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Hematology and Immune-Related Disorders

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Hematology

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… Note that platelet transfusions are not effective in ITP and may worsen TTP/HUS and HITT TREAT UNDERLYING CAUSE — discontinue medications that may cause thrombocytopenia (platelets may return to normal in 7–14 days). … Avoid platelet transfusion, ASA and antimotility agents. …

Case report: Daratumumab treatment in pre-transplant alloimmunization and severe hemolytic anemia.

Daratumumab, a CD38 monoclonal antibody that has been FDA-approved to treat multiple myeloma, has acquired popularity and is used off-label for both auto- and alloantibody mediated disorders, particularly in refractory/resistant circumstances. Much of the published data for its use in pediatric blood disorders has been in post-transplant autoimmune cytopenias. Here we describe three patients in whom daratumumab was used outside of post-transplant autoimmune cytopenias, highlighting further potential uses of this medication.

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… She demonstrated severe platelet refractoriness up-front requiring frequent platelet transfusions. Work-up for platelet refractoriness demonstrated low corrected count increment (CCI) indicating immune-mediated platelet destruction. … After 16 weeks of IST, our patient showed improved hemoglobin and absolute neutrophil count but continued needing platelet transfusions on a weekly basis. …

Safety and Feasibility of Transcatheter Aortic Valve Replacement in Patients With Hematologic Neoplasms.

For patients with severe, symptomatic aortic stenosis (AS) who are poor surgical candidates, transcatheter aortic valve replacement (TAVR) offers a less invasive alternative to surgical valve replacement. Patients with hematologic neoplasms and severe AS are a high-risk subset, with increased risk of bleeding and infection, who have historically been excluded from clinical investigation in TAVR. Prior case reports have demonstrated successful TAVR in patients with hematologic neoplasms; however, there remains minimal literature on TAVR in this population. We present a series of 10 patients wit

Remote vital signs data monitoring during home blood transfusion: A pilot study.

BACKGROUND AND AIMS: Our study aimed to establish safer methods to manage home blood transfusion by using a remote vital signs data monitoring system. Home care is administered for patients with various medical disorders; however, home blood transfusion remains challenging owing to the risk of transfusion-related complications. METHODS: We set up a remote vital signs data monitoring system to improve the safety of home blood transfusions. Using an Internet-based vital signs data monitoring system, the heart rate, electrocardiography, respiration rate, and percutaneous oxygen saturation (SpO2)

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… In a report of anaphylactic shock caused by platelet transfusion, Mushino et al 13 noted that the blood pressure decreased simultaneously with a decrease in SpO 2 . Thus, multiple changes in vital signs reflect transfusion‐related complications. … A case of anaphylactic shock caused by platelet transfusion: washed platelet are useful for preventing anaphylaxis. Jpn J Transfus Cell Ther. 2018;64:529–533. …

The Role of Platelet Dysfunctions in the Pathogenesis of the Hemostatic-Coagulant System Imbalances.

Platelet dysfunction plays a critical role in the pathogenesis of various disorders affecting the hemostatic-coagulant system. This review aims to explore the mechanisms by which platelet dysfunctions contribute to the disruption of hemostasis, leading to an increased risk of both thrombosis and bleeding. Platelets, traditionally known for their role in clot formation, can exhibit altered functionality under pathological conditions such as cardiovascular diseases, metabolic disorders, and autoimmune diseases, impacting their interaction with coagulation factors and vascular endothelium. The re

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… Moreover, the optimal use of platelet transfusions has been questioned following the PATCH trial, which compared platelet transfusion to standard care in intracranial hemorrhage. … For patients without contraindications, platelet transfusion remains the primary treatment option for Glanzmann’s thrombasthenia. …

Blood transfusion in haematology: A qualitative exploration of patients' and healthcare professionals' perceptions.

ObjectivesRepeated blood transfusions are indicated for the management of patients with cancer or blood disorders. Patients' perceptions about transfusions may be associated with decision-making and coping, which has been under-explored in the haematology context. This study therefore aimed to explore haematology transfusion patients' and HCPs' perceptions of blood transfusion, drawing on theory and previously identified themes of transfusion perceptions.DesignSemi-structured interview study with 14 adult blood transfusion patients and 14 HCPs (consultants, registrars, nurses) at two UK haemat

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… Burden Half of the patients interviewed reported that transfusions were a part of their routine life, that attendance was not a great burden and that the experience was consistent over time and easy (switching between red cell and platelet transfusions). …

Profile of pomalidomide and its potential in the treatment of myelofibrosis.

Myelofibrosis, a Philadelphia-negative myeloproliferative neoplasm, is in a new treatment era after the discovery of the JAK2V617F mutation in 2005. JAK inhibitors boast improvements in disease-related symptoms, splenomegaly, and overall survival; however, treatment of myelofibrosis remains a challenge, given the lack of improvement in cytopenias with these agents. Second-generation immunomodulatory agents, such as pomalidomide, have shown efficacy in myelofibrosis-associated anemia within multiple clinical trials. Five major pomalido-mide clinical trials have been completed to date, and demon

Next Generation Therapeutics for the Treatment of Myelofibrosis.

Myelofibrosis is a myeloproliferative neoplasm characterized by splenomegaly, constitutional symptoms, bone marrow fibrosis, and a propensity towards transformation to acute leukemia. JAK inhibitors are the only approved therapy for myelofibrosis and have been successful in reducing spleen and symptom burden. However, they do not significantly impact disease progression and many patients are ineligible due to coexisting cytopenias. Patients who are refractory to JAK inhibition also have a dismal survival. Therefore, non-JAK inhibitor-based therapies are being explored in pre-clinical and clini

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… There was also improvement in transfusion requirements with five of 31 patients (16%) achieving red blood cell transfusion independence and six of 13 patients (46%) achieving platelet transfusion independence. …

New-Onset Severe Cytopenia After CAR-T Cell Therapy: Analysis of 76 Patients With Relapsed or Refractory Acute Lymphoblastic Leukemia.

Although chimeric antigen receptor T (CAR-T) cell therapy has proven to be effective in treating relapsed or refractory B-cell hematological malignancies, severe hematological toxicities remain an intractable issue. This retrospective study assessed the characteristics and risk factors of new-onset severe cytopenia following CAR-T cell infusion in 76 patients with r/r acute lymphoblastic leukemia. The rates of new-onset severe cytopenia were high, including severe neutropenia (SN) (39/56, 70%), severe anemia (SA) (35/66, 53%), and severe thrombocytopenia (ST) (31/64, 48%). Comparatively, cohor

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… Platelet recovery was defined as platelet counts > 20×10 9 /L for three consecutive days in the absence of platelet transfusion. Prolonged HT (PHT) was defined as the presence of SN, SA, or ST on day 28 post infusion. … Among 76 patients, 66 (86.84%) received G-CSF, 38 (50%) received PRBC transfusions, and 35 (46.05%) received platelet transfusions. …

Экстренные ситуации в гематологии: Почему, когда и как я лечусь?

Гематологические чрезвычайные ситуации являются критическими медицинскими условиями, требующими неотложного внимания в силу их быстрого развития и опасного для жизни характера. В качестве примера можно привести гиперкальцемию, которая часто ассоциируется с раком, таким как множественная миелома, что может привести к серьезной неврологической и сердечной дисфункции. Гиперлейкоцитоз, распространенный в острой миэлоидной лейкемии, повышает риск лейкостаза и многоорганического отказа. Кислородный кризис, обычное осложнение серповидноклеточной болезни, является результатом вазооккуляции, приводящей

Совпадение в полном тексте источника
… With active bleeding, the platelet transfusion threshold should be considered 50 × 10 9 /L, and fibrinogen replacement therapy is indicated to reach a level >1.5 g/L. …

How I treat cytopenias after CAR T-cell therapy.

Increasing use of chimeric antigen receptor T-cell therapy (CAR-T) has unveiled diverse toxicities warranting specific recognition and management. Cytopenias occurring after CAR-T infusion invariably manifest early (90 days). Variable etiologies of these cytopenias, some of which remain incompletely understood, create clinical conundrums and uncertainties about optimal management strategies. These cytopenias may cause additional sequelae, decreased quality of life, and increased resource use. Early cytopenias are typically attributed to lymphodepletion chemotherapy, however, infections and hyp

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… Over the course of the next 3 weeks, ANC remained under 1000/mm 3 and he required PRBC and platelet transfusions every 3 to 4 days to maintain a goal of hemoglobin >7 g/dL and platelets >10 × 10 3 /μL. … For early cytopenias, we use supportive treatment with PRBC and platelet transfusions when indicated by institutional thresholds. …

Dengue virus infection in hematopoietic stem cell transplant recipients: A case series and comparative literature review from dengue endemic region.

This case series describes the wide spectrum of clinical presentation, laboratory findings, and morbidity/mortality associated with dengue fever in hematopoietic stem cell transplant recipients, treated in a dengue endemic region. The risk of acquiring viral infections increases manifold after transplant due to the severely immunocompromised state amid conditioning toxicity and immunosuppressive therapy. The classical warning signs of dengue viremia are often masked in posttransplant patients, leading to a missed diagnosis of dengue and grave consequences observed in some of the patients. Accu

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… Nephrologist was taken on board and patient was managed conservatively with hydration, continuous bladder irrigation, and platelet transfusion support. … transfusions if indicated. …

Cell Therapy as a Way to Increase the Effectiveness of Hematopoietic Stem Cell Transplantation.

Hematopoietic stem cell transplantation (HSCT) is a standard method for treating a number of pathologies, primarily blood diseases. Timely restoration of the immune system after HSCT is a critical factor associated with the development of complications such as relapses or secondary tumors and various infections, as well as the graft-versus-host reaction in allogeneic transplantation, which ultimately affects the survival of patients. Introduction into the recipient's body of immune system cells that are incapable of sensitization by recipient antigens during the period of immune reconstitution

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… And in patients with allotransplantation of stem cells in the early post-transplantation period, platelet transfusion did not cause transplant rejection reactions [ 21 ]. We summarized the various data on the side effects of red blood cell and platelet transfusion after HSCT ( Table 1 ). … Impact of platelet transfusion on toxicity and mortality after hematopoietic progenitor cell transplantation Transfusion 2015 55 253 258 10.1111/trf.12817 25125085 24. Gjærde L.K. Sørensen A.L.T. von Stemann J.H. Fischer-Nielsen A. Hansen M.B. Sengeløv H. Ostrowski S.R. …

Lenalidomide: a brief review of its therapeutic potential in myelodysplastic syndromes.

Lenalidomide is a novel thalidomide analogue with enhanced immunomodulatory and antiangiogenic action lacking most of the typical thalidomide-associated adverse events. In myelodysplastic syndromes (MDS), it has been used primarily in the IPSS low- and intermediate-1 risk setting. Several trials have demonstrated its potential to lead to both erythroid and cytogenetic responses in these disease groups. In a clinical trial of patients with a del(5q) chromosomal abnormality, lenalidomide treatment resulted in red blood cell (RBC) transfusion independence in 67% of patients. Moreover, 45% of pati

Investigation of the efficacy and safety of eltrombopag to correct thrombocytopenia in moderate to severe dengue patients - a phase II randomized controlled clinical trial.

BACKGROUND: The dengue-infected patients with or without hemorrhagic manifestations, typically exhibit moderate to severe thrombocytopenia. A thrombopoietin receptor agonist - eltrombopag has been efficacious in correcting thrombocytopenia in patients with various pathological conditions including immune thrombocytopenia, chronic liver disease, and severe aplastic anemia. This study investigated the efficacy and safety of eltrombopag to correct dengue-mediated thrombocytopenia. METHODS: In this open-label, randomized controlled phase-II trial, patients with dengue fever (DF) and dengue hemorr

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… Platelet transfusion to correct dengue-induced thrombocytopenia remains controversial since it failed to have a positive impact on reducing the incidence of bleeding in patients exhibiting low platelet count consequently platelet transfusion is not routinely performed in the management of dengue. … In light of the notion that platelet transfusion may not be beneficial to dengue patients, several studies raised the argument that platelet transfusion should not be routinely performed in the management of dengue, and indeed WHO does not recommend platelet transfusion for dengue patients [14] . …

Plasma Cell Leukemia: A Review of 3 Cases Managed in Kenya.

Plasma Cell Leukemia (PCL) is a rare and aggressive form of plasma cell dyscrasia that can arise either de novo (primary plasma cell leukemia) or evolve from previously diagnosed and treated multiple myeloma (secondary PCL). We highlight three clinical cases with very different presentations as a reminder of this diagnosis. The cases also highlight the diversity and variability that cover a patient's journey that is highly dependent on accessibility based on financial capability and social support. The clinical presentation is more aggressive due to the higher tumour burden and more proliferat

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… He required transfusions whenever his haemoglobin dropped but did not need platelet transfusion. He slowly improved initially getting off inotropes, weaning off oxygen successfully to the point of discharge after 30 days in the hospital. … Bortezomib was held after a few treatments due to low platelet counts and challenges of platelet transfusions; she, therefore, continued with low-dose lenalidomide and dexamethasone. …

Differences in Perspectives and Policies Regarding End-of-Life Care Between Hematologists and Gastroenterologists.

BackgroundPatients with hematologic malignancies often receive aggressive treatment until the terminal phase and transition to end-of-life (EOL) care less frequently than those with solid tumors. A 2015 survey compared hematologists' and solid tumor-oncologists' attitudes toward EOL care, revealing differences. A decade later, no similar reports have appeared despite therapeutic advances. Therefore, we repeated the survey with additional questions to examine whether perspectives on EOL care have changed.MethodsA 50-item questionnaire was administered to hematologists and gastroenterologists. R

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… Platelet transfusions ranked highest among hematologists, who also reported platelet and red blood cell transfusions more frequently than gastroenterologists. … Platelet transfusions are distinctly expensive, and as anticipated, hematologists were more likely than gastroenterologists to indicate that the high cost of platelet transfusions and the inability to administer them in PCWs prevented patients from being transferred from acute care wards to PCWs. …

Low molecular weight heparin for the prevention of hepatic veno-occlusive disease (VOD) after hematopoietic stem cell transplantation: a prospective phase II study.

We evaluated 40 patients undergoing high-dose chemo/radiotherapy (HDCT) and hematopoietic stem cell transplantation (HSCT) (allogeneic (22), autologous (18)) to determine the safety and feasibility of administering low molecular weight heparin (LMWH) as hepatic veno-occlusive disease (VOD) prophylaxis. Patients received a once daily subcutaneous injection of dalteparin 2500 anti-Xa i.u. commencing the day prior to starting HDCT, and continuing until day +30 post HSCT or hospital discharge, whichever came first. Dosage adjustments were made for patients developing renal failure. All bleeding ep

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… Therefore, careful assessments of bleeding events, red cell and platelet transfusion requirements, time to white cell and platelet engraftment and adverse events were made. The secondary objective was to determine the incidence and severity of VOD for patients undergoing HSCT. … Platelet transfusion thresholds remained at 10 × 10 9 /l for all patients in the study, with the exception of the three patients, with clinically significant bleeding episodes. …

Immune effector cell-associated hematotoxicity: mechanisms, clinical manifestations, and management strategies.

Chimeric antigen receptor (CAR) T-cell therapy has transformed the treatment landscape for hematologic malignancies. However, it is frequently complicated by immune effector cell-associated hematotoxicity (ICAHT), a potentially life-threatening adverse event encompassing neutropenia, anemia, and thrombocytopenia. These cytopenias elevate the risk of severe infections, transfusion dependence, and prolonged hospital stays, contributing substantially to morbidity and non-relapse mortality. This review delineates the incidence, mechanisms, and risk factors for ICAHT, highlighting the complex inter

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… transfusions. … transfusion dependence at day +30 or beyond following CAR T-cell infusion, 76.3% (29 patients) achieved platelet transfusion independence after a median of 32 days of eltrombopag treatment. …