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Topic Review
Diagnosis of Primary Vitreoretinal Lymphoma
Intraocular lymphomas (IOLs) include vitreoretinal lymphomas (VRLs) and primary uveal or choroidal lymphomas. VRLs are further subdivided into primary VRLs and secondary VRLs, the latter deriving from systemic lymphomas. Primary uveal or choroidal lymphomas are usually low-grade neoplasms and are frequently extranodal marginal zone lymphomas with very good outcomes, unlike primary vitreoretinal lymphomas (PVRLs) which are high-grade diseases with poor outcomes. Secondary IOLs derive from ocular involvement by systemic lymphomas through haematogenous spread. Systemic lymphomas mainly disseminate to the uvea, due to its rich blood flow. PVRL represents a diagnostic challenge for both clinicians and pathologists, and it is critical, for the patient’s life, to shorten the time between the onset of symptoms often mistaken for chronic uveitis and correct diagnosis. Different laboratory methods are in use to diagnose PVRL. The main employed techniques are described, highlighting the principal diagnostic issues with the different laboratory methods.
  • 736
  • 18 Oct 2022
Topic Review
Primary Bone Lymphoma
Primary bone lymphoma is a rare neoplasm of malignant lymphoid cells presenting with one or more bone lesions without nodal or other extranodal involvement. It accounts for approximately 1% of all lymphomas and 7% of malignant primary bone tumors.
  • 732
  • 20 Mar 2023
Topic Review
Factors Associated with Post-Chimeric Antigen Receptor T Cytopenias
Chimeric Antigen Receptor (CAR) T-cell therapy is a promising treatment option for patients suffering from B-cell- and plasma cell-derived hematologic malignancies and is being adapted for the treatment of solid cancers. CAR T is usually associated with cytopenias. These are often biphasic and sometimes prolonged over several months. Cytopenias often lead to infections, need for transfusions of blood products, and increased CAR T morbidity.
  • 723
  • 24 Feb 2023
Topic Review
Therapy of Relapsed/Refractory Mantle Cell Lymphoma after BTKis
Mantle cell lymphoma (MCL) is a rare mature B-cell non-Hodgkin lymphoma (B-NHL) with historically poor outcomes. Virtually all patients will eventually experience refractory or relapsed (R/R) disease, with a virulent course of resistance and serial relapses, making treatment challenging. The available therapies for R/R MCL are not curative with conventional therapy, their goal being to palliate and prolong survival. A variety of agents approved for R/R MCL, including Bruton’s tyrosine kinase inhibitors (BTKi), changed the treatment landscape of R/R MCL. In the pre-BTKi era, the median progression-free survival (PFS) in R/R disease was 4–9 months. With the introduction of ibrutinib, the median PFS improved to 13–14.6 months. Despite these impressive results, the duration of response is limited, and resistance to BTKi inevitably develops in a subset of patients. Outcomes after progression on BTKi are extremely poor, with a median overall survival (OS) of 6 to 10 months. Certain therapies, such as chimeric antigen receptor (CAR) T cells, have shown promising results after BTKi failure. The preferred combination and sequencing of therapies beyond BTKi remain unestablished.
  • 720
  • 23 Mar 2022
Topic Review
Pediatric Acute Myeloid Leukemia
Pediatric acute myeloid leukemia is a clonal disorder characterized by malignant transformation of the hematopoietic stem cell. The incidence and the outcome remain inferior when compared to pediatric ALL, although prognosis has improved in the last decades, with 80% overall survival rate reported in some studies. The standard therapeutic approach is a combined cytarabine and anthracycline-based regimen followed by consolidation with allogeneic stem cell transplantation (allo-SCT) for high-risk AML and allo-SCT for non-high-risk patients only in second complete remission after relapse.
  • 720
  • 29 Jun 2022
Topic Review
Priapism at Diagnosis of Pediatric Chronic Myeloid Leukemia
Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1/100,000) typically presenting with leucocyte counts >100,000/µL. However, clinical signs of leukostasis are observed at diagnosis in only approximately 10% of all cases and among these, priapism is infrequent.
  • 718
  • 08 Aug 2023
Topic Review
Extramedullary Relapse of FLT3-ITD Acute Myeloid Leukemia
FMS-like tyrosine kinase 3 (FLT3) is a receptor tyrosine kinase family member. Mutations in FLT3, as well known, represent the most common genomic alteration in acute myeloid leukemia (AML), identified in approximately one-third of newly diagnosed adult patients. In recent years, this has represented an important therapeutic target. Drugs such as midostaurin, gilteritinib, and sorafenib, either alone in association with conventional chemotherapy, play a pivotal role in AML therapy with the mutated FLT3 gene. A current challenge lies in treating forms of AML with extramedullary localization. 
  • 715
  • 06 Jun 2022
Topic Review
MiRNA-Based Therapies in NHL
Non-Hodgkin’s lymphoma (NHL) is a very heterogenous group of lymphoid malignancies originating from different stages of B-cell (~90% of the cases) and T-cell or NK-cell differentiation. Increasing evidence has demonstrated the functional roles of miRNAs and lncRNAs in lymphoma onset and progression, either by acting as tumor-promoting ncRNAs or as tumor suppressors, emphasizing their appeal as lymphoma therapeutics. In fact, their intrinsic ability to modulate multiple dysregulated genes and/or signaling pathways makes them an attractive therapeutic approach for a multifactorial pathology like lymphoma.
  • 709
  • 24 Dec 2021
Topic Review
Non-Coding RNAs for Leukemia
Early-stage leukemia identification is crucial for effective disease management and leads to an improvement in the survival of leukemia patients. Approaches based on cutting-edge biomarkers with excellent accuracy in body liquids provide patients with the possibility of early diagnosis with high sensitivity and specificity. Non-coding RNAs have received a great deal of interest as possible biomarkers in leukemia due to their participation in crucial oncogenic processes such as proliferation, differentiation, invasion, apoptosis, and their availability in body fluids. Studies have revealed a strong correlation between leukemia and the deregulated non-coding RNAs. On this basis, these RNAs are also great therapeutic targets. 
  • 703
  • 31 Jan 2023
Topic Review
Mutations and Biomarkers of DNA Damage in Myeloma
Multiple myeloma (MM) is a plasma cell malignancy characterized by several genetic abnormalities, including chromosomal translocations, genomic deletions and gains, and point mutations. DNA damage response (DDR) and DNA repair mechanisms are altered in MM to allow for tumor development, progression, and resistance to therapies.
  • 703
  • 28 Aug 2023
Topic Review
Hypomethylating Agents Failure
Hypomethylating agents (HMA) such as azacitidine and decitabine are a mainstay in the current management of patients with myelodysplastic syndromes/neoplasms (MDS) and acute myeloid leukemia (AML) as either single agents or in multidrug combinations. Resistance to HMA is not uncommon, and it can result due to several tumor cellular adaptations. Several clinical and genomic factors have been identified as predictors of HMA resistance. However, the management of MDS/AML patients after the failure of HMA remains challenging in the absence of standardized guidelines.
  • 701
  • 05 May 2023
Topic Review
Immunosuppressive Microenvironment in the Progression of Multiple Myeloma
The immunosuppressive multiple myeloma bone marrow microenvironment consists of myeloid-derived suppressor cells (MDSCs), tumor-associated M2-like macrophages (M2 TAMs), N2 neutrophils, regulatory T cells (Tregs), regulatory B cells (Bregs), and plasmacytoid dendritic cells (pDCs). 
  • 699
  • 21 Dec 2022
Topic Review
Clinical Approach to Immune Thrombocytopenia
Primary immune thrombocytopenia (ITP) is a complex autoimmune disease whose hallmark is a deregulation of cellular and humoral immunity leading to increased destruction and reduced production of platelets. The heterogeneity of presentation and clinical course hampers personalized approaches for diagnosis and management.
  • 698
  • 30 Oct 2023
Topic Review
Toll-like Receptors in Hematology
Toll-like receptors (TLR) belong to the pattern recognition receptors (PRR). TLRs are primarily expressed in hematopoietic cells. TLRs also direct the formation of blood cellular components and make a contribution to the pathogenesis of certain hematopoietic malignancies. 
  • 696
  • 22 May 2023
Topic Review
Therapeutic Landscape of Peripheral T-Cell Lymphomas
T-cell lymphomas are a relatively rare group of malignancies with a diverse range of pathologic features and clinical behaviors. Recent molecular studies have revealed a wide array of different mechanisms that drive the development of these malignancies and may be associated with resistance to therapies. 
  • 693
  • 29 Mar 2022
Topic Review
Gilteritinib
Gilteritinib is a next-generation tyrosine kinase inhibitor (TKI) primarily targeting FLT3FLT3 and AXL (an onco-genic tyrosine kinase) receptors. 
  • 693
  • 06 Jun 2023
Topic Review
Blood Transfusion Donor Sex Influences Newborn Outcomes
The risk of both short- and long-term mortality increases with each red blood cell (RBC) transfusion exposure in critically ill patients. In the extremely preterm newborn, one of the most heavily transfused patient groups, observational data has proposed that the association between RBC transfusion and adverse neonatal outcomes may be influenced by donor sex, although the data remains limited.
  • 690
  • 27 Dec 2022
Topic Review
Therapeutic Anticoagulation in COVID-19
Thrombotic complications from COVID-19 are now well known and contribute to significant morbidity and mortality. Different variants confer varying risks of thrombotic complications. Heparin has anti-inflammatory and antiviral effects. Due to its non-anticoagulant effects, escalated-dose anticoagulation, especially therapeutic-dose heparin, has been studied for thromboprophylaxis in hospitalized patients with COVID-19.
  • 690
  • 24 Nov 2023
Topic Review
Advancements and Considerations in HSCT for CML
Hematopoietic stem cell transplantation (HSCT) for chronic myeloid leukemia (CML) patients has transitioned from the standard of care to a treatment option limited to those with unsatisfactory tyrosine kinase inhibitor (TKI) responses and advanced disease stages. In recent years, the threshold for undergoing HSCT has increased. Most CML patients now have life expectancies comparable to the general population, and therefore, the goal of therapy is shifting toward achieving treatment-free remission (TFR). While TKI discontinuation trials in CML show potential for achieving TFR, relapse risk is high, affirming allogeneic HSCT as the sole curative treatment. HSCT should be incorporated into treatment algorithms from the time of diagnosis and, in some patients, evaluated as soon as possible.
  • 688
  • 21 Feb 2024
Topic Review
Diagnostics and Therapeutics in Sepsis
Sepsis is a clinical syndrome resulting from a dysregulated inflammatory response to infection.  Sepsis management demands early diagnosis and timely treatment that includes source control, antimicrobial therapy, and resuscitation. 
  • 684
  • 12 May 2021
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