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Mimicking urticaria: a Schnitzler syndrome case.

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Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

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… Ferri C La Civita L Longombardo G Zignego AL Pasero G Mixed cryoglobulinaemia: a cross-road between autoimmune and lymphoproliferative disorders Lupus. 1998 7 4 275 279 9643318 10.1191/096120398678920091 18. O’Donnell B Black AK Urticarial vasculitis Int Angiol. 1995 14 2 166 174 8609443 19. …

Secondary Immunodeficiency in Hematological Malignancies: Focus on Multiple Myeloma and Chronic Lymphocytic Leukemia.

Secondary immunodeficiency is reported in most patients with hematological malignancies such as chronic lymphocytic leukemia and multiple myeloma. The aim of our review was to evaluate the existing literature data on patients with hematological malignancies, with regard to the effect of immunodeficiency on the outcome, the clinical and therapeutic approach, and on the onset of noninfectious complications, including thrombosis, pleural effusion, and orofacial complications. Immunodeficiency in these patients has an intense impact on their risk of infection, in turn increasing morbidity and mort

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… In the clinical trial with idelalisib (a phosphatidylinositol 3-kinase inhibitor) for refractory CLL, different infections were described such as pneumonia (6%) and febrile neutropenia (5%) ( 165 ); a diverse study performed with idelalisib for diverse lymphoproliferative disorders confirmed the occurrence … Subcutaneous Immunoglobulin in Lymphoproliferative Disorders and Rituximab-Related Secondary Hypogammaglobulinemia: A Single-Center Experience in 61 Patients . …

Extreme Peripheral Blood Plasmacytosis Mimicking Plasma Cell Leukemia as a Presenting Feature of Angioimmunoblastic T-Cell Lymphoma (AITL).

Angioimmunoblastic T-cell lymphoma (AITL) is one of four major subtypes of nodal peripheral T cell lymphoma, characterized by its cell of origin, the follicular helper T-cell (TFH). Patients typically present with prominent constitutional (B) symptoms, generalized lymphadenopathy, hepatosplenomegaly, cytopenias, and rash. Here we present a case of a 62-year-old male with progressive cervical adenopathy, fevers and weight loss presenting with extreme polyclonal plasmacytosis and high plasma EBV viral load. While the initial presentation appeared to mimic plasma cell leukemia or severe infection

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… After bone marrow and lymph node biopsies were obtained, dexamethasone 40 mg daily was initiated, as concerns for an EBV associated lymphoproliferative disorder (EBV-LPD) were high. … Epstein-barr virus–associated lymphoproliferative disorders: review and update on 2016 WHO Classification. J Pathol Transl Med. (2017) 51:352–8. 10.4132/jptm.2017.03.15 9. Okuyama S, Terada T, Kumagai H, Tsumanuma R, Omoto E, Ueki T, et al. …

A prospective clinical trial of GVHD prophylaxis with posttransplant cyclophosphamide and abatacept.

AbstractWe conducted a prospective randomized clinical trial to investigate the combination of posttransplant cyclophosphamide (PTCy) and abatacept (Aba) for graft-versus-host disease (GVHD) prophylaxis. Patients with hematologic malignancies undergoing an allogeneic transplant from an 8/8 matched related or unrelated donor were randomized 1:1 to tacrolimus and methotrexate (standard-of-care arm [SOC]) or PTCy on days +3 and +4, followed by Aba on an extended schedule: days +5, +14, and +28, and every 4 weeks up to day +168 (PTCy+Aba). All patients received peripherally collected stem cells. T

The choice of new treatments in autoimmune hemolytic anemia: how to pick from the basket?

Autoimmune hemolytic anemia (AIHA) is defined by increased erythrocyte turnover mediated by autoimmune mechanisms. While corticosteroids remain first-line therapy in most cases of warm-antibody AIHA, cold agglutinin disease is treated by targeting the underlying clonal B-cell proliferation or the classical complement activation pathway. Several new established or investigational drugs and treatment regimens have appeared during the last 1-2 decades, resulting in an improvement of therapy options but also raising challenges on how to select the best treatment in individual patients. In severe w

A case of coincident multiple myeloma and non-Hodgkin's lymphoma.

A rare case of coexisting multiple myeloma and non-Hodgkin's lymphoma at the time of diagnosis is presented. The patient presented with petechiae, melena and weight loss. IgA lambda monoclonal gammopathy in the serum and free lambda chain in urine were documented. Bone marrow biopsy demonstrated an interstitial infiltration of neoplastic plasma cells coexisting with localized collection of neoplastic lymphoid cells composed of monotonous small lymphocytes with occasional cleaved nuclei. Immunophenotype of plasma cell was IgA lambda. The patient also had a jejunal mass, with biopsy proven malig

Recommendations of the SFH (French Society of Haematology) for the diagnosis, treatment and follow-up of hairy cell leukaemia.

Hairy cell leukaemia (HCL) is a rare haematological malignancy, with approximately 175 new incident cases in France. Diagnosis is based on a careful examination of the blood smear and immunophenotyping of the tumour cells, with a panel of four markers being used specifically to screen for hairy cells (CD11c, CD25, CD103 and CD123). In 2011, the V600E mutation of the BRAF gene in exon 15 was identified in HCL; being present in HCL, it is absent in the variant form of HCL (HCL-v) and in splenic red pulp lymphoma (SRPL), two entities related to HCL. The management of patients with HCL has changed

Overview of approved CAR-T products and utility in clinical practice.

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Incidence of malignancies after lung transplantation and their effect on the outcome. 26 years' experience.

BackgroundMalignancy is a significant, life-limiting complication after lung transplantation (LuTx) and the second common long-term cause of death. We aimed to investigate its incidence and effect on the outcome.MethodsThis is a retrospective observational study. Between 1996 and 2022, n = 627 lung transplantations (LuTx) were performed in our department. We used our institutional database to identify recipients with malignancies after LuTx and examined the malignancies' incidence and mortality.ResultsN = 59 malignancies occurred in n = 55 (8.8%) LuTx recipients. The post-LTx malignancies inci

Autoimmune Hemolytic Anemias: Challenges in Diagnosis and Therapy.

BackgroundAutoimmune hemolytic anemia (AIHA) is a rare disease due to increased destruction of erythrocytes by autoantibodies, with or without complement activation.SummaryAIHA is usually classified in warm AIHA (wAIHA) and cold agglutinin disease (CAD), based on isotype and thermal amplitude of the autoantibody. The direct antiglobulin test (DAT) or Coombs test is the cornerstone of AIHA diagnosis, as it is positive with anti-IgG in wAIHA, and with anti-C3d/IgM antisera plus high titer cold agglutinins in CAD. Therapy is quite different, as steroids and rituximab are effective in the former,

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… As for other AIHAs secondary forms (identified as cold agglutinin syndrome or secondary CAD) should be carefully investigated, as the association with infections, lymphoproliferative disorders and tumors is particularly frequent [ 1 – 4 ]. … Of note, bone marrow re-examination by a centralized expert center greatly increased the proportion of CAD diagnosis versus other indolent lymphoproliferative disorders [ 12 ]. …

Exploring the Significance of Immune Checkpoints and EBV Reactivation in Antibody Deficiencies with Near-Normal Immunoglobulin Levels or Hyperimmunoglobulinemia.

This study delves into the intricate landscape of primary immunodeficiencies, with a particular focus on antibody deficiencies characterized by near-normal immunoglobulin levels or hyperimmunoglobulinemia. Contrary to the conventional focus on genetic dysregulation, these studies investigate the key roles of immune checkpoints, such as PD-1/PD-L1, CTLA-4/CD86, and CD200R/CD200, on selected subpopulations of T and B lymphocytes and their serum concentrations of soluble forms in patients recruited for the studies in healthy volunteers. In addition, the studies also show the role of Epstein-Barr

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… The Epstein-Barr Virus Hacks Immune Checkpoints: Evidence and Consequences for Lymphoproliferative Disorders and Cancers. Biomolecules. 2022;12:397. doi: 10.3390/biom12030397. 34. …

History of autoimmune disease is associated with impaired survival in multiple myeloma and monoclonal gammopathy of undetermined significance: a population-based study.

Multiple myeloma (MM) is a plasma cell disorder preceded by monoclonal gammopathy of undetermined significance (MGUS). Incidence of MM and MGUS is higher among patients with autoimmune disease. The aim of this study was to determine whether a history of autoimmunity has an impact on survival in MM and MGUS. Using high-quality national Swedish registries, we identified 8367 patients with MM, 18,768 patients with MGUS, and 110,251 matched control subjects, and obtained information on previous autoimmune disease in patients and controls. Cox regression was used to calculate hazard ratios (HRs) fo

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… Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic, pre-malignant condition with an average risk of progression to MM or other lymphoproliferative disorders of 1 % per year [ 3 ]. The etiology of MM and MGUS is largely unknown. … Risk of plasma cell and lymphoproliferative disorders among 14621 first-degree relatives of 4458 patients with monoclonal gammopathy of undetermined significance in Sweden. Blood. 2009;114:791–795. doi: 10.1182/blood-2008-12-191676. 7. …

Acute kidney injury following CAR-T cell therapy: a nephrologist's perspective.

Chimeric antigen receptor T (CAR-T) cell therapy, an emerging personalized immunotherapy for various haematologic malignancies, autoimmune diseases and other conditions, involves the modification of patients' T cells to express a chimeric antigen receptor that recognizes tumour or autoimmune cell antigens, allowing CAR-T cells to destroy cancerous and other target cells selectively. Despite remarkable clinical improvements in patients, multiple adverse effects have been associated with CAR-T cell therapy. Among the most recognized adverse effects are cytokine release syndrome, immune effector

Clinical outcomes of immunoglobulin treatment for patients with secondary antibody deficiency: Data from the Ontario immunoglobulin treatment case registry.

BackgroundDespite the increasing number of cases of secondary antibody deficiency (SAD) and immunoglobulin (Ig) utilization, there is a paucity of data in the literature on clinical and patient-reported outcomes in this population.ObjectiveTo describe immunoglobulin utilization patterns, clinical and patient-reported outcomes in patients with SAD on immunoglobulin replacement therapy (IgRT).MethodsA cross-sectional study of patients with secondary antibody deficiency enrolled in the Ontario Immunoglobulin Treatment (ONIT) Case Registry from June 2020 to September 2022 was completed. Demographi

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… The most common SOT was kidney transplant (KT) (N = 5, 45.4%), 2 of whom had prolonged immunosuppressive therapies (>10years) for post-transplant lymphoproliferative disorder and chronic organ rejection. … Subcutaneous immunoglobulin in lymphoproliferative disorders and rituximab-related secondary hypogammaglobulinemia: a single-center experience in 61 patients. Haematologica. 2014. Jun;99(6):1101–6. doi: 10.3324/haematol.2013.101261 18. …

Chronic Lymphocytic Leukemia-Induced Humoral Immunosuppression: A Systematic Review.

Secondary immunodeficiency is observed in all patients with chronic lymphocytic leukemia (CLL) in varying degrees. The aim of the study was to review the available literature data on patients with CLL, with particular regard to the pathogenesis of the disease and the impact of humoral immunity deficiency on the clinical and therapeutic approach. A systematic literature review was carried out by two independent authors who searched PubMed databases for studies published up to January 2020. Additionally, Google Scholar was used to evaluate search results and support manual research. The search r

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… According to the 2008 World Health Organization (WHO) classification, Chronic Lymphocytic Leukemia is defined as a lymphoproliferative disorder characterized by a progressive monoclonal absolute lymphocytosis of small, mature-looking CD5 + /CD19 + B cells’ accumulation in the peripheral blood (PB), bone … Functional B cell receptors (BCR) expressed on the surface of chronic lymphocytic leukemia cells and superficial IG expression are crucial for the survival and function of normal B cells and many lymphoproliferative disorders [ 8 ]. …

A case of renal damage induced by cryoglobulinemia associated with Monoclonal Gammopathy of Renal Significance.

BACKGROUND: Cryoglobulins are abnormal immunoglobulins present in circulation, capable of precipitating at lower temperatures and redissolving upon rewarming to body temperature (37 °C). It is widely accepted that mixed cryoglobulinemia is more commonly seen in chronic infections and autoimmune diseases. Here, we present a case of cryoglobulinemic nephritis due to Monoclonal Gammopathy of Renal Significance. CASE PRESENTATION: The patient was a 60-year-old male who presented with “bilateral lower limb edema for one month.” Clinical features included purpura on the lower extremities, proteinuri

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… Mixed cryoglobulinemia syndrome associated with Non-HCV B-Cell lymphoproliferative disorder presenting with gangrene and peripheral neuropathy. R I Med J (2013). 2024;107:15–9. 39186395 7. … Kidney involvement of patients with Waldenström macroglobulinemia and other IgM-Producing B cell lymphoproliferative disorders. Clin J Am Soc Nephrology: CJASN. 2018;13:1037–46. 10.2215/CJN.13041117 PMC6032586 29848505 12. …

Intrathoracic manifestations of hematologic malignancies: a narrative review.

BACKGROUND AND OBJECTIVE: Although the global incidence of hematologic malignancies is decreasing, the risk of developing these cancers is increasing due to increased lifespan and modern treatments including targeted therapy, chemotherapy, and radiation. Intrathoracic manifestations of hematologic malignancies may be the presenting sign leading to diagnosis or result from various treatment toxicity. The most common respiratory manifestation of hematologic malignancies is infectious, but up to half of pulmonary findings are from non-infectious etiologies. We aim to summarize the current literat

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… Many primary mediastinal malignancies in both adults and children include lymphoproliferative disorders such as HL, primary mediastinal (thymic) large B-cell lymphoma (PMBCL), T-lymphoblastic lymphoma (T-LBL) and anaplastic large cell lymphoma ( Figure 2 ). … Mediastinal Lymphoproliferative Disorders. Adv Anat Pathol 2021;28:307-34. 10.1097/PAP.0000000000000305 9. Falchi L, Keating MJ, Marom EM, et al. Correlation between FDG/PET, histology, characteristics, and survival in 332 patients with chronic lymphoid leukemia. …

Tailoring cells for clinical needs: Meeting report from the Advanced Therapy in Healthcare symposium (October 28-29 2017, Doha, Qatar).

New technologies and therapies designed to facilitate development of personalized treatments are rapidly emerging in the field of biomedicine. Strikingly, the goal of personalized medicine refined the concept of therapy by developing cell-based therapies, the so-called "living drugs". Breakthrough advancements were achieved in this regard in the fields of gene therapy, cell therapy, tissue-engineered products and advanced therapeutic techniques. The Advanced Therapies in Healthcare symposium, organized by the Clinical Research Center Department of Sidra Medicine, in Doha, Qatar (October 2017),

Clinical characteristics and outcome of 318 families with familial monoclonal gammopathy: A multicenter Intergroupe Francophone du Myélome study.

Familial forms of monoclonal gammopathy, defined as multiple myeloma (MM) or Monoclonal Gammopathy of Undetermined Significance (MGUS), are relatively infrequent and most series reported in the literature describe a limited number of families. MM rarely occurs in a familial context. MGUS is observed much more commonly, which can in some cases evolve toward full-blown MM. Although recurrent cytogenetic abnormalities have been described in tumor cells of sporadic cases of MM, the pathogenesis of familial MM remains largely unexplained. In order to identify genetic factors predisposing to familia

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… Visram et al. reported a higher risk of progression to a plasma cell or a lymphoproliferative disorder in familial versus nonfamilial MGUS cases in spite of a 50% lower risk of death in familial MGUS patients. … Risk of lymphoproliferative disorders among first‐degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population‐based study in Sweden. Blood. 2008;112(8):3052‐3056. 4. Schinasi LH, Brown EE, Camp NJ, et al. …