Zoonotic Bacterial Infections Triggering Cytokine Storm Syndrome.

Zoonotic infections can result in life-threatening complications that can manifest with hemophagocytic lymphohistiocytosis (HLH)/cytokine storm syndrome (CSS). Bacteria constitute the largest group of zoonotic infection-related HLH cases. The growing list of zoonotic bacterial infections associated with HLH/CSS include Brucella spp.

Clinical and laboratory characteristics of patients with cold agglutinin disease: A retrospective analysis at a tertiary medical center.

Background: Cold agglutinin disease (CAD) is relatively rare and has primarily been reported as retrospective case series.

Aim: We reviewed our experience with CAD to shed light on this disease.

Study Settings And Design: This was a retrospective review of all patients with CAD managed at our institution between 2007 and 2018.

Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease.

Sickle cell disease is one of the most common inherited hemoglobinopathies diagnosed in the United States. Patients often present with severe anemia, pain crises, infections, and vaso-occlusive phenomena. Complications of these disorders can lead to significant debilitating morbidity and mortality.

Extracorporeal photopheresis: A case of graft-versus-host-disease and hemophagocytic lymphohistiocytosis following liver transplantation.

Background: Graft-versus-host-disease (GVHD) is one of the rare complications following liver transplantation. We report on the efficacy and safety of extracorporeal photopheresis (ECP) in managing GVHD and hemophagocytic lymphohistiocytosis (HLH) after liver transplantation.

Case Report: The patient is a 63-year-old male with hepatitis B cirrhosis who underwent liver transplantation.

Hemophagocytosis on ascitic fluid cytology: Diagnosis of HLH.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of pathologic immune response characterized by excessive activation of macrophages. Hemophagocytosis is one of the diagnostic criteria for HLH, and it usually involves the bone marrow, spleen, lymph nodes, or any part of the reticuloendothelial system. Hemophagocytosis in the ascitic fluid has rarely been reported in HLH.

Lack of alloimmunization to the D antigen in D-negative orthotopic liver transplant recipients receiving D-positive red blood cells perioperatively.

Background And Objectives: D-negative patients undergoing orthotopic liver transplantation (OLT) might require a large number of red blood cell (RBC) units, which can impact the inventory of D-negative blood. The blood bank might need to supply these patients with D-positive RBCs because of inventory constraints. This study evaluates the prevalence of anti-D formation in D-negative OLT patients who received D-positive RBCs perioperatively, as this will assist in successful patient blood management.

Platelet Transfusion: An Update on Indications and Guidelines.

Platelets are commonly transfused either therapeutically or prophylactically to maintain hemostasis. Most platelet transfusions are used to manage patients with hematologic malignancies. Although platelet transfusion guidelines have been published, platelet transfusion practices are still heterogeneous.

Thrombocytapheresis for acquired von Willebrand syndrome in a patient with essential thrombocythemia and recent multivisceral transplantation.

Background: Essential thrombocythemia (ET) is associated with increased risk of bleeding secondary to acquired von Willebrand syndrome (AVWS). Bleeding in ET requires urgent platelet reduction by cytoreductive therapy such as hydroxyurea or thrombocytapheresis. We report on the efficacy and safety of thrombocytapheresis in managing AVWS in a patient with ET and multivisceral transplantation.

Transfusion requirements and alloimmunization to red blood cell antigens in orthotopic liver transplantation.

Background And Objectives: Orthotopic liver transplantation (OLT) has been associated with high blood transfusion requirements. We evaluated the transfusion needs and frequency of alloimmunization to RBC antigens among OLT recipients pre- and post-transplantation.

Materials And Methods: We reviewed the medical records of patients who underwent a first OLT between January 2007 and June 2017.

P-selectin expression assay in a repeatedly serotonin-release assay-negative patient with heparin-induced thrombocytopenia.

Heparin-induced thrombocytopenia (HIT) is an immune complication of heparin therapy caused by antibodies to complexes of platelet factor 4 (PF4) and heparin. Pathogenic antibodies to PF4/heparin bind and activate platelets to propagate a hypercoagulable state culminating in life-threatening thrombosis. The serotonin-release assay (SRA) is considered the gold-standard test to diagnose HIT.

Increasing ferritin predicts early death in adult hemophagocytic lymphohistiocytosis.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of pathologic immune activation. Most studies on adult HLH have evaluated prognostic factors for overall survival; factors predicting early mortality have not been sufficiently investigated.

Methods: This was a collaborative study between Henry Ford Hospital and Barnes-Jewish Hospital.

Acute Splenic Sequestration Crisis in Hemoglobin SC Disease: Efficiency of Red Cell Exchange.

Acute splenic sequestration crisis (ASSC) is recognized as a serious complication of sickle cell disease in children. ASSC presents with progressive splenic enlargement, transfusion-dependent anemia, and, eventually, circulatory compromise. ASSC is rare in adult patients, thus making its management and outcome in adults not well-defined.

Pharmacologic Therapies to Prevent Relapse of Acute Myeloid Leukemia After Allogeneic Hematopoietic Stem Cell Transplantation.

Relapse is the main cause of mortality in patients with acute myeloid leukemia (AML) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Adverse cytogenetic or molecular risk factors, as well as refractory disease or persistent measurable residual disease (MRD) at the time of transplantation are associated with an increased risk of recurrence. Salvage therapy for AML relapse after allo-HSCT is often limited to chemotherapy, donor lymphocyte infusions and/or second transplants and is rarely successful.

Paradoxical embolic strokes in a liver transplant recipient with atrial septal defect undergoing therapeutic plasma exchange.

Therapeutic plasma exchange (TPE) is a technique used to separate blood components into layers based on their density difference, thus removing plasma and exchanging it with replacement fluids. A variety of adverse reactions has been described during TPE. Thrombotic events, especially strokes, are extremely rare complications of TPE.

FLT3 inhibitors in acute myeloid leukemia: ten frequently asked questions.

The FMS-like tyrosine kinase 3 (FLT3) gene is mutated in approximately one third of patients with acute myeloid leukemia (AML), either by internal tandem duplications (FLT3-ITD), or by a point mutation mainly involving the tyrosine kinase domain (FLT3-TKD). Patients with FLT3-ITD have a high risk of relapse and low cure rates. Several FLT3 tyrosine kinase inhibitors have been developed in the last few years with variable kinase inhibitory properties, pharmacokinetics, and toxicity profiles.

Human ehrlichiosis at a tertiary-care academic medical center: Clinical associations and outcomes of transplant patients and patients with hemophagocytic lymphohistiocytosis.

Background: Ehrlichiosis is an acute febrile tick-borne disease which can rarely be a trigger for secondary hemophagocytic lymphohistiocytosis (HLH).

Methods: We reviewed our experience with Ehrlichia infections at a tertiary-care academic medical center.

Results: Over 10 years, 157 cases of ehrlichiosis were identified.

Transfusion requirements and 30-day mortality predictors for adult hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by an uncontrolled hyper-inflammatory response. We assessed the transfusion requirements and predictors of 30-day mortality for adult HLH patients. We identified all adult patients with a diagnosis of HLH at a large academic hospital from October 2003 through February 2017.

Vascular access for red blood cell exchange.

Red blood cell exchange is the process of removing red blood cells from a patient and replacing them with donated blood using either automated or manual techniques. Red blood cell exchange is a well-recognized and effective therapy for many red blood cell-related diseases, especially sickle cell disease. However, decisions regarding the best methods for vascular access are not intuitive and must account for the patient's clinical condition, complication risks, and lifestyle, especially in the context of long-term vascular access.

Elevated serum ferritin is not specific for hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, syndrome of excessive and ineffective activation of the immune system. The majority of the reported data on HLH is from pediatric patients and lacks specificity. This makes HLH diagnosis challenging especially in adults where HLH is triggered by many conditions and can resemble many disease entities.

Diagnostic Challenges of Hemophagocytic Lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by excessive activation of the immune system, resulting in overproduction of inflammatory cytokines. Patients usually present with high fever, cytopenias, hyperferritinemia, and hepatosplenomegaly, and their disease process ranges from mild to fatal multiorgan failure. HLH is a heterogeneous group of disorders that can be triggered by infections, neoplasms, or autoimmune diseases.

A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults.

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations, and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition.

Adverse events of cryopreserved hematopoietic stem cell infusions in adults: a single-center observational study.

Background: Autologous hematopoietic stem cell (HSC) transplantation has been used for almost three decades for the management of malignant hematologic diseases and some solid tumors. Dimethyl sulfoxide (DMSO) is used as a cryoprotective agent for hematopoietic progenitor cells (HPCs) collected by apheresis (HPC-A). We evaluated the factors contributing to the occurrence of adverse events (AEs) of cryopreserved HPC-A infusion.

Thymoma with Concomitant Pure Red Cell Aplasia, Good's Syndrome and Myasthenia Gravis Responding to Rituximab.

Thymomas are often associated with a variety of autoimmune diseases, mostly myasthenia gravis. The association of thymomas with both pure red cell aplasia (PRCA) and Good's syndrome is exceedingly rare. To the best of our knowledge, the combination of a thymoma with manifestations of myasthenia gravis, PRCA, and Good's syndrome, as in our case herein, has not been described before in the medical literature.