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Hematology Reports is published by MDPI from Volume 14 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.

Hematol. Rep., Volume 10, Issue 1 (March 2018) – 7 articles

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5 pages, 159 KiB  
Article
Chromosome Positioning in Interphase Nuclei of Hematopoietic Stem Cell and Myeloid Precursor
by Mariana Lomiento, Fabiana Mammoli, Emilia Maria Cristina Mazza, Silvio Bicciato and Sergio Ferrari
Hematol. Rep. 2018, 10(1), 7515; https://doi.org/10.4081/hr.2018.7515 - 3 Apr 2018
Cited by 6 | Viewed by 519
Abstract
Human myelopoiesis is an intriguing biological process during which multipotent stem cells limit their differentiation potential generating precursors that evolve into terminally differentiated cells. The differentiation process is correlated with differential gene expression and changes in nuclear architecture. In interphase, chromosomes are distinct [...] Read more.
Human myelopoiesis is an intriguing biological process during which multipotent stem cells limit their differentiation potential generating precursors that evolve into terminally differentiated cells. The differentiation process is correlated with differential gene expression and changes in nuclear architecture. In interphase, chromosomes are distinct entities known as chromosome territories and they show a radial localization that could result in a constrain of inter-homologous distance. This element plays a role in genome stability and gene expression. Here, we provide the first experimental evidence of 3D chromosomal arrangement considering two steps of human normal myelopoiesis. Specifically, multicolor 3D-FISH and 3D image analysis revealed that, in both normal human hematopoietic stem cells and myelod precursors CD14-, chromosomal position is correlated with gene density. However, we observed that inter-homologue distances are totally different during differentiation. This could be associated with differential gene expression that we found comparing the two cell types. Our results disclose an unprecedented framework relevant for deciphering the genomic mechanisms at the base of normal human myelopoiesis. Full article
4 pages, 628 KiB  
Case Report
Neurofibromatosis Type I and Multiple Myeloma Coexistence: A Possible Link?
by Fabrizio Accardi, Valentina Marchica, Cristina Mancini, Elena Maredi, Costantina Racano, Laura Notarfranchi, Davide Martorana, Paola Storti, Eugenia Martella, Benedetta Dalla Palma, Luisa Craviotto, Massimo De Filippo, Antonio Percesepe, Franco Aversa and Nicola Giuliani
Hematol. Rep. 2018, 10(1), 7457; https://doi.org/10.4081/hr.2018.7457 - 3 Apr 2018
Cited by 1 | Viewed by 482
Abstract
The association between Neurofibromatosis type I (NF1) and multiple myeloma (MM), a plasma cell, dyscrasia is very rare. Here we put to the attention of the scientific community two new cases. The first one is a patient with active MM whereas the second [...] Read more.
The association between Neurofibromatosis type I (NF1) and multiple myeloma (MM), a plasma cell, dyscrasia is very rare. Here we put to the attention of the scientific community two new cases. The first one is a patient with active MM whereas the second with smoldering MM. Both patients present typical features of NF1 but skeletal alterations were present only in the second case including dysplasia, marked scoliosis and osteoporosis. MM osteolytic lesions were absent in both patients. In addition to the clinical diagnosis of NF1, a molecular testing for NF1 gene mutations has been performed finding that patient one was heterozygous for the c.6855C>A (Tyr2285Ter) mutation, while patient two was heterozygous for the c.7838dupC (Lys2614GlufsTer20) mutation. The two mutations were diagnosed both in genomic DNA from peripheral blood and from MM cells. The potential link between NF1 mutation and the increased risk of MM is discussed in the report. Full article
6 pages, 654 KiB  
Article
Extranodal Diffuse Large B-Cell Lymphomas: A Retrospective Case Series and Review of the Literature
by Stergios Boussios, Ioannis Zerdes, Amalia Vassou, Eleni Bareta, Esmeralda Seraj, Alexandra Papoudou-Bai, Nicholas Pavlidis, Anna Batistatou and George Pentheroudakis
Hematol. Rep. 2018, 10(1), 7070; https://doi.org/10.4081/hr.2018.7070 - 3 Apr 2018
Cited by 18 | Viewed by 592
Abstract
Non-Hodgkin lymphomas commonly show extranodal involvement (25–30%) but primary diffuse large B-cell lymphomas (DLBCL) with extranodal localization represent clinically and molecularly distinct entities. The present study involved retrospective analysis of case records of 4 patients who were diagnosed with extranodal DLBCL between 2010 [...] Read more.
Non-Hodgkin lymphomas commonly show extranodal involvement (25–30%) but primary diffuse large B-cell lymphomas (DLBCL) with extranodal localization represent clinically and molecularly distinct entities. The present study involved retrospective analysis of case records of 4 patients who were diagnosed with extranodal DLBCL between 2010 and 2016 at the Medical Oncology and Hematology Departments of the Ioannina University Hospital, Greece. Median age of presentation was 69 years (range 60–77 years). There were 2 males and 2 females. The sites of DLBCL involvement included adrenal gland, mandible, cervix uteri, and ileum. Two patients had B symptoms while none had bone marrow involvement. After staging workup, all the patients fell into IE stage. The treatment approach included chemotherapy combined with rituximab (R), whereas one patient received additionally irradiation therapy. Post-treatment positron emission tomography-computed tomography scan was performed in 3 patients. In terms of the outcome, 3 patients are alive in complete response, whereas one was lost in follow-up. Further prospective data analyses are required so as to better elucidate the biology and course of extranodal DLBCL. Full article
3 pages, 574 KiB  
Case Report
Paroxysmal Nocturnal Hemoglobinuria: When Delay in Diagnosis and Long Therapy Occurs
by Salvatrice Mancuso, Giuseppe Sucato, Melania Carlisi, Marco Santoro, Giuseppe Tarantino, Emilio Iannitto, Mariasanta Napolitano and Sergio Siragusa
Hematol. Rep. 2018, 10(1), 7523; https://doi.org/10.4081/hr.2018.7523 - 29 Mar 2018
Cited by 5 | Viewed by 511
Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disorder characterized by hemolytic anemia, bone marrow failure and thrombosis, caused by a somatic mutation in PIG-A gene that results in the absence of CD55 and CD59, two important complement regulatory proteins. In this paper, [...] Read more.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disorder characterized by hemolytic anemia, bone marrow failure and thrombosis, caused by a somatic mutation in PIG-A gene that results in the absence of CD55 and CD59, two important complement regulatory proteins. In this paper, a case of PNH is retrospectively examined looking for clinical and laboratory features, and the entire course of the disease from the onset of the symptoms is described, together with an adequate follow-up over a 7-years treatment period. In this case, the not specificity and the limited clinical relevance of the symptoms led to a delay in diagnosis. After thrombosis, Eculizumab therapy has been shown to be effective, and during seven years of follow-up no events have occurred that put the patient’s life at risk. A multidisciplinary approach is crucial in cases like this, in order to allow early diagnosis and minimize the risks for the patients. Full article
6 pages, 675 KiB  
Article
Acute Monocytic Leukemia Diagnosed by Flow Cytometry Includes Acute Myeloid Leukemias with Weakly or Faintly Positive Non-Specific Esterase Staining
by Yuriko Zushi, Miho Sasaki, Ayano Mori, Toshiharu Saitoh, Takae Goka, Yumi Aoyama, Yuta Goto, Hiroko Tsunemine, Taiichi Kodaka and Takayuki Takahashi
Hematol. Rep. 2018, 10(1), 7435; https://doi.org/10.4081/hr.2018.7435 - 29 Mar 2018
Cited by 8 | Viewed by 540
Abstract
A diagnosis of acute monocytic leukemia (AML-M5) based on α-naphthyl butyrate esterase (α-NB) staining has some problems, because AML-M5 leukemic cells often show weak or faint positivity on α-NB staining. In these situations, some cases of AML-M5 tend to be misdiagnosed as AML-M0. [...] Read more.
A diagnosis of acute monocytic leukemia (AML-M5) based on α-naphthyl butyrate esterase (α-NB) staining has some problems, because AML-M5 leukemic cells often show weak or faint positivity on α-NB staining. In these situations, some cases of AML-M5 tend to be misdiagnosed as AML-M0. Therefore, we evaluated the significance of weak or faint α-NB staining in AML-M5 diagnosed by flow cytometry (FCM). Nineteen AML cases in which leukemic cells were negative for naphthol AS-D chloroacetate esterase staining were studied. For FCM, we defined leukemic cells as having a monocytic nature when more than 10% of the leukemic cells were positive for at least one of the following antigens: CD4, CD11c, CD14, and CD64. The monocytic nature determined by FCM was consistent with positive or weak positivity on α-NB staining. Five of 6 cases in which leukemic cells exhibited faint positivity for α-NB staining could be diagnosed as AML-M5 by FCM, while negative α-NB staining was consistent with a diagnosis of AML-M0. These results suggest that AML-M5 should be taken into consideration even when leukemic cells are faintly positive for α-NB staining. Full article
6 pages, 677 KiB  
Review
Bone-Targeted Agents in Multiple Myeloma
by Hiroko Nishida
Hematol. Rep. 2018, 10(1), 7401; https://doi.org/10.4081/hr.2018.7401 - 29 Mar 2018
Cited by 13 | Viewed by 504
Abstract
Osteolytic bone disease, characterized by bone pain, increased risk of pathologic fractures, tumor-induced hypercalcemia known as skeletal-related events (SREs), is a frequent complication of patients with multiple myeloma (MM) and persists even in the absence of active disease, resulting in a major cause [...] Read more.
Osteolytic bone disease, characterized by bone pain, increased risk of pathologic fractures, tumor-induced hypercalcemia known as skeletal-related events (SREs), is a frequent complication of patients with multiple myeloma (MM) and persists even in the absence of active disease, resulting in a major cause of morbidity and mortality. The interaction between myeloma cells and their surrounding cells in the bone marrow (BM) microenvironment promotes both myeloma cell growth and bone destruction and forms the vicious cycle of MM bone disease. Therefore, therapeutic strategies targeting the interaction between myeloma cells and cellular components including osteoclasts (OCs), stromal cells and osteoblasts (OBs) in the BM is crucial not only to attain tumor regression but also to prevent or delay the incidence of SREs, which leads to improve survival and quality of life in affected patients. Recently, several novel targets which act on components of the cycle for treating MM-associated bone disease have been identified in addition to current treatments including nitrogen-containing bisphosphonates. This review focuses on the overview of pathophysiology in MM-associated bone disease and summarizes its current clinical management. Several novel bone-targeted agents in preclinical setting will be also discussed.
Full article
4 pages, 709 KiB  
Case Report
Congenital Methemoglobinemia Misdiagnosed as Polycythemia Vera: Case Report and Review of Literature
by Dina Sameh Soliman and Mohamed Yassin
Hematol. Rep. 2018, 10(1), 7221; https://doi.org/10.4081/hr.2018.7221 - 2 Mar 2018
Cited by 24 | Viewed by 1475
Abstract
Methemoglobinemia is a rare overlooked differential diagnosis in patients presented with cyanosis and dyspnea unrelated to cardiopulmonary causes. Our patient is 29 year old Indian non-smoker male, his story started 6 months prior to presentation to our center when he had generalized fatigue [...] Read more.
Methemoglobinemia is a rare overlooked differential diagnosis in patients presented with cyanosis and dyspnea unrelated to cardiopulmonary causes. Our patient is 29 year old Indian non-smoker male, his story started 6 months prior to presentation to our center when he had generalized fatigue and discoloration of hands. He presented with persistent polycythemia with elevated hemoglobin level. The patient was misdiagnosed in another center as polycythemia and treated with Imatinib. The diagnosis of PV was revisited and ruled out in view of negative JAK2, normal erythropoietin level and absence of features of panmyelosis. Clinical cyanosis and lowoxygen saturation in the presence of normal arterial oxygen tension was highly suggestive of methemoglobinemia. Arterial blood gas revealed a methemoglobin level of 38% (normal: 0–1.5%). Cytochrome B5 reductase (Methemoglobin reductase B) was deficient at level of <2.6 U/g Hb) (normal: 6.6–13.3), consistent with methemoglobin reductase (cytochrome b5) deficiency and hence the diagnosis of congenital methemoglobinemia was established. The role of Imatinib in provoking methemoglobinemia is questionable and association between Imatinib and methemoglobinemia never described before. In our case, there were no other offending drugs in aggravating the patients’ symptoms and cyanosis. The patient started on Vitamin C 500 mg once daily for which he responded well with less cyanosis and significant reduction of methemoglobin level. Congenital methemoglobinemia is a rare underreported hemoglobin disease and often clinically missed. Upon extensive review of English literature for cases of congenital methemoglobinemia due to deficiency of cytochrome b5 reductase, we found 23 cases diagnosed as type I (including the case reported here). 17 cases (~74%) of type I and 6 cases (27%) of type II. There is male predominance 73% versus 26% in females. Almost half of reported cases 12 cases (52%) are Indian, 2 Japanese, 3 English, 2 Arabic, one case Spanish and one case Italian. For type I, the median calculated age is 31 years with cyanosis and shortness of breath being the most common sign and symptoms. For type II: Six cases were reported in English literature, all in pediatric age group with median calculated age at presentation is 6 years with neurologic manifestations and mental retardation are the most common type II associated symptoms. Due to lack of systematic epidemiological studies, congenital methemoglobinemia is under diagnosed as it is under investigated and usually overlooked especially when presenting in adulthood and in absence of obvious acquired agents. Full article
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