Hematology Reports

4 pages, 731 KiB

Open AccessCase Report

Germline CEBPA Mutation in Familial Acute Myeloid Leukemia

by Matilde Boada, Ana Inés Catalan, Carolin Ottati, Florencia Bentancour, Daniela Lens, Cecilia Guillermo and Sofia Grille

Hematol. Rep. 2021, 13(3), 9114; https://doi.org/10.4081/hr.2021.9114 - 4 Oct 2021

Cited by 12 | Viewed by 1926

Abstract

Myeloid Neoplasms with germline predisposition become part of 2016 World Health Organization (WHO) classification of hematological malignancies since 2016. CCAAT/enhancer binding protein-alpha (CEBPA) is a myeloid transcription factor located in chromosome 19q. Acute myeloid leukemia (AML) with biallelic mutations of CEBPA AML with [...] Read more.

Myeloid Neoplasms with germline predisposition become part of 2016 World Health Organization (WHO) classification of hematological malignancies since 2016. CCAAT/enhancer binding protein-alpha (CEBPA) is a myeloid transcription factor located in chromosome 19q. Acute myeloid leukemia (AML) with biallelic mutations of CEBPA AML with recurrent genetic abnormalities according to WHO classification. The inheritance of a germline CEBPA mutation predisposes to the development of AML with autosomal dominant inheritance. Familial CEBPA AML share characteristics with somatic CEBPA AML. However, a higher relapse incidence is reported. We present the case of a 46-years-old male with family history of acute leukemia who was diagnosed with single mutated CEBPA acute myeloid leukemia. The same mutation was found in two of his siblings. The clinical suspicion and proper diagnosis of familial cases is necessary, especially when a related allogenic transplant is indicated in order to select an adequate donor. Full article

5 pages, 299 KiB

Open AccessArticle

Low Diagnostic Accuracy and Inter-Observer Agreement on CT and MRI in Diagnosis of Spinal Fractures in Multiple Myeloma

by Viktor Dalen, Anne-Sofie Vegsgaard Olsen, Claude-Pierre Jerome, Jonn-Terje Geitung and Anders E. A. Dahm

Hematol. Rep. 2021, 13(3), 9037; https://doi.org/10.4081/hr.2021.9037 - 4 Oct 2021

Cited by 2 | Viewed by 1457

Abstract

Skeletal disease is common in multiple myeloma. We investigated the inter-observer agreement and diagnostic accuracy of spinal fractures diagnosed by computer tomography (CT) and magnetic resonance imaging (MRI) from 12 myeloma patients. Two radiologists independently assessed the images. CT, MRI, and other images [...] Read more.

Skeletal disease is common in multiple myeloma. We investigated the inter-observer agreement and diagnostic accuracy of spinal fractures diagnosed by computer tomography (CT) and magnetic resonance imaging (MRI) from 12 myeloma patients. Two radiologists independently assessed the images. CT, MRI, and other images were combined to a gold standard. The inter-observer agreement was assessed with Cohen’s kappa. Radiologist 1 diagnosed 20 malignant spinal fractures on CT and 26 on MRI, while radiologist 2 diagnosed 12 malignant spinal fractures on CT and 22 on MRI. In comparison the gold standard diagnosed 10 malignant spinal fractures. The sensitivity for malignant fractures varied from 0.5 to 1 for CT and MRI, and the specificity varied from 0.17 to 0.67. On MRI, the specificity for malignant spinal fractures was 0.17 for both radiologists. The inter-observer agreement for malignant spinal fractures on CT was −0.42 (Cohen’s kappa) and −0.13 for MRI, while for osteoporotic fractures it was −0.24 for CT and 0.53 for MRI. We conclude that malignant spinal fractures were over-diagnosed on CT and MRI. The inter-observer agreement was extremely poor. Full article

8 pages, 491 KiB

Open AccessConference Report

Abstract Book of the SOHO 3rd Italian Conference | 27–30 September 2021

by Claudio Cerchione, Giovanni Martinelli and Hagop Kantarjian

Hematol. Rep. 2021, 13(3), 9389; https://doi.org/10.4081/hr.2021.9389 - 28 Sep 2021

Viewed by 1122

Abstract

Bruton’s tyrosine kinase (BTK) inhibition is an emerging standard of care for Waldenström macroglobulinemia [...] Full article

12 pages, 580 KiB

Open AccessConference Report

How I Manage Systemic Light Chain Amyloidosis

by Gregory P. Kaufman and Claudio Cerchione

Hematol. Rep. 2021, 13(3), 9383; https://doi.org/10.4081/hr.2021.9383 - 24 Sep 2021

Viewed by 1049

Abstract

Light chain amyloidosis (AL) is a protein deposition disorder with a heterogenous pattern of organ involvement and dysfunction that varies by affected patient. Often diagnosed late after the onset of symptoms, appropriate and correct diagnosis, and prompt initial management, typically with anti-plasma cell [...] Read more.

Light chain amyloidosis (AL) is a protein deposition disorder with a heterogenous pattern of organ involvement and dysfunction that varies by affected patient. Often diagnosed late after the onset of symptoms, appropriate and correct diagnosis, and prompt initial management, typically with anti-plasma cell therapy targeting the underlying cell producing the aberrant light chain protein, can lead to significant improvement in patient symptoms, organ function and lifespan. With recent publication of phase III studies focused on AL, and a changing regulatory landscape providing greater availability of novel therapies, the management of AL is in some ways more complex with greater options to consider. In this clinical review, a discussion of the diagnosis, staging, and goals of therapy transitions to a focus on contemporary management questions to outline our clinical approach to multi-disciplinary management and long term follow up for patients with suspected or confirmed AL. Full article

3 pages, 489 KiB

Open AccessCase Report

Apixaban and Eftrenonacog Alfa Treatment in a Patient with Moderate Hemophilia B and Cardiovascular Disease

by Rita C. Santoro, Mariapia Falbo and Alessandro Ferraro

Hematol. Rep. 2021, 13(3), 9169; https://doi.org/10.4081/hr.2021.9169 - 24 Sep 2021

Cited by 5 | Viewed by 1297

Abstract

In developed countries, the life expectancy of patients with hemophilia (PwH) is now close to that of the unaffected male population. This means that these patients are at risk of developing age-related comorbidities, including cardiovascular disease. Managing cardiovascular disease in PwH patients can [...] Read more.

In developed countries, the life expectancy of patients with hemophilia (PwH) is now close to that of the unaffected male population. This means that these patients are at risk of developing age-related comorbidities, including cardiovascular disease. Managing cardiovascular disease in PwH patients can be particularly challenging, due to their high bleeding risk. To our knowledge, this is the first report of a male patient with moderate hemophilia B and hypertensive ischemic heart disease complicated by arrhythmia due to nonvalvular atrial fibrillation, who was treated with apixaban and left atrial appendage closure while receiving concomitant anti-hemorrhagic prophylaxis with eftrenonacog alfa. Full article

3 pages, 558 KiB

Open AccessCase Report

Transformation of Low-Grade Follicular Yymphoma with Partial Marginal Zone Differentiation: Two Cases

by Karen Nalbandyan, Daniel Benharroch, Anna Gurevitch and Itai Levi

Hematol. Rep. 2021, 13(3), 8896; https://doi.org/10.4081/hr.2021.8896 - 17 Sep 2021

Viewed by 1112

Abstract

Two cases of low-grade follicular lymphoma, with marginal zone differentiation and/or with high proliferation rate in one of them, are reported with transformation into high grade B-cell and B-lymphoblastic lymphomas. The contribution of these features to the transforming process, although previously described, is [...] Read more.

Two cases of low-grade follicular lymphoma, with marginal zone differentiation and/or with high proliferation rate in one of them, are reported with transformation into high grade B-cell and B-lymphoblastic lymphomas. The contribution of these features to the transforming process, although previously described, is infrequent, and has not been deciphered to date. Full article

2 pages, 365 KiB

Open AccessCommentary

Transfusion-Associated Graftversus-Host Disease: A Brief Comment on Blood Safety

by Palma Manduzio

Hematol. Rep. 2021, 13(3), 9280; https://doi.org/10.4081/hr.2021.9280 - 14 Sep 2021

Cited by 1 | Viewed by 934

Abstract

Dear Editor, A recent paper from Dr Elliot published in Transfusion, entitled ‘Missed irradiation of cellular blood components from vulnerable patients: Insight from 10 years of SHOT data’ updates on Transfusion-associated graft-versus-host disease and blood safety [...] Full article

6 pages, 3553 KiB

Open AccessArticle

COVID-19 and Blood Groups: A Six-Months Observational Study in Ferrara, Italy

by Salvatore Greco, Nicolò Fabbri, Alessandro Bella, Beatrice Bonsi, Alessandra Violi, Valeria Fortunato, Maurizio Govoni, Giuseppe Graldi and Angelina Passaro

Hematol. Rep. 2021, 13(3), 9177; https://doi.org/10.4081/hr.2021.9177 - 6 Sep 2021

Cited by 3 | Viewed by 1425

Abstract

The current literature still gives a little information about the relationships between the ABO blood group system and the immune response to the virus or the different disease outcomes. Hypothesizing the presence of a predisposition by some blood groups to COVID-19, we searched [...] Read more.

The current literature still gives a little information about the relationships between the ABO blood group system and the immune response to the virus or the different disease outcomes. Hypothesizing the presence of a predisposition by some blood groups to COVID-19, we searched for differences between patients towards the different outcomes of disease.We enrolled 330 inpatients with a diagnosis of COVID-19, determining both their ABO blood group system and Rh factor, collecting demographic, clinical and laboratory data. We searched for relationships with COVID-19 outcomes within an observation period of 180 days (Intensification of Care-IoC, Inhospital death, 180-days mortality). The most frequent ABO blood group was A (45.8%); a minor part was represented by group O (38.8%), B (11.5%), AB (3.9%). As for the Rh factor, 86.7% of patients were Rh-positive. There were no significant differences between blood groups and Rh factors as for age, length of hospital stays (LoS), or Charlson Comorbidity Index (CCI), nor we found significant relationships between the ABO groups and COVID-19 outcomes. A significant relation was found between AB group and IoC (p = 0.03) while as for the Rh factor, the patients with Rh factor positive died with less frequency during the stay (p = 0.03). Cox regression analyses showed substantial differences in the survival functions concerning the Rh factors. The Rh factor seems to be involved in the 180-day prognosis. The survival functions of patients with Rh factor positive show, in fact, significantly better curves when compared to those with Rh factor negative. Full article

4 pages, 858 KiB

Open AccessArticle

The Correlation between Hemostatic Parameters and Mortality Rate in Patients with Non-Small Cell Lung Cancer

by Noni Novisari Soeroso, Fannie Rizki Ananda, Ganda Samosir, Herman Hariman and Putri Chairani Eyanoer

Hematol. Rep. 2021, 13(3), 8361; https://doi.org/10.4081/hr.2021.8361 - 6 Sep 2021

Cited by 6 | Viewed by 1352

Abstract

The increasing level of hemostatic parameters and tumor markers were associated with cancer progression and poor prognosis, particularly in NSCLC. The objective of this study is to determine whether there was a correlation between hemostatic parameters and mortality rate in patients with NSCLC. [...] Read more.

The increasing level of hemostatic parameters and tumor markers were associated with cancer progression and poor prognosis, particularly in NSCLC. The objective of this study is to determine whether there was a correlation between hemostatic parameters and mortality rate in patients with NSCLC. This was a prospective analytical study with a pretest-posttest design which included 41 patients with diagnosis of NSCLC. Plasma levels of PT, APTT, TT, D-dimer, and fibrinogen were measured before initiation of chemotherapy and remeasured after 4 cycles or 6 cycles of chemotherapy, based on the clinical condition of patients. Then, patients were followed up for 1 year to evaluate the mortality rate. The majority of subjects were male (85.4%) with adenocarcinoma (75.6%). There was no significant difference in mean between adenocarcinoma and squamous cell carcinoma (p > 0.05). Most patients died after one month of follow up (61%). The parameters which could predict high mortality rate in NSCLC were prolonged PT and the increased of D-dimer with RR > 1, although they had not significant in statistical analysis (p > 0.05). There is no correlation between hemostatic parameters and mortality rate in patients with NSCLC. Full article

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Hematol. Rep., Volume 13, Issue 3 (September 2021) – 9 articles

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