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Does Double Mean Trouble? Coexistence of Myeloproliferative and Lymphoproliferative Neoplasms.

Background: The occurrence of myeloproliferative neoplasms (MPNs) that evolve into each other is well-described, as is this occurrence of lymphoproliferative neoplasms (LPNs). However, less is known about rare MPN/LPN coexistence, and the aim of our study was to analyze charachteristics of these patients after long term follow-up. Methods: Fourteen patients with MPN/LPN coexistence were diagnosed and treated according to guidelines at a single university center across two decades. Results: The overall median age was 53 years (22-69). MPNs patients with subsequent LPNs had a shorter period of s

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… Myeloproliferative and lymphoproliferative disorders: State of the art. Hematol. Oncol. 2020;38:121–128. doi: 10.1002/hon.2701. 9. Landgren O., Goldin L.R., Kristinsson S.Y., Helgadottir E.A., Samuelsson J., Björkholm M. … Lymphoproliferative disorders in patients with chronic myeloproliferative neoplasms: A systematic review. Am. J. Hematol. 2018;93:698–703. doi: 10.1002/ajh.25049. 17. …

Noninfectious pulmonary complications of haematopoietic stem cell transplantation.

Haematopoietic stem cell transplantation (HSCT) is an established treatment for a variety of malignant and nonmalignant conditions. Pulmonary complications, both infectious and noninfectious, are a major cause of morbidity and mortality in patients who undergo HSCT. Recent advances in prophylaxis and treatment of infectious complications has increased the significance of noninfectious pulmonary conditions. Acute lung injury associated with idiopathic pneumonia syndrome remains a major acute complication with high morbidity and mortality. On the other hand, bronchiolitis obliterans syndrome is

COVID-19 in 96 Patients With Hematologic Disease: The First Single-center Experience From the Czech Republic.

BackgroundCoronavirus disease 2019 (COVID-19) represents an important infectious complication associated with high mortality rates in patients with hematologic diseases. There have not been published any epidemiologic studies from Czech Republic so far.Patients and methodsThis study is the first analysis of patients with hematologic malignancies and bone marrow failure syndromes treated at single hematology center in the Czech Republic between March 1 and December 31, 2020, in whom COVID-19 infection was confirmed.ResultsThe sample comprised 96 patients aged 26 to 84 years (median, 66.0 years)

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… Underlying Hematologic Disease Lymphoproliferative disorders were present in 65 patients (67.7%), accounting for the largest subgroup of diagnoses in the sample. … Lymphoproliferative disorders were seen in 9 patients (including 6 patients with chronic lymphocytic leukemia) and myeloproliferative disorders in 6 patients (4 acute myeloid leukemia cases including 1 acute promyelocytic leukemia case). …

Effects of erythropoiesis-stimulating agents on survival and other outcomes in patients with lymphoproliferative malignancies: a study-level meta-analysis.

Erythropoiesis-stimulating agents (ESAs) are approved to treat anemia in patients with non-myeloid malignancies receiving myelosuppressive chemotherapy. ESAs reduce transfusion rates, but some clinical studies suggest that ESAs may reduce survival or increase disease progression. This study-level meta-analysis examined the effects of darbepoetin alfa, epoetin alfa or epoetin beta on mortality, disease progression and transfusion incidence in patients with lymphoproliferative malignancies, using randomized, controlled trials of patients receiving chemotherapy and ESAs or standard of care. The o

Liver Disease Associated with Non-Hepatitis Viruses

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… Thus, reduction in immunosuppression, anticancer chemotherapy, and B-cell depleting antibodies are needed to treat EBV-related lymphoproliferative disorders. … Fatal case of Epstein-Barr virus-associated lymphoproliferative disorder associated with a human immunodeficiency virus infection Archives of Pathology & Laboratory Medicine 111 1987 250 253 3030225 Bonacini M. …

Treating rheumatic patients with a malignancy.

Management of patients with inflammatory rheumatic disease and a history of (or even a current) malignant disease poses some particular challenges. As direct evidence of the risk of (recurrent or de novo) malignancy in patients with a history of malignant disease is scarce, such a risk may be estimated indirectly from the principal carcinogenicity of the respective drug to be used or (also indirectly) from cancer reactivation data from the transplant literature. In general, cancer risk is increased in patients receiving combination immunosuppressive treatment, but the risk in patients receivin

Non-infectious pulmonary complications after haematopoietic progenitor transplantation: a diagnostic approach.

Haematopoietic stem cell transplantation (HCT) is an established treatment for a wide variety of haematological diseases, both malignant and non-malignant. Infectious and non-infectious post-HCT pulmonary complications are a major cause of morbidity and mortality, with non-infectious complications becoming more prominent in recent decades as prophylaxis has led to a decrease in infectious complications. Globally, these complications can be divided into three phases (neutropenic, early and late phase) depending on their time of onset in relation to the graft. There is a growing awareness that t

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… disorder; d, days; m, month; y, years. … Thoracic presentations of posttransplant lymphoproliferative disorders. Chest 2004;126:2013-20. 10.1378/chest.126.6.2013 47. Heslop HE, Slobod KS, Pule MA, et al. …

Current clinical practice and challenges in the management of secondary immunodeficiency in hematological malignancies.

ObjectiveDespite long-standing safe and effective use of immunoglobulin replacement therapy (IgRT) in primary immunodeficiency, clinical data on IgRT in patients with secondary immunodeficiency (SID) due to B-cell lymphoproliferative diseases are limited. Here, we examine the correlation between approved IgRT indications, treatment recommendations, and clinical practice in SID.MethodsAn international online survey of 230 physicians responsible for the diagnosis of SID and the prescription of IgRT in patients with hematological malignancies was conducted.ResultsSerum immunoglobulin was measured

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… Subcutaneous immunoglobulin in lymphoproliferative disorders and rituximab‐related secondary hypogammaglobulinemia: a single‐center experience in 61 patients . Haematologica . 2014 ; 99 : 1101 ‐ 1106 . 24682509 10.3324/haematol.2013.101261 PMC4040915 19 Berger M . …

Viruses

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… Alemtuzumab in particular has been associated with rates of CMV viremia in patients with lymphoproliferative disorders of 15–44% [ 44 – 46 ]. … Castleman disease, a lymphoproliferative disorder, can lead to HHV-8-positive plasmablastic lymphoma. …

Clinical outcome of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly in the rituximab era.

Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of malignant lymphoma. The incidence of Epstein-Barr virus (EBV)-positive DLBCL in Asian and Latin American countries ranges from 8 to 10%. The prognosis of patients with EBV-positive DLBCL is controversial. To compare the clinical outcome of EBV-positive and EBV-negative patients with DLBCL in the rituximab era, we analyzed 239 patients with de novo DLBCL diagnosed between January 2007 and December 2011. The presence of EBV in lymphoma cells was detected using EBV-encoded RNA in situ hybridization, and it was found that 18 (6.9%

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… Lymphoblastoid cell lines cause some lymphoid malignancies, including Burkitt lymphoma, extranodal natural killer/T-cell lymphoma, aggressive natural killer leukemia/lymphoma, angioimmunoblastic T-cell lymphoma, Hodgkin's lymphoma, immunodeficiency-associated lymphoproliferative disorders, and some DLBCLs … Latency I is associated with EBV-related Burkitt lymphoma, latency II with classical Hodgkin's lymphoma and T-cell non-Hodgkin's lymphoma, and latency III occurs mainly in immunocompromised individuals suffering from post-transplant lymphoproliferative disorders and HIV-associated lymphoproliferative …

Pure red cell aplasia in a patient with rheumatoid arthritis after JAK inhibitor exposure-diagnostic challenges.

We describe a patient with long-standing rheumatoid arthritis (RA) who developed severe refractory transfusion-dependent anaemia lasting several months following treatment with a JAK1 inhibitor (Filgotinib). The patient proceeded with extensive investigations, including bone marrow assessment, and was initially diagnosed with Filgotinib-associated anaemia. However, despite stopping this agent, the anaemia persisted with fluctuating reticulocyte count resulting in multiple hospital admissions. Although the anaemia was initially attributed to Filgotinib, its persistence alongside delayed reticul

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… ANA = Anti-nuclear antibody; BM: Bone Marrow; CCP = Cyclic Citrullinated Peptide; CTD = Connective Tissue Disease; dsDNA = double stranded DNA; ENA = Extractable nuclear antigen; Epo = Erythropoietin; HIV = Human Immunodeficiency Virus; LPD: Lymphoproliferative disorder; MDS: Myelodysplastic Syndrome … Discussion Acquired PRCA is characterised by severe anaemia and reticulocytopenia, with a broad differential including autoimmune disease, lymphoproliferative disorders, infections and medications ( Table 3 ) [ 2 ]. …

Posttransplant complications: molecular mechanisms and therapeutic interventions.

Posttransplantation complications pose a major challenge to the long-term survival and quality of life of organ transplant recipients. These complications encompass immune-mediated complications, infectious complications, metabolic complications, and malignancies, with each type influenced by various risk factors and pathological mechanisms. The molecular mechanisms underlying posttransplantation complications involve a complex interplay of immunological, metabolic, and oncogenic processes, including innate and adaptive immune activation, immunosuppressant side effects, and viral reactivation.

Ухоженный терапевтический подход в пациенте с диффузной большой лимфомой B-Cell с инфильтрацией Менингеала, Consurning Classic Hodgkin Limphoma и Smolding Multieloma: Case Report.

КОЛИЧЕСТВО: Сосущественные первичные гематологические маньяки в неочищенных множественных миеломах (ММ) редки. Case: A 69-year-old Человек с мрачной миеломой (SMM) с лимфаданопатией и внутричерепной массой. У него диффузная большая лимфома B-клеток (DLBCL), классическая лимфома Ходжкина (cHL) и SMM. Пациент получил R-CHOP и R-ICE, за ним последовала аутлогическая трансплантация стволовых клеток, полная ремиссия DLBCL и CHL, и очень хорошая частичная ремиссия MM. ЗАКЛЮЧЕНИЕ: Этот случай иллюстрирует сложность регулирования множественных нарушений и важность индивидуального терапевтического подх

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… Suspecting a lymphoproliferative disorder, a CT‐guided core biopsy of the retroperitoneal mass was undertaken. … Previous case reports demonstrated the derivation from the same B‐cell lineage of concurrent B‐cell lymphoproliferative disorders using a direct sequencing method of CDR2, CDR3, and VH3 sequences of immunoglobulin genes [ 10 ]. …

Cryoglobulin Test and Cryoglobulinemia Hepatitis C-Virus Related.

Cryoglobulins are immunoglobulins that precipitate in serum at temperatures below 37°C and resolubilize upon warming. The clinical syndrome of cryoglobulinemia usually includes purpura, weakness, and arthralgia, but the underlying disease may also contribute other symptoms. Blood samples for cryoglobulin are collected, transported, clotted and spun at 37°C, before the precipitate is allowed to form when serum is stored at 4°C in a Wintrobe tube for at least seven days. The most critical and confounding factor affecting the cryoglobulin test is when the preanalytical phase is not fully complete

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… The majority of data concern HCV-related autoimmune and/or lymphoproliferative disorders, from mixed cryoglobulinemia vasculitis to frank lymphomas. 32 In particular, chronic infection of immunocompetent cells (T and B lymphocytes, macrophages) may be responsible for the proliferation of B lymphocytes … disorders. …

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler's Syndrome.

Schnitzler's syndrome is a rare autoinflammatory syndrome with unidentified mechanism of disease and etiology with unknown definitive treatment algorithm. The two obligatory criteria for the diagnosis of Schnitzler's syndrome include chronic urticarial rash and monoclonal gammopathy (immunoglobulin M or immunoglobulin G). In this article, we describe two patients with different courses of disease with different average lengths of time between initial symptoms and the final diagnosis (6 months to 8 years). Exclusion of more common conditions is needed to ensure the correct diagnosis. Treatment

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… Regular follow-ups of patients with Schnitzler’s syndrome are warranted due to higher risk for the development of secondary amyloidosis and lymphoproliferative disorders especially in untreated and undertreated patients. 12 In conclusion, this article reports two diverse clinical courses and different …

Extrahepatic Cancer Risk in Patients with Hepatitis C Virus Infection Treated with Direct-Acting Antivirals.

Chronic hepatitis C virus (HCV) infection is associated with an increased risk of extrahepatic cancers, particularly non-Hodgkin lymphoma. The introduction of direct-acting antivirals (DAAs) has revolutionized HCV therapy, resulting in high cure rates. However, concerns have been raised about potential effects on cancer risk. This review summarizes the current evidence on extrahepatic cancer risk in HCV-infected patients treated with DAAs. We examined epidemiologic data on HCV-associated extrahepatic cancers and explored potential mechanisms linking HCV to carcinogenesis outside the liver. Stu

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… B Cell Abnormalities Chronic HCV infection is associated with significant B-cell dysregulation, which may contribute to the increased risk of B-cell lymphoproliferative disorders observed in HCV-infected individuals. … This restoration of B-cell homeostasis could potentially reduce the risk of B-cell lymphoproliferative disorders associated with chronic HCV infection. …

Dermatological manifestations of hematologic neoplasms. Part II: nonspecific skin lesions/paraneoplastic diseases.

Cutaneous manifestations occur in the course of hematologic malignancies and precede, accompany or occur late in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, immunosuppression resulting from the hematologic disease itself or its treatment. The dermatologist must be aware of these conditions that may be helpful both in the diagnosis of the underlying disease and in reducing patient morbidity. This review (part II) addresses the paraneoplastic dermatological changes associated with systemic hematologic malignancies.

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… disorders. 73 , 74 The pathogenesis of DPX is not clearly defined. … Aprepitant in refractory pruritus of systemic lymphoproliferative disorders. J Eur Acad Dermatol Venereol. 2020;34:e422–e424. doi: 10.1111/jdv.16370. 42. Serra-García L., Riera-Monroig J., Riquelme-Mc Loughlin C., Daniel C.M.C. Chronic prurigo as a onset of Hodgkin’s lymphoma. …

Extraluminal amyloidoma of the pelvic cavity causing large bowel obstruction.

AMYLOIDOSIS IS A GROUP OF DIVERSE DISORDERS THAT FALL INTO SEVERAL MAJOR CATEGORIES: primary, secondary, dialysis-associated, and hereditary forms. Clinically, amyloidosis may be categorized as localized or systemic. The gastrointestinal tract is among the most common places for deposition of amyloid, but large, localized amyloid deposits are an uncommon occurrence and rarely cause extraluminal bowel compression resulting in obstruction as was seen in the case presented in this clinical scenario.

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… Aggressive chemotherapy has been used in cases where underlying lymphoproliferative disorders were known, sometimes in combination with marrow ablation and stem cell rescue [ 1 , 3 ]. …

Нынешний подход к решению проблемы вторичного недоедания у пациентов с гематологическими отклонениями: рекомендации в отношении консенсуса экспертов Испании.

Для оценки мнений клинических гематологов было проведено обследование на базе Делфи (n = 17) и клинических иммунологов (n = 18) по всей Испании о вторичных иммунодефицитах (СИД) в области лечения онкогематологических пациентов. Там было 100% :: согласие с необходимостью наличия руководящих принципов в отношении лечения иммунодефицита у пациентов, страдающих гематологией; проведение базовой иммунологической оценки у пациентов с хронической лимфоцитной лейкемией (КЛЛ), множественной миеломой (ММ), лимфомой и гематопоиетической трансплантацией стволовых клеток (ХСБТ); и количественная оценка уров

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… The incidence of SID is increasing due to the rapid expansion of the therapeutic armamentarium for lymphoproliferative disorders (LPD) in hematological malignancies. … Combined Immune Defect in B-Cell Lymphoproliferative Disorders Is Associated with Severe Infection and Cancer Progression. Biomedicines. 2022;10:2020. doi: 10.3390/biomedicines10082020. 35. …

Effectiveness of darbepoetin alfa in multiple myeloma patients receiving chemotherapy including novel agents.

BackgroundThe aim of this study was to evaluate the effects of darbepoetin alfa (DA) on hemoglobin (Hb) concentration and the need for transfusions in multiple myeloma (MM) patients receiving chemotherapy with novel agents.MethodsOf 251 patients with MM who received DA therapy for at least 4 weeks, 142 who did not receive RBC transfusion during 4 weeks after DA initiation and started DA therapy at baseline Hb ResultsAfter 4 weeks of DA therapy, 80 (60.6%) of 132 patients with evaluable data had Hb that increased ≥1.0 g/dL from baseline, while 50 (37.9%) had Hb that increased ≥2.0 g/dL from bas

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… Whether measurement of endogenous EPO levels is a useful tool for predicting response in patients with solid cancers or lymphoproliferative disorders is questionable [ 17 5 ]. …