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Hematology Reports
Volume 13
Issue 2
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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 13, Issue 2 (June 2021) – 6 articles

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4 pages, 602 KiB  
Case Report
The Diagnostic Role of Next Generation Sequencing in Uncovering Isolated Splenomegaly: A Case Report
by Giuseppe Auteri, Daniela Bartoletti, Clara Bertuzzi, Francesco Bacci, Valeria Tonini, Lucia Catani, Nicola Vianelli, Michele Cavo and Francesca Palandri
Hematol. Rep. 2021, 13(2), 8814; https://doi.org/10.4081/hr.2021.8814 - 21 Jun 2021
Viewed by 753
Abstract
Many diseases can induce splenomegaly, however, about 5% of splenomegalies are idiopathic. When there is no underlying treatable cause, and the splenomegaly significantly affects the quality of life, splenectomy is the best therapeutic choice. A 67-year-old woman had idiopathic and asymptomatic splenomegaly. The [...] Read more.
Many diseases can induce splenomegaly, however, about 5% of splenomegalies are idiopathic. When there is no underlying treatable cause, and the splenomegaly significantly affects the quality of life, splenectomy is the best therapeutic choice. A 67-year-old woman had idiopathic and asymptomatic splenomegaly. The increase in splenomegaly resulted in hypersplenism with cytopenia and symptoms related to abdominal discomfort. The patient underwent splenectomy which led to clinical improvement. A histological examination showed the presence of hematopoietic tissue. Peripheral blood Next Generation Sequencing with the myeloid panel SOPHiA Genetics showed the following mutations: ASXL1, SRSF2, KRAS and TET2. Three out of these four mutations were also found in the splenic tissue. Next Generation Sequencing could be useful in the diagnosis of splenomegalies associated with myeloproliferative neoplasms otherwise defined as idiopathic, in order to address a therapeutic strategy. Full article
5 pages, 871 KiB  
Article
Immune Checkpoint Inhibitors in Combination with Radiotherapy as Salvage Treatment for Relapsed/Refractory Classical Hodgkin Lymphoma: A retrospective Analysis in 12 Patients
by Elisa Lucchini, Chiara Rusconi, Mario Levis, Francesca Ricci, Armando Santoro, Umberto Ricardi, Stefano Volpetti, Fabio Matrone, Anna di Russo, Manuela Caizzi, Anna Schiattarella and Francesco Zaja
Hematol. Rep. 2021, 13(2), 9080; https://doi.org/10.4081/hr.2021.9080 - 09 Jun 2021
Cited by 6 | Viewed by 1130
Abstract
The rate of complete remission (CR) with the anti-PD1 immune checkpoint inhibitors (ICI) nivolumab (N) and pembrolizumab (P) in patients with relapsed/refractory (R/R) classical Hodgkin lymphoma (cHL) is low (20–30%), and the majority of patients eventually relapse. One strategy to improve their outcome [...] Read more.
The rate of complete remission (CR) with the anti-PD1 immune checkpoint inhibitors (ICI) nivolumab (N) and pembrolizumab (P) in patients with relapsed/refractory (R/R) classical Hodgkin lymphoma (cHL) is low (20–30%), and the majority of patients eventually relapse. One strategy to improve their outcome is to combine ICI with radiotherapy (ICI-RT), taking advantage of a supposed synergistic effect. We retrospectively collected data of 12 adult patients with R/R cHL treated with ICI-RT delivered during or within 8 weeks from the start or after the end of ICI. Median age at ICI-RT was 37 years, 50% had previously received an autologous stem cell transplantation (SCT) and 92% brentuximab vedotin. RT was given concurrently, before or after ICI in 4, 1 and 7 patients. Median RT dose was 30 Gy, for a median duration of 22 days. Median number of ICI administrations was 15. Overall response and CR rate were 100% and 58%. Nine patients received subsequent SCT consolidation (7 allogeneic and 2 autologous). After a median follow-up of 18 months, 92% of patients were in CR. No major concerns about safety were reported. ICI-RT combination appears to be a feasible and highly active bridge treatment to transplant consolidation. Full article
5 pages, 644 KiB  
Article
The Risk of Venipuncture in Newborn with Severe Hemophilia: Case Report of a Large Elbow Hemorrhage and Literature Review of Compartment Syndrome
by Giuseppe Lassandro, Anna Amoruso, Valentina Palladino, Viviana Valeria Palmieri and Paola Giordano
Hematol. Rep. 2021, 13(2), 8967; https://doi.org/10.4081/hr.2021.8967 - 09 Jun 2021
Cited by 3 | Viewed by 927
Abstract
Hemophilias are hemorrhagic congenital rare diseases. The gold standard of therapy in hemophilics is the intravenously replacement therapy. We can infuse intravenously plasma derived factors (FVIII for Hemophilia A and FIX for Hemophilia B) or recombinant products (i.e., clotting factor synthetically produced). Venipuncture [...] Read more.
Hemophilias are hemorrhagic congenital rare diseases. The gold standard of therapy in hemophilics is the intravenously replacement therapy. We can infuse intravenously plasma derived factors (FVIII for Hemophilia A and FIX for Hemophilia B) or recombinant products (i.e., clotting factor synthetically produced). Venipuncture is not a safe procedure in subjects with hemorrhagic diseases. It is considered an invasive technique with potential massive bleeding and it requires standardized procedures to prevent complications. Local pressure after the procedure (with eventually ice rest) must be always done. In case of bleeding a rapid replacement therapy must be conducted. A severe complication in hemophilia is compartment syndrome. We report a case of massive bleeding in a hemophilic newborn after venipuncture and a literature review of compartment syndrome in hemophiliacs. The aim of this paper is to help physicians in the clinical management to prevent the evolution of a massive bleeding in compartment syndrome. Full article
3 pages, 280 KiB  
Case Report
Successful Use of Phlebotomy to Treat Severe Secondary Polycythemia Due to Chronic Lung Disease
by Jacob Fuqua, Josephine Reece and Sarah Sofka
Hematol. Rep. 2021, 13(2), 8961; https://doi.org/10.4081/hr.2021.8961 - 09 Jun 2021
Cited by 2 | Viewed by 1233
Abstract
Secondary polycythemia (SP) occurs as a result of increase erythropoietin levels most commonly as a result of tissue hypoxia. Symptoms such as erythromelalgia, pruritis, and bleeding, which are frequently seen in polycythemia vera (PV), do not commonly occur in SP. Phlebotomy is considered [...] Read more.
Secondary polycythemia (SP) occurs as a result of increase erythropoietin levels most commonly as a result of tissue hypoxia. Symptoms such as erythromelalgia, pruritis, and bleeding, which are frequently seen in polycythemia vera (PV), do not commonly occur in SP. Phlebotomy is considered one of the mainstays of therapy for PV but is rarely used for treatment of SP due to concern about worsening tissue hypoxia. We present the case of a patient with severe SP due to chronic hypoxic lung disease who presented with erythromelalgia, pruritis, and bleeding and was treated successfully with therapeutic phlebotomy. This case illustrates the importance of considering the use of therapeutic phlebotomy in symptomatic patients with severe SP. Full article
4 pages, 1577 KiB  
Case Report
Kikuchi-Fujimoto Disease Involving Retroperitoneal Lymph Nodes: An Uncommon Presentation
by Jane E. Hon, Maria E. Vergara-Lluri, Imran Siddiqi, Christopher Foss, Donald I. Feinstein and Russell I. Brynes
Hematol. Rep. 2021, 13(2), 9001; https://doi.org/10.4081/hr.2021.9001 - 05 Mar 2021
Cited by 1 | Viewed by 682
Abstract
Kikuchi-Fujimoto disease is a self-limited disease of unknown etiology that is clinically defined by fevers accompanied by tender posterior cervical lymphadenopathy. It often presents acutely or sub-acutely, and due to its non-specific features, the differential diagnosis is broad and includes infectious, autoimmune, and [...] Read more.
Kikuchi-Fujimoto disease is a self-limited disease of unknown etiology that is clinically defined by fevers accompanied by tender posterior cervical lymphadenopathy. It often presents acutely or sub-acutely, and due to its non-specific features, the differential diagnosis is broad and includes infectious, autoimmune, and malignant causes. Although cases of extra-cervical disease are not uncommon, involvement of retroperitoneal lymph nodes has only rarely been reported. Here, we describe a patient with Kikuchi-Fujimoto disease who presented with fever of unknown origin, abdominal pain, and enlarged hypermetabolic retroperitoneal lymph nodes. Full article
4 pages, 597 KiB  
Case Report
Long-Term and Quality of Survival in Patients Treated for Acute Lymphoblastic Leukemia during the Pediatric Age
by Lara Devilli, Chiara Garonzi, Rita Balter, Elisa Bonetti, Matteo Chinello, Ada Zaccaron, Virginia Vitale, Massimiliano De Bortoli, Giulia Caddeo, Valentina Baretta, Gloria Tridello and Simone Cesaro
Hematol. Rep. 2021, 13(2), 8847; https://doi.org/10.4081/hr.2021.8847 - 05 Mar 2021
Cited by 3 | Viewed by 756
Abstract
Long-term survival for acute lymphoblastic leukemia (ALL) in children improved over the last three decades up to 80–90% of affected patients. Consequently, the quality of life of survivors has become increasingly important. This study analyses the clinical features and outcome of 119 children [...] Read more.
Long-term survival for acute lymphoblastic leukemia (ALL) in children improved over the last three decades up to 80–90% of affected patients. Consequently, the quality of life of survivors has become increasingly important. This study analyses the clinical features and outcome of 119 children with ALL, focusing on the quality of long-term survival in a subset of 22 patients over 18 years of age. Among this group, the 10-year event-free survival and overall survival were 83.1% (C.I. 74.0–89.2) and 88.4% (C.I. 80.9–93.1), respectively. Treatment related long-term medical complications were reported only in 2 patients (9.1%). Secondary school was completed successfully in 20 of 22 patients (89.9%). The remaining 2 patients were still attending at the time of the analysis. In conclusion, current treatment for ALL is well tolerated and does not compromise significantly the quality of life of survivors. Full article
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