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Plasmablastic plasmacytoma of the rectum and anal canal in a patient with ulcerative colitis.

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… Sternal biopsy showed unspecific reactive bone marrow changes without elements of lymphoproliferative disorders. …

Guidance for Facing Dilemmas of Hematopoietic Stem Cell Transplant Clinicians in the COVID-19 Pandemic: An Iranian Consensus.

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Unraveling Obesity and Multiple Myeloma: Insights from Epidemiology and Molecular Mechanisms.

Purpose of reviewThe global incidence of obesity has risen dramatically in recent decades, with consequent detrimental health effects. Extensive studies have demonstrated that obesity significantly affects the risk, prognosis, and progression of various cancers, including multiple myeloma (MM). As an established modifiable risk factor for both MM and its precursor stages -monoclonal gammopathy of unknown significance (MGUS) and smoldering MM (SMM)- the association between obesity and disease onset has become a compelling area of research. This review presents a comprehensive overview of the cu

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… Similarly, findings from the Age, Gene/Environment Susceptibility-Reykjavik Study (AGES-RS) showed that increased BMI during midlife significantly correlated with a high risk of progression from MGUS to MM and further lymphoproliferative disorders. …

Sirolimus versus cyclosporine in haploidentical stem cell transplantation with posttransplant cyclophosphamide and mycophenolate mofetil as graft-versus-host disease prophylaxis.

Sirolimus has emerged as an alternative to calcineurin inhibitors-based (CNI) graft-versus-host disease (GVHD) prophylaxis. This retrospective study compares the outcome of 133 consecutive adult patients with haematological malignancies undergoing haploidentical stem cell transplantation with posttransplant cyclophosphamide (PTCy) and mycophenolate mofetil (MMF), combined with cyclosporine A (PTCy-CsA-MMF, n = 67) or sirolimus (PTCy-Sir-MMF, n = 66) as GVHD prophylaxis strategy. The median follow-up was 48 (range 22-83) and 13 (range 3-33) months, respectively. PTCy-CsA-MMF was associated in m

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… Management of Epstein–Barr virus infections and post‐transplant lymphoproliferative disorders in patients after allogeneic hematopoietic stem cell transplantation: Sixth European Conference on Infections in Leukemia (ECIL‐6) guidelines. Haematologica. 2016;101:803–11. 21. …

Pure red cell aplasia and minimal residual disease conversion associated with immune reconstitution in a patient with high-risk multiple myeloma.

A second bone marrow aspiration and biopsy showed pure red cell aplasia in this case.

Acute Kidney Injury in the Patient with Cancer.

Over the last three decades, advancements in the diagnosis, treatment, and supportive care of patients with cancer have significantly improved their overall survival. However, these advancements have also led to a higher rate of cancer-related complications. Acute kidney injury (AKI) and chronic kidney disease (CKD) are highly prevalent in patients with cancer, and they are associated with an increased risk of all-cause mortality. This bidirectional interplay between cancer and kidney, termed "the kidney-cancer connection" has become a very active area of research. This review aims to provide

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… Renal Involvement in Myeloproliferative and Lymphoproliferative Disorders. A Study of Autopsy Cases General Diagn. Pathol. 1997 142 147 153 9065578 59. Lommatzsch S.E. Bellizzi A.M. Cathro H.P. Rosner M.H. Acute Renal Failure Caused by Renal Infiltration by Hematolymphoid Malignancy Ann. Diagn. …

Hodgkin's Disease in Patients with HIV Infection.

Hodgkin lymphoma (HL) represents one of the most common non-AIDS-defining cancers with an increasing incidence overtime. Clinically, patients present advanced stages of disease with extranodal involvement in the majority of cases. In the last years, significant improvements in the treatment of patients with HL and HIV infection have been achieved. In the lack of randomized trials, several phase II studies have showed that in the era of highly active antiretroviral therapy (HAART) the same regimens employed in HIV-negative patients with HL can be used in HIV setting with similar results. Moreov

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… Epstein-Barr virus-associated lymphoproliferative disorders. Human Pathology. 2007;38(9):1293–1304. doi: 10.1016/j.humpath.2007.05.020. 31. Carbone A, Gloghini A, Dotti G. EBV-associated lymphoproliferative disorders: classification and treatment. …

Orbital Plasmacytoma as Initial Presentation of Multiple Myeloma.

Multiple myeloma (MM) is a hematologic malignancy characterized by clonal proliferation of plasma cells in the bone marrow. It represents approximately 10% of hematologic malignancies and is the most common primary bone malignancy. This disease usually occurs between the ages of 40 and 70, although it is more frequently seen in patients over 65 years of age. On the other hand, extramedullary plasmacytoma-related diseases have a global annual incidence of three per 100,000 people. Orbital involvement in MM is considered rare. Orbital plasmacytomas represent less than 1% of all orbital lesions,

Secondary Neutropenias.

Neutrophils are a critical component of immunity, particularly against bacteria and other pathogens, but also in inflammation and tissue repair. As a consequence, individuals with neutropenia, defined by a reduction in absolute neutrophil counts, exhibit a strong propensity to severe infections that typically present with muted symptoms. Neutropenias encompass a heterogeneous set of disorders, comprising primary neutropenias, in which specific genes are mutated, and the more common secondary neutropenias, which have diverse non-genetic causes. These include hematological and other cancers, inv

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… disorders | LGL immunodeficiencies | CVID incompatibility | maternal/fetal, HSCT Infection | viral | chickenpox, CMV, COVID-19, EBV, HIV, influenza, measles, viral hepatitis bacterial | brucellosis, rickettsia, sepsis, shigellosis, typhoid fever, tuberculosis other | malaria Nutrient deficiencies | … Other immune-related causes include lymphoproliferative disorders, especially large granular lymphocyte (LGL) syndrome that impacts peripheral neutrophil numbers by multiple mechanisms: auto-antibody mediated apoptosis in the periphery, suppression of neutrophil production in the bone marrow, and inhibition …

COVID-19 in Adult Patients with Hematological Malignancies-Lessons Learned after Three Years of Pandemic.

The COVID-19 pandemic is undoubtedly the most difficult health challenge of the 21st century with more than 600 million laboratory-confirmed SARS-CoV-2 infections and over 6.5 million deaths worldwide. The coronavirus pandemic contributed to rapid development of mRNA vaccines, which, along with new antiviral drugs, have been the subject of extensive research for many decades. Nevertheless, elderly, multi-morbid and immunocompromised patients continue to face a more severe clinical course and a higher risk of death from COVID-19, even now that the risk of COVID-19 in the general population is s

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… Most deaths were reported among patients with lymphoproliferative disorders (71.4%), and none were reported among patients with acute myeloid leukemia, which is a significant difference compared to the pre-vaccination period [ 129 ]. …

Post-transplant cyclophosphamide versus anti-thymocyte globulin after reduced intensity peripheral blood allogeneic cell transplantation in recipients of matched sibling or 10/10 HLA matched unrelated donors: final analysis of a randomized, open-label, multicenter, phase 2 trial.

The use of post-transplantation cyclophosphamide (PTCy) for graft-versus-host disease (GVHD) prophylaxis is not established after reduced intensity conditioning (RIC) hematopoietic stem cell transplantation (HSCT) from fully matched donors. This was a randomized, open-label, multicenter, phase 2 trial. All patients received a RIC regimen with fludarabine, intravenous busulfan for 2 days (Flu-Bu2), and a peripheral blood stem cell (PBSC) graft from a matched related or 10/10 HLA-matched unrelated donor. Patients were randomly assigned to receive anti-thymocyte globulin (ATG) 5 mg/kg plus standa

Secondary Hemophagocytic Syndrome in a Patient with Plasma Cell Myeloma and CNS Involvement Treated with Lenalidomide.

We present an extremely rare case report of a 29-year-old multiple myeloma patient with central nervous system involvement and secondary hemophagocytic lymphohistiocytosis (HLH). We observed that HLH was presumably triggered by the immunomodulatory drug-lenalidomide. HLH is frequently misdiagnosed or underdiagnosed. As HLH requires immediate treatment, our report emphasizes the need to consider HLH in the differential diagnosis when the condition of a patient receiving chemotherapy rapidly deteriorates and an infectious etiology is excluded. We furthermore discuss the pathogenesis of HLH, with

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… Interestingly, PCM contributes to <1% of the causes of HLH in patients with lymphoproliferative disorders [ 7 ] Malignancy-associated HLH (Mal-HLH) remains the most common form of secondary HLH and has the worst prognosis of all HLH subgroups [ 7 ]. …

Immune defects in the risk of infection and response to vaccination in monoclonal gammopathy of undetermined significance and multiple myeloma.

The plasma cell proliferative disorders monoclonal gammopathy of undetermined significance (MGUS) and malignant multiple myeloma (MM) are characterized by an accumulation of transformed clonal plasma cells in the bone marrow and production of monoclonal immunoglobulin. They typically affect an older population, with median age of diagnosis of approximately 70 years. In both disorders, there is an increased risk of infection due to the immunosuppressive effects of disease and conjointly of therapy in MM, and response to vaccination to counter infection is compromised. The underlying factors in

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… Indeed, in a study of 34 patients with chronic lymphoproliferative disorders and MM, some receiving therapy, response rates were comparable to healthy controls ( 160 ). … CLPD, chronic lymphoproliferative disorders; HC, healthy controls; HD, Hodgkin disease; MP, mephalan; NHL, non-Hodgkin lymphoma; VAD, vincristine–adriamycin–dexamethasone . …

Novel Molecular Therapies and Genetic Landscape in Selected Rare Diseases with Hematologic Manifestations: A Review of the Literature.

Rare diseases affect less than 1 in 2000 people and are characterized by a serious, chronic, and progressive course. Among the described diseases, a mutation in a single gene caused mastocytosis, thrombotic thrombocytopenic purpura, Gaucher disease, and paroxysmal nocturnal hemoglobinuria (KIT, ADAMTS13, GBA1, and PIG-A genes, respectively). In Castleman disease, improper ETS1, PTPN6, TGFBR2, DNMT3A, and PDGFRB genes cause the appearance of symptoms. In histiocytosis, several mutation variants are described: BRAF, MAP2K1, MAP3K1, ARAF, ERBB3, NRAS, KRAS, PICK1, PIK3R2, and PIK3CA. Genes like H

References

Аннотация не предоставлена источником. Откройте карточку для перехода к публикации.

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… Risk factors for developing EBV-related B cell lymphoproliferative disorders (BLPD) after non-HLA-identical BMT in children. Bone Marrow Transplant. 1996;18:377–382. 260. Lucas KG, Burton RL, Zimmerman SE, Wang J, Cornetta KG, Robertson KA. … Prevention of EBV-induced B-lymphoproliferative disorder by ex vivo marrow B-cell depletion in HLA-phenoidentical or non-identical T-depleted bone marrow transplantation. Br J Haematol. 1998;103:543–551. doi: 10.1046/j.1365-2141.1998.00972.x. 269. …

Leukemias in the Context of Rheumatoid Arthritis: Shared Pathways and Clinical Perspectives.

Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterized by systemic inflammation, progressive joint destruction, and increased risk of malignancies, particularly hematological cancers such as leukemias. RA patients appear to have a higher incidence of leukemias, suggesting a possible association between the two conditions. This association is driven by shared pathogenic mechanisms, including chronic inflammation, immune dysregulation, and genetic predispositions. Pro-inflammatory cytokines, notably tumor necrosis factor-alpha (TNF-α) and interleukin 6 (IL-6), play a crucial ro

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… The primary MeSH terms included “Rheumatoid Arthritis,” “Leukemia,” “Lymphoproliferative Disorders,” “Hematologic Neoplasms,” “Cytokines,” and “Immune Dysregulation.” …

Daratumumab monotherapy in refractory warm autoimmune hemolytic anemia and cold agglutinin disease.

AbstractAutoimmune hemolytic anemia (AIHA) is a rare autoantibody-mediated disease. For steroid and/or rituximab-refractory AIHA, there is no consensus on optimal treatment. Daratumumab, a monoclonal antibody targeting CD38, could be beneficial by suppression of CD38+ plasma cells and thus autoantibody secretion. In addition, because CD38 is also expressed by activated T cells, daratumumab may also act via immunomodulatory effects. We evaluated the efficacy and safety of daratumumab monotherapy in an international retrospective study including 19 adult patients with heavily pretreated refracto

Real-World Effectiveness, Safety, and Tolerability of Facilitated Subcutaneous Immunoglobulin 10% in Secondary Immunodeficiency Disease: A Systematic Literature Review.

Background: Secondary immunodeficiency disease (SID) is a complex, heterogeneous condition that occurs when extrinsic factors weaken the immune system. Expert consensus guidelines recommend immunoglobulin replacement therapy to manage immunoglobulin G (IgG) levels and mitigate severe, recurrent, and persistent infections. Hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIG) 10% is a dual-vial unit of IgG and recombinant human hyaluronidase; the latter enables absorption of higher volumes of IgG than conventional subcutaneous therapies. Methods: For this systematic literature review, E

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… However, there is consensus on IgRT as primary prophylaxis in patients assessed for hypogammaglobulinemia, namely those receiving chimeric antigen receptor T (CAR T)-cell therapy and bispecific antibody therapies, those with lymphoproliferative disorders or severe comorbidities, and those with IgG levels … Combined immune defect in B-cell lymphoproliferative disorders is associated with severe infection and cancer progression. Biomedicines. 2022;10:2020. doi: 10.3390/biomedicines10082020. 6. Jolles S., Michallet M., Agostini C., Albert M.H., Edgar D., Ria R., Trentin L., Levy V. …

Ларингейл амилоидоз: один центр four- Серия пациентов, освещающих диагностические проблемы и клиническую неоднородность.

Ларингейл амилоидоз является редким заболеванием, которое может имитировать доброкачественные, злокачественные или функциональные расстройства гортани. Мы представляем единый центр four- Серия пациентов, демонстрирующих клиническую неоднородность при анатомическом распределении, амилоидном печатании, системном взаимодействии и управлении. Все four Эти случаи были подтверждены гистологически. Two Пациенты, по результатам специализированной оценки, имели локальный ларингеальный амилоидоз, one У пациента было ларинготражающее амилоидное осаждение в контексте известной множественной миэломы, и one

Cutaneous paraneoplastic syndromes in cats: a comparative clinical review.

PRACTICAL RELEVANCE: Cutaneous paraneoplastic syndromes (CPSs) in cats represent a diverse group of rare dermatological manifestations that occur as indirect consequences of underlying neoplasia. These syndromes are thought to arise due to tumour-associated systemic effects, including dysregulation of immune responses, metabolic disturbances and aberrant production of cytokines or growth factors. Recognising CPSs is clinically relevant, as they may serve as early indicators of occult neoplasia, guiding timely diagnosis and intervention. CLINICAL CHALLENGES: Diagnosing CPSs requires a high ind