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Successful treatment of hemophagocytic syndrome in a patient with T cell lymphoma, EBV infection, and bone marrow necrosis: A case report.

RationaleHemophagocytic syndrome (HPS) is associated with a high mortality rate, and Epstein-Barr virus infection and hematological malignancies, especially T/natural killer cell lymphomas, are the most common causes; however, due to the complexity of clinical manifestations, the diagnosis is usually delayed. There are few reports of lymphoma-associated HPS (LAPS) in combination with bone marrow necrosis, and there is still no standard treatment for LAPS.Patient concernsA 64-year-old man developed a fever, mild jaundice, fatigue, and bone pain. Positron emission tomography and bone marrow biop

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… Lim et al [ 12 ] found that rituximab is an effective treatment for a number of EBV-mediated conditions, including EBV-induced lymphoproliferative disorders. …

Systemic Mastocytosis with Smoldering Multiple Myeloma: Report of a Case.

Systemic mastocytosis (SM) is a disease characterized by a clonal infiltration of mast cells affecting various tissues of the body. It is grouped into six different subtypes according to the World Health Organization classification. It is called indolent systemic mastocytosis (ISM) when there is no evidence of end organ dysfunction, while the presence of end organ dysfunction defines aggressive systemic mastocytosis (ASM). When SM coexists with a clonal hematological disorder, it is classified as systemic mastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD)

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… While associated myeloid/myelomonocytic neoplasia in SM-AHNMD accounts for 82%–89% of SM-AHNMD, associated lymphoproliferative disorders are rare [ 3 , 4 ]. … In 2 cases of SM associated with lymphoproliferative disorders, the D816V mutation was detected in the neoplastic mast cells but not in the malignant lymphocytes, suggesting that the SM and the coexisting lymphoid malignancy are clonally distinct [ 18 , 19 ]. …

Primary cutaneous lymphomas: diagnosis and treatment.

Primary cutaneous lymphomas (CLs) are a heterogeneous group of lymphoproliferative neoplasms, with lymphatic proliferation limited to the skin with no involvement of lymph nodes, bone marrow or viscera at the diagnosis. Cutaneous lymphomas originate from mature T-lymphocytes (65% of all cases), mature B-lymphocytes (25%) or NK cells. Histopathological evaluation including immunophenotyping of the skin biopsy specimen is the basis of the diagnosis, which must be complemented with a precise staging of the disease and identification of prognostic factors, to allow for the choice of the best treat

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… Lymphoproliferative disorders, most often ALF, MF, HL or non-Hodgkin lymphoma (nHL) may coincide with LyP in 30–50% of the patients. … Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment Blood 2000 95 3653 61 10845893 52 Vonderheid EC Sajjadian A Kadin ME Methotrexate is effective therapy for lymphomatoid papulosis and other primary cutaneous CD30-positive lymphoproliferative disorders …

Waldenström macroglobulinemia: clinical and immunological aspects, natural history, cell of origin, and emerging mouse models.

Waldenström macroglobulinemia (WM) is a rare and currently incurable neoplasm of IgM-expressing B-lymphocytes that is characterized by the occurrence of a monoclonal IgM (mIgM) paraprotein in blood serum and the infiltration of the hematopoietic bone marrow with malignant lymphoplasmacytic cells. The symptoms of patients with WM can be attributed to the extent and tissue sites of tumor cell infiltration and the magnitude and immunological specificity of the paraprotein. WM presents fascinating clues on neoplastic B-cell development, including the recent discovery of a specific gain-of-function

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… The result supports the existence of common B-lineage cancer susceptibility alleles that render carriers susceptible to both LPL/WM and a number of “kindred” lymphoproliferative disorders [ 85 , 86 ]. … disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenström macroglobulinemia patients: a population-based study in Sweden Blood 2008 112 8 3052 3056 2-s2.0-54049129700 18703425 10.1182/blood-2008-06-162768 PMC2569164 85 Goldin LR Pfeiffer RM Gridley G Familial aggregation of Hodgkin …

T-Cell Lymphoma With Sarcoid-Like Reaction and Secondary Myelofibrosis: A Unique Case Report and Literature Review.

INTRODUCTION: T-cell lymphomas are rare, comprising 10%-15% of non-Hodgkin lymphomas (NHLs). Their etiology remains unclear, and pathophysiology varies widely among subtypes. Sarcoid-like reactions (SLRs) have been reported in approximately 7.3% of NHLs, including T-cell lymphomas, and can closely resemble sarcoidosis clinically/histologically. This overlap complicates diagnosis, particularly when secondary myelofibrosis-a rare and poorly understood complication-arises. Myelofibrosis has also been observed in sarcoidosis, further blurring the distinction between these entities. Treatment for T

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… Introduction T‐cell lymphomas, a type of non‐Hodgkin lymphoma (NHL), are a rare and heterogeneous group of lymphoproliferative disorders arising from mature T‐cells. … disorders [ 7 ]. …

Cytokine-induced killer cells: new insights for therapy of hematologic malignancies.

BackgroundCytokine-induced killer (CIK) cells are a novel subgroup of immune effectors, classified as one of the modified T cell-mediated arms for immunotherapy. These cells exert MHC-unrestricted cytotoxicity against both hematological and solid malignancies with low incidence of treatment-related severe complications. This study reviews the application of CIK cells in treating cases with hematologic malignancies.Main bodyCIK cells consist of CD3+/CD56+ natural killer (NK) T cells, CD3-/CD56+ NK cells, and CD3+/CD56- cytotoxic T cells. In this regard, the CD3+/CD56+ NK T cells are the primary

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… Post-transplant lymphoproliferative disorders (PTLD) can be a fatal complication following allogeneic HSCT [ 117 ]. … EBV-induced post transplant lymphoproliferative disorders: a persisting challenge in allogeneic hematopoetic SCT. Bone Marrow Transplant. 2014;49(2):163–7. 118. van Esser JWJ, van der Holt B, Meijer E, Niesters HGM, Trenschel R, Thijsen SFT, et al. …

Nephrology's Next Frontier: Expanding the Reach of CAR T-Cell Therapy for Refractory Lupus Nephritis and Beyond.

Chimeric antigen receptor (CAR) T-cell therapy has recently evolved beyond cancer therapy's boundary to treating autoimmune diseases such as lupus nephritis. In CAR T-cell therapy, the genetically engineered patient's T cells express a receptor specifically targeting antigens such as CD19, a protein found on the surface of B cells. By directing the immune system to eliminate B cells, which play a central role in the pathogenesis of systemic lupus erythematosus, CAR T-cell therapy offers a novel and potent approach to resetting the immune system and achieving remission in difficult-to-treat lup

A rare case of acute liver failure due to disseminated Varicella-Zoster Virus (VZV) infection.

Varicella zoster virus (VZV) is a human alpha herpes virus that typically manifests in humans with two distinct cutaneous phenotypes: primary varicella, or chickenpox, and secondary reactivation, or Shingles, after establishing latency in the cranial and dorsal root ganglia. VZV infection can present with rarer manifestations including encephalitis, pneumonia, hepatitis, myocarditis, and nephritis. Immunocompromised populations are at the greatest risk for cutaneous and visceral dissemination. The progression of VZV hepatitis to acute liver failure is exceedingly rare, with very few published

Hematology

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… Patients should then seek medical advice urgently medical alert bracelet Neutropenia Differential Diagnosis ★PANIC★ P ost-infectious —sepsis A utoimmune —drug induced, SLE N eoplastic —lymphoproliferative disorders, myelodysplasia, leukemias, myelophthisis I nfections —sepsis, HIV I nsufficiency …

Immunotherapeutic approaches in EBV-associated nasopharyngeal carcinoma.

Epstein-Barr virus (EBV) was the first tumor virus in humans. Nasopharyngeal carcinoma (NPC) accounts for approximately 60% of the 200,000 new tumor cases caused by EBV infection worldwide each year. NPC has an insidious onset and is highly malignant, with more than 70% of patients having intermediate to advanced disease at the time of initial diagnosis, and is strongly implicated in epithelial cancers as well as malignant lymphoid and natural killer/T cell lymphomas. Over 90% of patients with confirmed undifferentiated NPC are infected with EBV. In recent decades, much progress has been made

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… EBV-specific cytotoxic T lymphocyte therapy EBV-associated posttransplant lymphoproliferative disorders (PTLDs) are common complications in solid organ and bone marrow transplant recipients ( 93 – 96 ). … Infusions of donor leukocytes to treat Epstein-Barr virus-associated lymphoproliferative disorders after allogeneic bone marrow transplantation. N Engl J Med (1994) 330(17):1185–91. doi: 10.1056/NEJM199404283301703 97. Rickinson AB, Moss DJ. …

The first description of a singular case of synchronous chronic myelomonocytic leukemia and diffuse large b-cell lymphoma.

In CMML, neoplastic monocytes can be distinguished based on their immunophenotype. Supportive care myeloid growth factors in concomitant extranodal non-Hodgkin Lymphoma are safe.

Progressive Multifocal Leukoencephalopathy after Administration of Front-Line Obinutuzumab and Venetoclax in a Patient with Chronic Lymphocytic Leukemia: Case Report.

INTRODUCTION: Progressive multifocal leukoencephalopathy (PML) is a rare and often fatal demyelinating disease of the central nervous system caused by the reactivation of the John Cunningham virus (JCV) in immunocompromised patients. Obinutuzumab is type II anti-CD 20 antibody, used in combination with venetoclax for chronic lymphocytic leukemia (CLL). CASE PRESENTATION: A 75-year-old female patient was diagnosed with PML after a short course of obinutuzumab and venetoclax started for a newly diagnosed CLL. There was no history of hepatitis B, C, or human-immunodeficiency virus. Quantitative

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… However, with the increased use of monoclonal antibodies for various lymphoproliferative disorders, more cases have been reported in non-human-immunodeficiency virus (HIV) patients on drugs such as natalizumab, and in particular rituximab. … Obinutuzumab is a humanized type II anti-CD 20 antibody, widely used in combination with chemotherapy as the therapeutic backbone for a variety of lymphoproliferative disorders, including chronic lymphocytic leukemia (CLL). It induces antibody-depended cytotoxicity, phagocytosis, and cell death. …

Role of Bioimpedance Phase Angle and Hand Grip Strength in Predicting 12-Month Mortality in Patients Admitted with Haematologic Cancer.

Background/Objectives: Haematologic cancers, such acute leukaemia, lymphoma, and multiple myeloma, are associated with high morbidity and mortality rates, often exacerbated by malnutrition and functional decline. This study aims to evaluate the prognostic value of bioimpedance phase angle (PhA) and hand grip strength (HGS) as nutritional and clinical markers for predicting 12-month mortality in hospitalized patients with haematologic cancers. Methods: A retrospective observational study was conducted on 121 patients admitted to Hospital Quironsalud Málaga between January 2019 and June 2021. Ph

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… The data reveal less common types of haematological malignancies such as T/NK-cell T/NK Non-Hodgkin Lymphoma and Mantle Cell Lymphoma, which are also present, though in smaller numbers, suggesting a range of lymphoproliferative disorders that require specialized attention. …

Changing trends in lymphoid neoplasm distribution in South Korea: analysis of 8615 cases from a single institute, 1997-2016: An observational study.

The purpose of this study was to evaluate changes in the proportion of lymphoid neoplasm subtypes in South Korea. A total of 8615 cases of lymphoid neoplasms diagnosed in 1997-2016 at Samsung Medical Center in South Korea were classified according to the 2008 World Health Organization system. The total number and proportion of lymphoid neoplasms were compared between these two decades, with data from nationwide studies, and with other countries. To evaluate changes in the proportion of subtypes, crude rate of each subtype per 100 lymphoma patients during each decade and age adjusted rate were

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… disorders,” “plasmacytoma,” and “myeloma.” … Posttransplantation lymphoproliferative disorders and cases with insufficient immunohistochemical evidence to allow 2008 WHO classification were excluded. Only the initial diagnosis for each patient was included. A total of 8615 patients diagnosed with lymphoid neoplasms in 1997–2016 were enrolled. …

Cryoglobulinemia Type II: Sustained Remission After B-Cell-Directed Therapy.

BACKGROUND Cryoglobulinemia is a rare disease with a prevalence of <5 cases per 10 000. In many cases there is significant diagnostic delay, leading to years of morbidity. There are no randomized trials on non-infectious type II cryoglobulinemia, and choice of therapy is based on clinical expertise and observational data. Therapy based on the monoclonal anti-CD20 antibody rituximab in combination with glucocorticosteroids is the preferred choice in many centers. This strategy induces clinical remission in two-thirds of patients. However, one-third of the initially-responding patients experienc

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… Type II cryoglobulins are a mixture of monoclonal IgM with rheumatoid factor activity and polyclonal IgG, and are associated with autoimmune diseases (systemic lupus erythematosus [SLE] and Sjögren syndrome), lymphoproliferative disorders or infection (particularly hepatitis C virus infection). …

Technology forecast: advanced therapies in late clinical research, EMA approval or clinical application via hospital exemption.

Background: The umbrella term ATMPs (Advanced Therapy Medicinal Products) comprises cell therapies, gene therapeutics and tissue engineered products. After implementation of the Regulation 1394/2007, only a couple of products have obtained a centralized European marketing authorisation. Objectives: The aim of the presented study is to give an overview on ATMPs available within the European Union either via centralized marketing authorisation or via national Hospital exemption. Additionally, a forecast on innovative ATMPs in the process of EMA approval as well as in phase III and IV clinical tr

Results of a multicenter phase I/II trial of TCRαβ and CD19-depleted haploidentical hematopoietic stem cell transplantation for adult and pediatric patients.

Hematopoietic stem cell transplantation (HSCT) from haploidentical donors is a viable option for patients lacking HLA-matched donors. Here we report the results of a prospective multicenter phase I/II trial of transplantation of TCRαβ and CD19-depleted peripheral blood stem cells from haploidentical family donors after a reduced-intensity conditioning with fludarabine, thiotepa, and melphalan. Thirty pediatric and 30 adult patients with acute leukemia (n = 43), myelodysplastic or myeloproliferative syndrome (n = 6), multiple myeloma (n = 1), solid tumors (n = 6), and non-malignant disorders (n

Transient Leukemoid Reaction from T-Cell Large Granular Lymphocytes Post Autologous Stem Cell Transplant in a Patient Affected by Hodgkin Lymphoma.

Monoclonal T-cell lymphocytosis has been reported in patients with concomitant autoimmune diseases, viral infections, or immunodeficiencies. Referred to as T-cell large granular lymphocytic leukemia (T-LGLL), most cases cannot identify the triggering cause. Only small case series have been reported in the literature, and no treatment consensus exists. T-cell lymphocytosis may also appear after the transplant of hematopoietic stem cells or solid organs. Rare cases have been reported in patients undergoing autologous stem cell transplant (ASCT) for hematological diseases (including multiple myel

Severe autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps.

Autoimmune hemolytic anemia (AIHA) is an acquired hemolytic disorder, mediated by auto-antibodies, and has a variable clinical course ranging from fully compensated low grade hemolysis to severe life-threatening cases. The rarity, heterogeneity and incomplete understanding of severe AIHA complicate the recognition and management of severe cases. In this review, we describe how severe AIHA can be defined and what is currently known of the severity and outcome of AIHA. There are no validated predictors for severe clinical course, but certain risk factors for poor outcomes (hospitalisation, trans

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… AIHA is classified as either primary or secondary to an underlying disease, including lymphoproliferative disorders, immunodeficiencies, infectious diseases and other auto-immune diseases ( 5 ). Primary cAIHA is referred to as cold agglutinin disease (CAD). … In the most recent revision of the WHO classification, the presence of a B-cell clone and CAs is defined as a separate entity called CAD-Lymphoproliferative disorder (CAD-LPD) ( 6 ). …

Review of Paraneoplastic Syndromes in Children with Malignancy.

A paraneoplastic syndrome (PNS) is a set of symptoms and signs that can accompany the formation of a cancer but is not due to its direct infiltration or metastasis. PNS results from the secretion of peptides or hormones by tumor cells or from an immunological cross-reaction between the tumor antigens and host antigens. In the adult population, PNS affects up to 15% of patients with cancer, but data on pediatric patients remain lacking. The remarkable fact is that PNS can precede an oncology diagnosis, even by months or years. PNS can involve virtually any organ of the human body. In children,

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… Hepatic manifestations of lymphoproliferative disorders. Clin Liver Dis. 2019;23(2):293–308. doi: 10.1016/j.cld.2018.12.010. 40. Bakhit M, McCarty TR, Park S, et al. Vanishing bile duct syndrome in Hodgkin’s lymphoma: A case report and literature review. …