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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

Once weekly selinexor, carfilzomib and dexamethasone in carfilzomib non-refractory multiple myeloma patients.

BACKGROUND: Proteasome inhibitors (PIs), including carfilzomib, potentiate the activity of selinexor, a novel, first-in-class, oral selective inhibitor of nuclear export (SINE) compound, in preclinical models of multiple myeloma (MM). METHODS: The safety, efficacy, maximum-tolerated dose (MTD) and recommended phase 2 dose (RP2D) of selinexor (80 or 100 mg) + carfilzomib (56 or 70 mg/m2) + dexamethasone (40 mg) (XKd) once weekly (QW) was evaluated in patients with relapsed refractory MM (RRMM) not refractory to carfilzomib. RESULTS: Thirty-two patients, median prior therapies 4 (range, 1-8),

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… Six (18.8%) patients received transfusions (either red blood cell or platelet transfusions). Six (18.8%) patients received the thrombopoietin receptor agonists eltrombopag or romiplostim, one (3.1%) patient received filgrastim and two patients (6.3%) received an erythropoietin-stimulating agent. …

Cost analysis of childhood hematopoietic stem cell transplantation in Sichuan, China.

ObjectiveTo analyze the inpatient cost of hematopoietic stem cell transplantation (HSCT) in children, so as to support clinical decision-making.MethodsStudy population were children who received HSCT in a tertiary children's hospital (Sichuan, China) between 1st January 2020 and 31st December 2021. The median and interquartile range (IQR) of total cost at 100 days post transplantation were calculated. Subgroup analyses were conducted based on age, gender, transplantation types, and post-transplant complications. The cost differences between subgroups were analyzed to determine whether it had a

Management of Primary Plasma Cell Leukemia Remains Challenging Even in the Era of Novel Agents.

Primary plasma cell leukemia (PCL) is a rare and aggressive variant of multiple myeloma (MM). PCL is characterized by peripheral blood involvement by malignant plasma cells and an aggressive clinical course leading to poor survival. There is considerable overlap between MM and PCL with respect to clinical, immunophenotypic, and cytogenetic features, but circulating plasma cell count exceeding 20% of peripheral blood leukocytes or an absolute plasma cell count of >2000/mm3 distinguishes it from MM. After initial stabilization and diagnosis confirmation, treatment of PCL in a fit patient typical

Ixazomib, pomalidomide and dexamethasone in triple-class exposed multiple myeloma patients - a phase II study.

Multi-class upfront regimens for multiple myeloma (MM) have improved outcomes, yet many patients become either triple-class exposed (TCE) or refractory (TCR) early in their disease course. This is a phase II, investigator initiated, open-label, single-arm, prospective, multicenter study evaluating all-oral Ixazomib-pomalidomide- dexamethasone (IPd) regimen for TCE RRMM patients. Sixty-one patients were enrolled between March 1st 2021 and March 17th 2024. The median age was 74 (range: 53-89) years; 25 (41%) were ≥75 years old. Fiftyseven percent had high-risk cytogenetics (t(4;14), t(14;16), +1

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… Platelet transfusions to help patients meet eligibility criteria are not allowed within 3 days of enrollment. • Total bilirubin ≤1.5 the upper limit of the normal range (ULN). • Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) ≤3 ULN. …

Impact of Anti-T-lymphocyte globulin dosing on GVHD and Immune reconstitution in matched unrelated myeloablative peripheral blood stem cell transplantation.

Data on the influence of different Anti-lymphocyte globulin (ATLG) doses on graft versus host disease (GVHD) incidence and immune reconstitution in matched unrelated (MUD) allogeneic Stem cell transplantation (allo-SCT) is limited. This retrospective study conducted at the University Medical-Center Hamburg compares GVHD and Immune reconstitution after myeloablative MUD (HLA 10/10) PBSC allogeneic stem cell transplant between 30 mg/Kg (n = 73) and 60 mg/Kg (n = 216) ATLG. Detailed phenotypes of T, B natural killer (NK), natural killer T (NKT) cells were analyzed by multicolor flow at day 30, 10

Прогрессивная многофокальная лейкоэнцефалопатия в химическом антигенном рецепторе T-Cell терапии: тематическое исследование.

Цимерическая антигенная рецепторная (CAR) T-клетотерапия — это новая иммунотерапия, которая продемонстрировала замечательные показатели реакции на рефрактные гематологические заболевания, включая множественные миеломы (ММ). Синдром цитокина (CRS) и нейротоксичность являются хорошо описываемыми побочными эффектами этой терапии. В результате иммунной дисфункции, многообразной терапии, лимфоразрушающей химиотерапии до инъекции клеток, а также продолжительной аплазии клеток B, бенефициары CAR-клеточной терапии также подвергаются повышенному риску инфицирования. Прогрессивная многофукальная лейкоэн

The potential impact of exercise upon symptom burden in adolescents and young adults undergoing cancer treatment

Abstract Purpose Adolescents and young adults (AYAs) experience vast symptom burden resulting from cancer treatment-related toxicities (TRTs). Evidence supports integrated exercise to mitigate several TRTs in other cohorts; however, evidence in AYAs is lacking. Conventional reporting of TRTs adopts a maximum grade approach failing to recognise the trajectory over time, of persistent, or lower grade toxicities. Alternatively, longitudinal analysis of toxicities over time (ToxT) may provide clinically meaningful summaries of this data. We evaluated the longitudinal impact of an exercise interven

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… Two participants (1:EG; 1:CG) required platelet transfusions and one (EG) required a whole blood transfusion, each of which may have otherwise been missed without patients adhering to this study protocol. …

VEXAS Syndrome in a Patient with Myeloproliferative Neoplasia.

VEXAS syndrome stands for vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome. The syndrome is a combined hematological and rheumatological condition caused by a somatic mutation in the UBA1. There is an association between VEXAS and hematological conditions such as myelodysplastic syndrome (MDS), monoclonal gammopathies of uncertain conditions (MGUS), multiple myeloma (MM), and monoclonal B-cell lymphoproliferative conditions. There are not many descriptions of patients having VEXAS in combination with myeloproliferative neoplasm (MPN). With this article, we want to present a ca

Duodenal plasmablastic lymphoma in an human immunodeficency virus-negative patient: a case report.

BackgroundPlasmablastic lymphoma is a rare type of non-Hodgkin lymphoma that generally presents an aggressive clinical course. It is strongly associated with human immunodeficency virus (HIV) infection, and the most common site of involvement is the oral cavity. Although extraoral PBL has been reported in several places, small intestine involvement is extremely rare.Case presentationHere, we describe an exceptionally rare case of a 24-year-old immunocompetent Asian Male patient with newly diagnosed plasmablastic lymphoma of the duodenum. The patient was admitted to our oncology facility due to

Autoimmune Cytopenias Post Hematopoietic Stem Cell Transplantation in Pediatric Patients With Osteopetrosis and Other Nonmalignant Diseases.

Autoimmune cytopenia (AIC) is a rare complication post hematopoietic stem cell transplantation (HSCT), with a higher incidence in nonmalignant diseases. The etiology of post-HSCT AIC is poorly understood, and in many cases, the cytopenia is prolonged and refractory to treatment. Diagnosis of post-HSCT AIC may be challenging, and there is no consensus for a standard of care. In this retrospective study, we summarize our experience over the past five years with post-HSCT AIC in pediatric patients with osteopetrosis and other nonmalignant diseases. All pediatric patients who underwent HSCT for no

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… Seven patients (54%) completely recovered, three patients (23%) recovered partially and still have mild-moderate thrombocytopenia not requiring platelet transfusions, and three patients (23%) died. …

Hematopoietic Tumors

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Emerging disease-modifying therapies for sickle cell disease.

Sickle cell disease afflicts millions of people worldwide and approximately 100,000 Americans. Complications are myriad and arise as a result of complex pathological pathways 'downstream' to a point mutation in DNA, and include red blood cell membrane damage, inflammation, chronic hemolytic anemia with episodic vaso-occlusion, ischemia and pain, and ultimately risk of cumulative organ damage with reduced lifespan of affected individuals. The National Heart, Lung, and Blood Institute's 2014 evidence-based guideline for sickle cell disease management states that additional research is needed bef

The Power of Extracellular Vesicles in Myeloproliferative Neoplasms: "Crafting" a Microenvironment That Matters.

Myeloproliferative Neoplasms (MPN) are acquired clonal disorders of the hematopoietic stem cells and include Essential Thrombocythemia, Polycythemia Vera and Myelofibrosis. MPN are characterized by mutations in three driver genes (JAK2, CALR and MPL) and by a state of chronic inflammation. Notably, MPN patients experience increased risk of thrombosis, disease progression, second neoplasia and evolution to acute leukemia. Extracellular vesicles (EVs) are a heterogeneous population of microparticles with a role in cell-cell communication. The EV-mediated cross-talk occurs via the trafficking of

Полнотрансформатная ретиноидальная кислота в гематологическое расстройство: не только острая промиелоцитическая лейкемия.

Всетранс-ретиноиновая кислота (ATRA) играет определенную роль в развитии тканей, нейронной функции, воспроизводстве, зрение, росте и дифференциации клеток, иммунитете от опухоли и апоптозе. АТРА может действовать, вызывая автофагическую сигнализацию, ангиогенез, клеточную дифференциацию, апоптоз и иммунную функцию. В крови ATRA впервые с большим успехом использовалась в острой промиелоцитической лейкемии (APL), где ATRA дифференцировала клетки лейкемии на зрелые гранулоциты. АТРА может играть определенную роль не только в ППН, но и в других гематоологических заболеваниях, таких, как иммунная т

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… VPA + ATRA treatment of low-risk MDS (LR-MDS) patients resulted in significant freedom from platelet transfusions lasting several months in approximately 30% of patients, reducing the burden of palliative care and improving quality of life ( Pilatrino et al., 2005 ). …

Management of Adverse Reactions for BCMA-Directed Therapy in Relapsed Multiple Myeloma: A Focused Review.

Anti-B-cell maturation antigen therapies consisting of bispecific antibodies, antibody-drug conjugates, and chimeric antigen receptor T cells have shown promising results in relapsed refractory multiple myeloma (RRMM). However, the severe side effects include cytokine release syndrome, immune effector cell-associated neurotoxicity syndrome, cytopenia(s), infections, hemophagocytic lymphohistiocytosis, and organ toxicity, which could sometimes be life-threatening. This review focuses on these most common complications post-BCMA therapy. We discussed the risk factors, pathogenesis, clinical feat

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… For the symptomatic or severe cases of cytopenia(s), supportive management with blood product transfusion (packed red blood cell transfusion for Hgb < 7 and platelet transfusion if count < 10 K) is the mainstay. …

Castleman Disease and Kaposi Sarcoma: A Review of the Literature and a Case Series.

Castleman disease and Kaposi sarcoma (KS) are both associated with infection by human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV). This virus plays a critical role in the pathogenesis of both conditions, particularly in immunocompromised individuals, such as those with HIV/AIDS. Multicentric Castleman disease (MCD) generally presents with systemic inflammatory symptoms, lymphadenopathy, and organ dysfunction, while Kaposi sarcoma typically appears as vascular tumors on the skin, with occasional involvement of mucous membranes or internal organs. We prese

Anakoinosis: Communicative Reprogramming of Tumor Systems - for Rescuing from Chemorefractory Neoplasia.

Disruptive technologies, such as communicative reprogramming (anakoinosis) with cellular therapies in situ for treating refractory metastatic cancer allow patient care to accelerate along a totally new trajectory and highlight what may well become the next sea change in the care of patients with many types of advanced neoplasia. Cellular therapy in situ consisted of repurposed drugs, pioglitazone plus all-trans retinoic acid or dexamethasone or interferon-alpha (dual transcriptional modulation) combined with metronomic low-dose chemotherapy or low-dose 5-azacytidine, plus/minus classic targete

Economic Benefits of Switching From Intravenous to Subcutaneous Epoetin Alfa for the Management of Anemia in Hemodialysis Patients.

BackgroundErythropoiesis-stimulating agents including epoetin alfa have been a mainstay of anemia management in patients with chronic kidney disease. Although the standard practice has been to administer epoetin alfa to patients on hemodialysis (HD) intravenously (IV), subcutaneous (SQ) epoetin alfa is longer acting and achieve the same target hemoglobin level to be maintained at a reduced dose and cost.ObjectiveThe primary objective of this study was to determine the economic benefits of change in route of epoetin alfa administration from IV to SQ in HD patients. The secondary objectives were

Возвращенная Множественная миелома в гастроинском кишечном тракте с аберрантным выражением CD3: доклад по делу.

В этом докладе описывается редкий случай рецидива множественной миэломы в желудочно-кишечном тракте с аберрантным выражением CD3. При наличии острой почечной недостаточности у пациента были аномальные вычисления томографического сканирования брюшной полости и таза. После этого колоноскопия обнаружила множество полипов и масс. Гистологический анализ биопсии one Из масс обнаружил диффузный атипичный клеточный инфильтрационный процесс с недифференцированной морфологией в субмукозе, который был положительным для CD3, CD4, слабого и переменного CD79a, CD138, BCL2, CMYC, MUM1, виментина и моноклонно