"Bone marrow-liver-spleen-type diffuse large B-cell lymphoma" presenting with cold autoimmune hemolytic anemia: a case report.

Introduction: Primary bone marrow diffuse large B-cell lymphoma is a rare clinical entity, and the "bone marrow-liver-spleen" type of diffuse large B-cell lymphoma is rarer, with only a few published cases in literature. Though bone marrow-liver-spleen-type diffuse large B-cell lymphoma has unique presentations such as fever, cytopenias, and hemophagocytic lymphohistiocytosis, no cases with cold autoimmune hemolytic anemia have been reported.

Case Presentation: A 39-year-old Sri Lankan woman, previously healthy, presented with shortness of breath, productive cough, and fever for 4 days.

Modified G-CSF/ATG-Based Haploidentical Transplantation Protocol in Pediatric Primary Hemophagocytic Lymphohistiocytosis: A Long-Term Follow-Up Single-Center Experience.

Background: Primary hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by immune dysregulation. Hematopoietic stem cell transplantation (HSCT) represents the only option for long-term cure for primary HLH. However, only around 25% of patients have a fully HLA-matched donor.

A predictive model for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

Background: Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a severe hyperinflammatory disorder induced by overactivation of macrophages and T cells. This study aims to identify the risk factors for the progression from infectious mononucleosis (EBV-IM) to EBV-HLH, by analyzing the laboratory parameters of patients with EBV-IM and EBV-HLH and constructing a clinical prediction model. The outcome of this study carries important clinical value for early diagnosis and treatment of EBV-HLH.

Tocilizumab and immune signatures for targeted management of cytokine release syndrome in immune checkpoint therapy.

Background: This study aimed to identify specific biomarkers in oncology patients experiencing immune-related cytokine release syndrome (irCRS)-like symptoms during immune checkpoint inhibitor (ICI) therapy, including severe cases like hemophagocytic lymphohistiocytosis (irHLH), and to distinguish these from sepsis. A secondary objective was to retrospectively analyze the efficacy of tocilizumab (TCZ) in treating corticosteroid (CS)-refractory high-grade irCRS.

Patients And Methods: A cohort of 35 patients presenting with irCRS-like symptoms was studied, including 9 with irHLH-like manifestations and 8 with sepsis.

A case of adult-onset X-linked lymphoproliferative disease mimicking pulmonary infection.

X-linked lymphoproliferative disease (XLP) is a rare primary immunodeficiency with susceptibility and vulnerability to Epstein-Barr virus (EBV) infection. Most patients were diagnosed in early childhood and do not survive into adulthood. Here we reported an adult-onset XLP patient presenting with fever, dyspnea, and pulmonary nodules, mimicking respiratory infection at disease onset.

Hemophagocytic lymphohistiocytosis in HIV-associated HHV8-positive multicentric castleman disease.

Objective: The clinical and laboratory characteristics of HHV8-associated Multicentric Castleman Disease (MCD) in people living with HIV (PLWH) overlap with those of Hemophagocytic Lymphohistiocytosis (HLH) disease and indeed the two diagnoses may co-exist. A risk-stratified treatment approach to MCD based on Rituximab immunotherapy for mild cases and chemo-immunotherapy for severe cases has been shown to yield excellent outcomes in PLWH. In contrast, HLH disease, previously known as secondary HLH, has a dismal prognosis even when promptly treated according to guidelines.

Retraction of: Lethal Disseminated Mucorales Infection With Positive Blood Cultures With Purpura Fulminans Complicating Hemophagocytic Lymphohistiocytosis After Chimeric Antigen Receptor T-Cell Therapy.

[This retracts the article DOI: 10.1093/ofid/ofae647.].

Ticked Off: A Case of Disappearing Cell Lines.

Human granulocytic anaplasmosis (HGA) is transmitted by the black-legged tick and presents with fever, thrombocytopenia, leukocytopenia, and elevated transaminases. If left untreated, HGA can progress to hemophagocytic lymphohistiocytosis (HLH), which can be fatal. Here, we discuss a case of a woman diagnosed with anaplasmosis who was treated promptly.

A Case of the Initial Presentation of Hemophagocytic Lymphohistiocytosis as Acute Unilateral Vision Loss.

Hemophagocytic lymphohistiocytosis (HLH) is an incredibly rare disease that is characterized by the overproduction of histiocytes and lymphocytes, which then start to attack the body instead of just invasive pathogens. It has been known to affect many organs including most commonly blood cells and bone marrow, but it has also been known to affect the liver, spleen, skin, and the brain. Ocular involvement is rare and is mostly associated with HLH's effect on other body systems (i.

Hemophagocytic Lymphohistiocytosis Secondary to Miliary Tuberculosis in a Resource-Limited Setting: A Case Report.

Hemophagocytic lymphohistiocytosis is a rare life-threatening condition, with a high mortality rate, characterized by a dysfunctional immune response resulting in multi-organ damage. The secondary or sporadic form of the disease can be triggered by a multitude of infections, malignancies, and autoimmune disorders. Tuberculosis is commonly involved as a trigger for hemophagocytic lymphohistiocytosis.

Case report: A cyclic neutropenia patient with mutation accompanied by hemophagocytic lymphohistiocytosis.

Many inborn errors of immunity may accompany secondary hemophagocytic lymphohistiocytosis (HLH), a condition typically characterized by impaired cytotoxic T and NK cell function. A considerable proportion of HLH cases also stem from chronic granulomatosis with phagocytic dysfunction. However, the development of secondary HLH in patients with severe congenital neutropenia (SCN) or cyclic neutropenia (CyN) with abnormal phagocytic cell counts has been less frequently reported.

Malignancy-associated HLH: mechanisms, diagnosis, and treatment of a severe hyperinflammatory syndrome.

Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome characterized by uncontrolled immune activation. While traditionally associated with genetic mutations affecting cytotoxic function, recent advances have highlighted the prevalence and significance of HLH in adults, particularly in hematologic malignancies. This review focuses on malignancy-associated HLH (M-HLH), a complex and challenging condition with a poor prognosis.

Fatal visceral disseminated varicella zoster virus infection during initial remission induction therapy in a patient with lupus nephritis: a case report and review of the literature.

Visceral disseminated varicella zoster virus (VZV) infection is a severe complication, characterized by a notably high mortality rate. Herein, we present a case of a 36-year-old-man involving visceral disseminated VZV infection that emerged during remission induction therapy involving high-dose prednisolone (PSL), mycophenolate mofetil (MMF), and hydroxychloroquine for lupus nephritis. Two months after starting the immunosuppressive therapy, he experienced a rapid onset of severe upper abdominal pain.

A rare case of complicated typhoid fever presenting with secondary Hemophagocytic Lymphohistiocytosis.

Enteric fever is one of the important causes of tropical fever with a prevalence of 11-21 million cases worldwide annually. It encompasses both typhoid and paratyphoid fever. Typhi is the causative organism for typhoid fever, manifesting as an uncomplicated febrile illness to life-threatening sepsis with multiorgan dysfunction.

Enforced CARD11/MALT1 signaling in dendritic cells triggers hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome fueled by uncontrolled mononuclear phagocyte activity, yet the innate immune mechanisms driving HLH pathogenesis remain elusive. Germline gain-of-function (GOF) mutations in CARD11, a pivotal regulator of lymphocyte antigen receptor signaling, cause the lymphoproliferative disease B-cell expansion with NF-κB and T-cell anergy, which is frequently associated with HLH development. Given that CARD11 is physiologically expressed not only in lymphocytes but also in dendritic cells (DCs), we explored whether enforced CARD11 signaling in DCs contributes to immunopathology.

Intravascular Large B-cell Lymphoma Associated With Hemophagocytosis as a Cause of Fever of Unknown Origin: A Review of Literature.

A 53-year-old woman was hospitalized due to a fever of unknown origin for three weeks. Given the presence of fever and fatigue, the laboratory findings, and a bone marrow biopsy confirming hemophagocytic lymphohistiocytosis (HLH), a hematological malignancy was suspected. Peripheral lymphocytic typing, bone marrow biopsy, and imaging could not identify an underlying cause of HLH.

Haemophagocytic Lymphohistiocytosis Triggered by Cytomegalovirus Reactivation in An Immunosuppressed Patient with Pauci-Immune Glomerulonephritis.

Unlabelled: We report on a 67-year-old male patient admitted to the Internal Medicine department for fever, joint pain and exertional dyspnoea. Two months before his admission, the patient had been diagnosed with pauci-immune necrotising glomerulonephritis, for which he had been treated with rituximab and corticosteroids. Upon admission the patient was stable, but within a few hours he became unstable as liver failure and acute cytopaenia occurred.

The Value of a Pet Scan in Selecting the Best Lymph Node to Biopsy, and Confirming the Diagnosis of Idiopathic Multicentric Castleman Disease with HLH And EBV Viremia in a Previously Healthy Adult.

Introduction: Castleman disease (CD) is a rare lymphoproliferative disorder having a variegated clinical presentation. Diagnosis of the idiopathic HIV- and HHV8-negative multicentric CD (iMCD) subtype poses a challenge given its non-specific clinical manifestations. iMCD presents as diffuse lymphadenopathy with inflammatory manifestations, primarily driven by interleukin-6 (IL-6).

Secondary haemophagocytic lymphohistiocytosis associated with scrub typhus: A case report with a brief review of literature.

Scrub typhus is a very important cause of acute undifferentiated febrile illness in the Asia-Pacific region. Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition characterised by widespread immune system activation, resulting in tissue damage throughout multiple organ systems. HLH is categorised into primary HLH, arising from intrinsic defects in lymphocytes and macrophages, and secondary HLH, primarily triggered by infections or connective tissue disorders.

Anti-PD-1 antibody (Tislelizumab) combined with gemcitabine and oxaliplatin for extranodal NK/T-cell lymphoma failing asparaginase: A multicenter phase II trial.

Background: Extranodal natural killer/T-cell lymphoma (ENKTCL) is almost always fatal after the failure of asparaginase. This phase II study aimed to investigate the efficacy and safety of tislelizumab combined with gemcitabine and oxaliplatin (Tisle-GemOx) in patients with ENKTCL failing asparaginase.

Methods: Eligible patients received Tisle-GemOx as initial induction for 6-8 cycles at 21-day intervals.

An Interesting Case of Hemophagocytic Lymphohistiocytosis in a Postpartum Female With Recent COVID-19 Vaccination.

Hemophagocytic lymphohistiocytosis is a life-threatening cryptogenic inflammatory process. In some cases, the etiology is obscure, while in others multiple potential etiologies may be present. Diagnosis relies on observed findings rather than context.