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Prognosis and treatment of plasmablastic lymphoma in the United States: a multicenter retrospective study.

Plasmablastic lymphoma (PBL) is a rare, aggressive AIDS-related lymphoma observed in patients with immunosuppressed states as well as in immunocompetent individuals. We sought to determine survival outcomes, prognostic factors, and optimal treatment regimens in a large, contemporary cohort of patients with PBL in the United States. We performed a multicenter, retrospective cohort study, including 344 patients diagnosed with PBL between 2005 and 2022. Patients were stratified into cohorts according to underlying immune status. Survival outcomes were calculated using Kaplan-Meier statistics, wit

Renal Involvement in Mixed Cryoglobulinemic Vasculitis: Current Perspectives.

Cryoglobulinemia is a rare disorder characterized by the presence of abnormal proteins (cryoglobulins) in the blood that precipitate at low temperatures. It presents with a wide clinical spectrum, from mild symptoms to severe, life-threatening disease. In mixed cryoglobulinemia (MC), vascular damage results from immune complexes-typically monoclonal IgM with rheumatoid factor activity and polyclonal IgG (Type II), or polyclonal/oligoclonal IgM and IgG (Type III). These complexes can obstruct small blood vessels, leading to ischemia and leukocytoclastic vasculitis. Renal involvement occurs in a

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… These include in situ binding of clonally restricted IgM with high affinity for glomerular matrix components, as well as subclinical, smoldering lymphoproliferative disorders that drive chronic and progressive renal injury [ 11 ]. 4. … Conversely, persistent cryoglobulinemia due to untreated chronic infections, systemic autoimmune disease with persistent activity, or untreated B-cell lymphoproliferative disorders are associated with a higher risk of systemic cryoglobulinemic vasculitis, cardiovascular complications, and also kidney …

Anti-Tubular Basement Membrane Antibody Nephritis Manifesting in a Patient With Chronic Lymphocytic Leukemia: A Very Rare Case Report.

Anti-tubular basement membrane (anti-TBM) antibody nephritis is a rare type of tubulointerstitial nephritis associated with progressive decline in kidney function. It is characterized histopathologically by tubular atrophy and dilation, interstitial fibrosis, lymphocyte and macrophage-predominant cellular infiltration, and linear deposition of IgG and complement along the tubular basement membrane. We herein present a case of a 69-year-old male who was recently diagnosed with chronic lymphocytic leukemia (CLL) and was referred for evaluation of kidney failure, ultimately diagnosed as anti-TBM

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… The present case is the first report of anti-TBM antibody nephritis in a background of a lymphoplasmacytic neoplasm, whereas a literature review revealed no clear association between lymphoproliferative disorders and anti-TBM disease. … This case also highlights the limited existing literature supporting a direct relationship between lymphoproliferative disorders and anti-TBM disease. …

Waldenström Macroglobulinemia - A State-of-the-Art Review: Part 1: Epidemiology, Pathogenesis, Clinicopathologic Characteristics, Differential Diagnosis, Risk Stratification, and Clinical Problems.

Waldenström macroglobulinemia (WM) is an infrequent variant of lymphoma, classified as a B-cell malignancy identified by the presence of IgM paraprotein, infiltration of clonal, small lymphoplasmacytic B cells in the bone marrow, and the MYD88 L265P mutation, which is observed in over 90% of cases. The direct invasion of the malignant cells into tissues like lymph nodes and spleen, along with the immune response related to IgM, can also lead to various health complications, such as cytopenias, hyperviscosity, peripheral neuropathy, amyloidosis, and Bing-Neel syndrome. Chemoimmunotherapy has hi

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… Risk of lymphoproliferative disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population-based study in Sweden. Blood. 2008 Oct 15;112(8):3052–6. doi: 10.1182/blood-2008-06-162768. 23. … MYD88 L265P in Waldenstrommacroglobulinemia, immunoglobulin M monoclonal gammopathy, and other B-cell lymphoproliferative disorders using conventional and quantitative allele-specific polymerase chain reaction. Blood. 2013;121:2051–8. doi: 10.1182/blood-2013-05-502211. 52. …

Ocular Manifestations Associated with Hematologic Malignancies: Mechanisms, Diagnosis, and Management.

Background: Hematologic malignancies (HMs), including leukemia and lymphoma, are systemic diseases that may cause a wide range of ocular manifestations. Methods: We searched PubMed/MEDLINE (2015-2026) and identified articles with an emphasis on clinically relevant studies and recent developments. Results: Clinically, ocular involvement presents with diverse manifestations, including retinal hemorrhage, vitreoretinal lymphoma, choroidal infiltration, orbital masses, treatment-related ocular toxicities, graft-versus-host disease, and secondary infectious complications. These findings may mimic o

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… Some MRI features can also be helpful in differentiating orbital lymphoma from benign orbital lymphoproliferative disorders. … Ill-defined tumor margins were significantly associated with orbital lymphoma, whereas “flow void sign” and radiologic evidence of sinusitis ( p = 0.0002) were associated with benign orbital lymphoproliferative disorders [ 31 ]. …

Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive life-threatening disease that consists of uncontrolled activated lymphocytes and macrophages that secrete excessive cytokines. Symptoms and laboratory findings of HLH include prolonged fever, cytopenia, hepatosplenomegaly, liver dysfunction, hypertriglyceridemia, hyperferritinemia, increased soluble interleukin-2 receptor, low fibrinogen, and neurological problems. HLH has two forms: primary (familial autosomal recessive) or secondary (related to infections, malignancy, autoimmune and metabolic disorders, transplantations, chimeric anti

Precision medicine: The use of tailored therapy in primary immunodeficiencies.

Primary immunodeficiencies (PID) are rare, complex diseases that can be characterised by a spectrum of phenotypes, from increased susceptibility to infections to autoimmunity, allergy, auto-inflammatory diseases and predisposition to malignancy. With the introduction of genetic testing in these patients and wider use of next-Generation sequencing techniques, a higher number of pathogenic genetic variants and conditions have been identified, allowing the development of new, targeted treatments in PID. The concept of precision medicine, that aims to tailor the medical interventions to each patie

Dural Plasmacytoma with Meningeal Myelomatosis in a Patient with Multiple Myeloma.

Here, we describe the case of a 66-year-old male diagnosed with multiple myeloma who presented with generalized tonic-clonic seizures. Magnetic resonance imaging demonstrated a right solid extra-axial parieto-occipital lesion with typical characteristics of meningeal myelomatosis. Biopsy was performed, which diagnosed a dural plasmacytoma. Because of this, we started concomitant therapy with radiotherapy and lenalidomide, but the patient has a poor response to treatment and died few weeks after its initiation. Myelomatous involvement of the dura mater is a rare occurrence, given that only few

Targeted treatment of autoimmune cytopenias in primary immunodeficiencies.

Primary Immunodeficiencies (PID) are a group of rare congenital disorders of the immune system. Autoimmune cytopenia (AIC) represents the most common autoimmune manifestation in PID patients. Treatment of AIC in PID patients can be really challenging, since they are often chronic, relapsing and refractory to first line therapies, thus requiring a broad variety of alternative therapeutic options. Moreover, immunosuppression should be fine balanced considering the increased susceptibility to infections in these patients. Specific therapeutic guidelines for AIC in PID patients are lacking. Treatm

Risk of Hematologic Malignancies in Patients with Inflammatory Bowel Disease: A Meta-Analysis of Cohort Studies.

Background/aimsInflammatory bowel disease (IBD) may contribute to the development of hematologic malignancies. In this study, the potential relationship between IBD and hematologic malignancies was investigated.MethodsWe searched the PubMed, Web of Science, Embase, and Cochrane Library databases for all cohort studies comparing the incidence of hematologic malignancies in non-IBD populations with that in IBD patients, and we extracted relevant data from January 2000 to June 2023 for meta-analysis.ResultsTwenty cohort studies involving 756,377 participants were included in this study. The resul

Supportive Care in Older Lymphoma Patients to Reduce Toxicity and Preserve Quality of Life

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… disorders. … Walewski J. 2010 Update of EORTC Guidelines for the Use of Granulocyte-Colony Stimulating Factor to Reduce the Incidence of Chemotherapy-Induced Febrile Neutropenia in Adult Patients with Lymphoproliferative Disorders and Solid Tumours Eur. J. …

GvHD prophylaxis with tacrolimus, sirolimus, and mycophenolate mofetil after reduced intensity conditioning hematopoietic stem cell allogeneic transplantation.

We present the largest prospective real-world experience in 159 patients who received the triple combination of tacrolimus/sirolimus/mycophenolate mofetil after reduced intensity conditioning allogeneic hematopoietic stem cell transplantation (RIC-alloHSCT) from matched-related (MRD), matched-unrelated (MUD) or mismatched-unrelated donors (MMURD). Despite the high-risk and elderly population, non-relapse mortality (NRM) at day +100 and 1 year was 5.1% and 8.6%. Grades 2-4 and 3-4 acute Graft-versus-host disease (GvHD) at day +180 was 30.3% and 13%, respectively. Chronic GvHD at 1 and 3 years w

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… Styczynski J van der Velden W Fox CP Engelhard D de la Camara R Cordonnier C 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) … 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. …

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 …

Prospective implementation of the HCT frailty scale in adults with lymphoproliferative syndromes candidates for autologous haematopoietic stem cell transplantation: Results from a multicentre GETH-TC study.

This prospective study evaluated the incidence and dynamics of frailty in 156 adults with lymphoproliferative syndromes undergoing autologous haematopoietic stem cell transplantation (HSCT). Frailty was assessed using the haematopoietic cell transplantation (HCT) frailty scale in patients from 15 Spanish institutions at initial consultation, transplant admission and day +100. Quality of life (QoL) was measured using the EuroQoL. At first consultation, 45 patients (28.8%) were classified as fit, 93 (59.6%) as pre-frail and 18 (11.6%) as frail. Frailty was more frequent among older patients, tho

Recognition and management of early complications in CAR-T cell therapy and virus-specific T-lymphocyte therapy: a practical clinical reference.

BACKGROUND: Cellular immunotherapies-including chimeric antigen receptor T-cell (CAR-T) therapies and adoptive transfer of virus-specific T lymphocytes (VSTs) - have transformed the treatment of refractory hematological malignancies and post-transplant infectious complications. Eight products are currently authorized by the European Medicines Agency, encompassing seven autologous CAR-T products targeting CD19 or BCMA and tabelecleucel (Ebvallo), the first approved allogeneic off-the-shelf EBV-specific T-cell product for EBV-positive post-transplant lymphoproliferative disease. Despite their cl

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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 . …

Case Report: Autologous stem cell transplantation in a patient diagnosed with AL amyloidosis in Kazakhstan.

INTRODUCTION: AL amyloidosis is a rare plasma cell disorder that causes progressive organ dysfunction due to extracellular deposition of misfolded immunoglobulin light chains. Reports from Central Asia are scarce, and there is limited documentation of successful integration of daratumumab-based therapy with autologous hematopoietic stem cell transplantation (auto-HSCT) in this region. CASE PRESENTATION: A 65-year-old woman presented with progressive left supraclavicular lymphadenopathy. Examination revealed bilateral cervical and supraclavicular lymphadenopathy with an ECOG performance status

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… First, the disease presented predominantly with extensive extramedullary lymph node involvement, an uncommon manifestation of AL amyloidosis that can mimic lymphoproliferative disorders and delay diagnosis. … Given the presence of progressive lymphadenopathy, the differential diagnosis initially included lymphoproliferative disorders such as non-Hodgkin lymphoma, plasma cell myeloma with extramedullary involvement, and other forms of systemic amyloidosis. …

Pityriasis liechenoides et varioliformis acuta у пациента, лечащегося cevostamab.

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Safety of CAR-T Cell Therapy in Patients With Renal Failure/Acute Kidney Injury: Focused Review.

Chimeric antigen receptor (CAR) T-cell therapy is novel immunotherapy targeting specifically cancerous cells, and has been shown to induce durable remissions in some refractory hematological malignancies. However, CAR T-cell therapy has adverse effects, such as cytokine release syndrome (CRS), immune effector-associated neurotoxicity syndrome (ICANS), tumor lysis syndrome (TLS), and acute kidney injury (AKI), among others. Not many studies have covered the repercussions of CAR T-cell therapy on the kidneys. In this review, we summarized the available evidence on the safety profile of CAR T-cel

Ubiquitin-specific peptidases in lymphoma: a path to novel therapeutics.

BackgroundUbiquitin-specific peptidases (USPs), also known as deubiquitinating enzymes (DUBs), play a crucial role in maintaining cellular homeostasis by selectively removing ubiquitin molecules from targeted proteins. This process affects protein stability, subcellular localization, and activity, thereby influencing processes such as DNA repair, cell cycle regulation, and apoptosis. Abnormal USP activities have been linked to various diseases, including cancer. Emerging evidence in lymphoma studies highlights the significance of USPs in controlling signaling pathways related to cancer initiat

Waldenstrom Macroglobulinemia Leading to Cholestatic Liver Injury and Rapid Progression to Liver Failure.

Waldenstrom macroglobulinemia (WM) is an indolent B-cell neoplasm with generally good prognosis, and while hepatic involvement in WM has been documented in the literature, it is rarely clinically significant. We present a case of a 77-year-old man with WM who presented with a cholestatic liver injury and rapid progression to decompensated liver disease. The patient ultimately transitioned to comfort care and died from liver failure. This case not only highlights the rarity of this poor outcome from WM, but also underscores the importance of early recognition of infiltrative malignancy as a pot