The challenging task of enumerating blasts in the bone marrow
Enumeration of blasts in the bone marrow is critical for diagnostic, prognostic, and therapeutic response evaluation in myelodysplastic syndromes, myeloproliferative neoplasms and acute leukemias. However, few studies have examined the accuracy and precision of marrow blast counting using standard microscopic procedures. In our study, four experienced hematopathologists evaluated blast percentages in marrow using either differential counts on aspirate smears or visual estimates on CD34-stained trephine biopsies. (Source: Seminars in Hematology)
Source: Seminars in Hematology - July 15, 2018 Category: Hematology Authors: Aaron Hodes, Katherine R. Calvo, Alina Dulau, Irina Maric, Junfeng Sun, Raul Braylan Source Type: research
The current approach to the diagnosis of myelodysplastic syndromes ☆
Myelodysplastic syndromes (MDS) are heterogeneous hematopoietic neoplasms that share key clinical and biologic features, including peripheral blood cytopenias, morphologic dysplasia, ineffective and clonal hematopoiesis, and a variable risk of transformation to acute myeloid leukemia. Diagnostic evaluation of MDS relies on morphologic assessment of the peripheral blood and bone marrow, conventional cytogenetics, and exclusion of secondary causes of dysplasia. The diagnosis of MDS has important clinical consequences, but it can be challenging and requires distinction from mimics. (Source: Seminars in Hematology)
Source: Seminars in Hematology - June 23, 2018 Category: Hematology Authors: Olga K. Weinberg, Robert P. Hasserjian Tags: Review Source Type: research
‘The Current Approach to the Diagnosis of Myelodysplastic Syndromes’
Myelodysplastic syndromes (MDS) are heterogeneous hematopoietic neoplasms that share key clinical and biologic features, including peripheral blood cytopenias, morphologic dysplasia, ineffective and clonal hematopoiesis, and a variable risk of transformation to acute myeloid leukemia (AML). Diagnostic evaluation of MDS relies on morphologic assessment of the peripheral blood and bone marrow, conventional cytogenetics, and exclusion of secondary causes of dysplasia. The diagnosis of MDS has important clinical consequences, but it can be challenging and requires distinction from mimics. (Source: Seminars in Hematology)
Source: Seminars in Hematology - June 23, 2018 Category: Hematology Authors: Olga K. Weinberg, Robert P Hasserjian Tags: Review Source Type: research
Acquired and Germline Predisposition to Bone Marrow Failure: Diagnostic features and clinical implications
Bone marrow failure and related syndromes are rare disorders characterized by ineffective bone marrow hematopoiesis and peripheral cytopenias. Although many are associated with characteristic clinical features, recent advances have shown a more complicated picture with a spectrum of broad and overlapping phenotypes and imperfect genotype – phenotype correlations. Distinguishing acquired from inherited forms of marrow failure can be challenging, but is of crucial importance given differences in the risk of disease progression to myelodysplastic syndrome, acute myeloid leukemia, and other malignancies, as well as the poten...
Source: Seminars in Hematology - June 23, 2018 Category: Hematology Authors: Michael E. Kallen, Alina Dulau-Florea, Katherine R. Calvo Tags: Review Source Type: research
C3-mediated extravascular hemolysis in PNH on eculizumab: mechanism and clinical implications
The introduction of eculizumab, a human monoclonal antibody against the C5 component of complement, has changed radically the management of paroxysmal nocturnal hemoglobinuria (PNH). The blockade of the terminal complement pathway by eculizumab eculizumab abrogates intravascular hemolysis, reduces the transfusion requirement and the risk of thrombosis in most of hemolytic PNH patients. However, in almost all PNH patients on eculizumab arises a fraction of PNH red cells that bind fragments of C3 and become a potential target of phagocytosis by macrophages. (Source: Seminars in Hematology)
Source: Seminars in Hematology - June 5, 2018 Category: Hematology Authors: Rosario Notaro, Michela Sica Source Type: research
Differences in presenting features, outcome and prognostic models in patients with Primary Myelofibrosis and post Polycythemia vera/post Essential Thrombocythemia Myelofibrosis treated with ruxolitinib. New perspective of the MYSEC-PM in a large multicenter study
Recently, the Myelofibrosis Secondary to PV and ET Prognostic Model (MYSEC-PM) was introduced to assess prognosis in myelofibrosis (MF) secondary to Polycythemia Vera and Essential Thrombocythemia (post-PV/post-ET MF), replacing the IPSS/DIPSS that was applied for primary MF (PMF). In a cohort of 421 ruxolitinib-treated patients (post-PV/post-ET MF: 44.2%), we evaluated: 1) disease phenotype, responses and toxicity to ruxolitinib; 2) performance of the MYSEC-PM in post-PV/post-ET MF. While the IPSS failed to correctly stratify post-PV/post-ET MF patients at diagnosis, the MYSEC-PM identified four risk categories projected ...
Source: Seminars in Hematology - June 4, 2018 Category: Hematology Authors: Francesca Palandri, Giuseppe Alberto Palumbo, Alessandra Iurlo, Nicola Polverelli, Giulia Benevolo, Massimo Breccia, Elisabetta Abruzzese, Mario Tiribelli, Massimiliano Bonifacio, Alessia Tieghi, Alessandro Isidori, Bruno Martino, Nicola Sgherza, Mariella Source Type: research
Philadelphia chromosome-like acute lymphoblastic leukemia: A review of the genetic basis, clinical features, and therapeutic options
Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL) is a recently identified high-risk subtype of B-lineage ALL (B-ALL), characterized by a gene expression profile similar to that of Philadelphia-positive (Ph+) ALL, but without the hallmark BCR-ABL1 oncoprotein. Ph-like ALL represents approximately 15% of childhood ALL and its frequency rises with age, peaking among adolescents, and young adults with B-ALL. This subtype is associated with adverse clinical features, persistence of minimal residual disease, and a poor prognosis despite modern chemotherapy regimens. (Source: Seminars in Hematology)
Source: Seminars in Hematology - May 29, 2018 Category: Hematology Authors: Maliha Khan, Rabbia Siddiqi, Thai Hoa Tran Tags: Review Source Type: research
Philadelphia-like acute lymphoblastic leukemia: a review of the genetic basis, clinical features and therapeutic options
Philadelphia-like acute lymphoblastic leukemia (Ph-like ALL) is a recently identified high-risk subtype of B-lineage ALL (B-ALL), characterized by a gene expression profile similar to that of Philadelphia-positive (Ph+) ALL, but without the hallmark BCR-ABL1 oncoprotein. Ph-like ALL represents approximately 15% of childhood ALL and its frequency rises with age, peaking among adolescents and young adults with B-ALL. This subtype is associated with adverse clinical features, persistence of minimal residual disease (MRD) and a poor prognosis despite modern chemotherapy regimens. (Source: Seminars in Hematology)
Source: Seminars in Hematology - May 29, 2018 Category: Hematology Authors: Maliha Khan, Rabbia Siddiqi, Thai Hoa Tran Tags: Review Source Type: research
Therapeutic complement modulation for hematological diseases: where we stand and where we are going
Complement-mediated hemolytic anemias are a heterogeneous group of diseases which are characterized by the pathogenic involvement of the complement cascade, irrespective of different etiologies and clinical presentations. Complement-mediated hemolytic diseases can be categorized according to different criteria; we have recently proposed a tentative classification based on the type of pathogenic involvement of the complement cascade (Table 1). [1] Accordingly, complement-mediated hemolytic anemias may be grouped into: i. (Source: Seminars in Hematology)
Source: Seminars in Hematology - May 29, 2018 Category: Hematology Authors: Antonio M Risitano Source Type: research
Advances in diagnosis of mastocytosis and hypereosinophilic syndrome ☆
Mastocytosis and hypereosinophilic syndrome is very rare neoplastic hematopoietic diseases. Mastocytosis is characterized by expansion and accumulation of clonal tissue mast cells in skin and/or various internal organs, while hypereosinophilic syndrome manifests with an increased number of eosinophils in the peripheral blood and tissue damage. These diseases represent a diagnostic challenge, since they can have overlapping clinical and pathologic features. Recently, great advances in the molecular and immunophenotypic diagnosis of these two entities were achieved, contributing to the new World Health Organization (WHO) cla...
Source: Seminars in Hematology - May 28, 2018 Category: Hematology Authors: Irina Maric, Xiaoping Sun Tags: Review Source Type: research
Profiling of lymphoma from formalin-fixed paraffin-embedded tissue
Molecular profiling of lymphoma samples has contributed enormously to our understanding of disease biology leading to detailed descriptions of diagnostic categories. These studies have also helped the field to recognize different subtypes of disease, different diseases that share similar cellular pathway perturbations, different immune responses, and different prognostic groups. While nearly all of these discoveries were made using unfixed, snap-frozen materials, with few exceptions, clinical biopsy materials are comprised of formalin-fixed and paraffin-embedded (FFPE) tissues. (Source: Seminars in Hematology)
Source: Seminars in Hematology - May 28, 2018 Category: Hematology Authors: Ryan S. Robetorye, Alanna Maguire, Alison C. Rosenthal, Lisa M. Rimsza Source Type: research
Increased Cardiovascular Comorbidities in Patients with Myelodysplastic Syndromes and Chronic Myelomonocytic Leukaemia Presenting with Systemic Inflammatory and Autoimmune Manifestations
In this study, we aimed to characterize patient- and disease-based differences in MDS/CMML patients with and without SIAMs and explore the impact of SIAMs on progression and survival. (Source: Seminars in Hematology)
Source: Seminars in Hematology - May 28, 2018 Category: Hematology Authors: B. Kipfer, T. Daikeler, S. Kuchen, M. Hallal, N. Andina, R. Allam, N. Bonadies Tags: Research Article Source Type: research
Profiling of Lymphoma from Formalin-Fixed Paraffin Embedded Tissue
Molecular profiling of lymphoma samples has contributed enormously to our understanding of disease biology leading to detailed descriptions of diagnostic categories. These studies have also helped the field to recognize different subtypes of disease, different diseases that share similar cellular pathway perturbations, different immune responses, and different prognostic groups. While nearly all of these discoveries were made using unfixed, snap-frozen materials, with few exceptions, clinical biopsy materials are comprised of formalin-fixed and paraffin-embedded (FFPE) tissues. (Source: Seminars in Hematology)
Source: Seminars in Hematology - May 28, 2018 Category: Hematology Authors: Ryan S. Robetorye, Alanna Maguire, Alison C. Rosenthal, Lisa M. Rimsza Source Type: research
Diagnosis and Classification of Lymphoma: Impact of Technical Advances
Our current understanding of the normal lymphoid system informs the modern classification of lymphomas. B-cell, T-cell and NK-cell neoplasms often recapitulate normal stages of lymphoid cell differentiation and function. Moreover, the clinical manifestations of lymphomas often reflect the normal function of lymphoid cells in vivo. The multiparameter approach to classification adopted by the REAL and subsequent WHO classifications facilitates the interpretation of clinical and translational studies, and provides a framework for the discovery of molecular alterations that drive these tumors. (Source: Seminars in Hematology)
Source: Seminars in Hematology - May 28, 2018 Category: Hematology Authors: Elaine S Jaffe Source Type: research
Genetic Studies in the Evaluation of Myeloproliferative Neoplasms
Myeloproliferative neoplasms that include the specific entities of chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are characterized by the clonal expansion of hematopoietic precursor cells and consequent neoplastic production of mature cells of myeloid, erythroid and/or megakaryocytic lineage. Genetic studies, encompassing both cytogenetic and molecular testing, play a central and ever-increasing role in the assessment of these neoplasms and are the focus of this review. (Source: Seminars in Hematology)
Source: Seminars in Hematology - May 28, 2018 Category: Hematology Authors: Gabriel C. Caponetti, Adam Bagg Source Type: research
