01-19T09:28:44+01:00 Shichun Wang Mai Itoh Erika Shiratori Mika Ohtaka Shuji Tohda NOTCH signaling diversely regulates the growth of acute myeloid leukemia (AML) cells. It is known that glycosylation of NOTCH receptors modulates NOTCH activation. However, little is known about glycosylation of NOTCH in AML cells. We examined the effects of ligand-induced NOTCH activation on the expression of NOTCHmodifying glycosyltransferases in two AML cell lines, THP-1 and TMD7. The cells were stimulated with recombinant NOTCH ligands JAGGED1 and DELTA1, and subjected to immunoblot analysis to evaluate the expression levels of glycosyltransferases. Ligand stimulation promoted the expression of POFUT1, LFNG, MFNG, RFNG, GXYLT1, GXYLT2, and XXYLT1 in THP-1 cells, and that of RFNG and GXYLT1 in TMD7 cells. We found that NOTCH activation promoted the expression of several glycosyltransferases in AML cells. This suggests that NOTCH activation modulates its sensitivity to NOTCH ligands by increased glycosylation of NOTCH receptors in AML cells. Further investigation is needed to elucidate its biological significance. 2018-09-24T14:24:22+02:00 ##submission.copyrightStatement##