3,693 results match your criteria Lymphohistiocytosis


Haemophagocytic lymphohistiocytosis induced by A/H1N1 influenza.

Presse Med 2019 Apr 16. Epub 2019 Apr 16.

Habib Bourguiba University Hospital, Department of Intensive Care, Sfax, Tunisia.

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http://dx.doi.org/10.1016/j.lpm.2019.02.034DOI Listing

Hydroa vacciniforme: a distinctive form of Epstein-Barr virus-associated T-cell lymphoproliferative disorders.

Eur J Dermatol 2019 Feb;29(1):21-28

Department of Dermatology, Kawasaki Medical School,, Department of Dermatology, Kawasaki Medical School General Medical Center, Okayama, Japan.

Hydroa vacciniforme (HV) is a cutaneous subset of Epstein-Barr virus (EBV)-associated T/NK lymphoproliferative disorders (LPDs). Our previous case series study clearly showed a clinical spectrum of EBV-associated T/NK LPDs including HV, hypersensitivity to mosquito bites (HMB), chronic active EBV infection (CAEBV), and hemophagocytic lymphohistiocytosis (HLH). Patients with HV are divided into two groups: a benign subtype designated "classic HV" (cHV) and more serious systemic HV (sHV), also called "HV-like LPD" in the 2017 World Health Organization (WHO) classification. Read More

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http://dx.doi.org/10.1684/ejd.2018.3490DOI Listing
February 2019

Lamotrigine and hemophagocytic lymphohistiocytosis.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Department of Neurology and Neurological Sciences (K.J.M.), Stanford University School of Medicine, Palo Alto, CA; and the School of Pharmacy and Department of Neurology (B.E.G.), University of Wisconsin, Madison.

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http://dx.doi.org/10.1212/WNL.0000000000007518DOI Listing

Hemophagocytic lymphohistiocytosis associated with the use of lamotrigine.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Division of Pharmacovigilance, Office of Surveillance and Epidemiology, US Food and Drug Administration, Silver Spring, MD.

Objective: To describe adverse event reports of hemophagocytic lymphohistiocytosis (HLH) reported in association with lamotrigine.

Methods: The Food and Drug Administration Adverse Event Reporting System database of spontaneous adverse event reports and medical literature databases were searched for cases of HLH reported in association with lamotrigine. Cases were included if they met the case definition of suspected or confirmed HLH and if causal association was assessed as robust or supportive. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007517DOI Listing

Recommendations for the management of hemophagocytic lymphohistiocytosis in adults.

Blood 2019 Apr 16. Epub 2019 Apr 16.

Department of Women's & Children's Health, Karolinska Institutet, Sweden.

Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic T-cells. The primary (genetic) form, caused by mutations affecting lymphocyte cytotoxicity and immune regulation, is most common in children, while the secondary (acquired) form is most frequent in adults. Secondary HLH is commonly triggered by infections or malignancies but may also be induced by autoinflammatory/autoimmune disorders, in which case it is called macrophage activation syndrome (MAS or MAS-HLH). Read More

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http://dx.doi.org/10.1182/blood.2018894618DOI Listing
April 2019
2 Reads

Successful treatment of a child with idiopathic multicentric Castleman disease associated with hemophagocytic lymphohistiocytosis using tocilizumab.

Pediatr Blood Cancer 2019 Apr 15:e27759. Epub 2019 Apr 15.

Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

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http://dx.doi.org/10.1002/pbc.27759DOI Listing
April 2019
1 Read

Long-lasting low NK cell activity after hemophagocytic lymphohistiocytosis in an infant with spontaneous recovery.

Pediatr Int 2019 Apr 16. Epub 2019 Apr 16.

Department of Pediatrics, Kanazawa University, Kanazawa, Ishikawa, Japan.

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http://dx.doi.org/10.1111/ped.13803DOI Listing

V348I mutation in UL23 gene of human herpesvirus 1 in a case of herpetic hepatitis and haemophagocytic lymphohistiocytosis.

New Microbes New Infect 2019 May 14;29:100529. Epub 2019 Mar 14.

Department of Medical Sciences, Infectious Diseases, University of Turin, Turin, Italy.

We herein report the case of a young immunocompetent adult patient with a rapidly fatal haemophagocytic lymphohistiocytosis syndrome related to human herpesvirus 1 (HHV-1) infection, with herpetic hepatitis and persistent high-level viraemia despite treatment with acyclovir. Haemophagocytic lymphohistiocytosis was confirmed in the patient's spleen and bone marrow. HHV-1 DNA was extracted from whole blood and liver biopsy and the UL23 gene was sequenced. Read More

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http://dx.doi.org/10.1016/j.nmni.2019.100529DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6447736PMC

Hemophagocytic lymphohistiocytosis due to Streptococcus suis in a 12-year-old girl: A case report.

Medicine (Baltimore) 2019 Apr;98(15):e15136

Department of Pediatric Hematology, The First Hospital of Jilin University, Changchun, Jilin Province, People's Republic of China.

Rationale: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that can be caused by bacterial infection. Streptococcus suis (S. suis) is a zoonotic pathogen that can cause severe disease in both pigs and humans. Read More

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http://dx.doi.org/10.1097/MD.0000000000015136DOI Listing
April 2019
5 Reads

Unusual Presentation of Hemophagocytic Lymphohistiocytosis in a Kidney Transplant Patient.

Case Rep Transplant 2019 10;2019:3682378. Epub 2019 Mar 10.

Division of Nephrology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.

We are presenting a case of a middle-aged woman with history of remote kidney transplantation who had multiple admissions for septic shock-like picture, recurrent fever, and hypotension. Her shock manifestation would resolve after stress dose steroid administration and less than 24 hours of vasopressor administration. Initially, extensive workup was performed without revealing etiology. Read More

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http://dx.doi.org/10.1155/2019/3682378DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6431471PMC
March 2019
1 Read

Case Series of Hemophagocytic Lymphohistiocytosis from a Tertiary Care Centre: An Underdiagnosed Entity.

Turk Patoloji Derg 2019 Apr 12. Epub 2019 Apr 12.

Department of Pathology, PGIMER, Dr. Ram Manohar Lohia Hospital, NEW DELHI, INDIA.

Objective: Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon, life-threatening hyperinflammatory syndrome, caused by severe hypercytokinemia, due to an overstimulated but ineffective immune process. The presenting features of HLH are non-specific, mimicking many other diseases, and hence its early recognition still remains a challenge. It requires a high index of suspicion and detailed analysis of clinical and laboratory findings to arrive at a conclusive diagnosis. Read More

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http://dx.doi.org/10.5146/tjpath.2018.01456DOI Listing

Correction to: Factors predicting the recurrence of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children after treatment using the HLH-2004 protocol.

Int J Hematol 2019 May;109(5):629

Department of Pediatrics, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto, Nagano, 390-8621, Japan.

The correct name of the first author should be ''Ryu Yanagisawa'', and not ''Ryu Yanagaisawa'' as given in the original publication of the article. Read More

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http://dx.doi.org/10.1007/s12185-019-02641-xDOI Listing

Hermansky-Pudlak syndrome type II and lethal hemophagocytic lymphohistiocytosis: case description and review of the literature.

J Allergy Clin Immunol Pract 2019 Apr 8. Epub 2019 Apr 8.

Department of Pediatrics, Fondazione MBBM, University of Milano-Bicocca, Monza, Italy.

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http://dx.doi.org/10.1016/j.jaip.2019.04.001DOI Listing
April 2019
1 Read

Hematopoietic stem cell transplantation in children with Griscelli Syndrome type 2: Experience and outcomes.

Indian J Pathol Microbiol 2019 Apr-Jun;62(2):279-282

Department of Transplant Immunology and Immunogenetics, All India Institute of Medical Sciences, New Delhi, India.

Griscelli syndrome is a rare autosomal recessive inherited disorder characterized by hypopigmentation, silver colored hair, and associated immunological deficiency, which proves fatal in the absence of timely intervention. Our patients diagnosed with Griscelli syndrome-2 presented with fever, hepatosplenomegaly, and deranged hematological and biochemical parameters. Both cases underwent detailed investigations comprising of hair mount microscopic examination, degranulation assay, and mutational studies. Read More

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http://dx.doi.org/10.4103/IJPM.IJPM_645_18DOI Listing
April 2019
1 Read

Treatment of refractory secondary hemophagocytic lymphohistiocytosis with umbilical cord mesenchymal stem cells.

J Int Med Res 2019 Apr 8:300060519836533. Epub 2019 Apr 8.

3 Stem cell bank of Hainan Province & Hainan Heze Biotechnology Co., Ltd., Haikou, PR China.

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. Mesenchymal stem cells (MSCs) generate an immunosuppressive microenvironment by secreting cytokines and have been used to treat autoimmune diseases. We report the first case of refractory secondary HLH treated with umbilical cord MSCs. Read More

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http://dx.doi.org/10.1177/0300060519836533DOI Listing
April 2019
1 Read

An unusual case of hemophagocytic lymphohistiocytosis diagnosed by spinal nerve root biopsy.

J Neurosurg Pediatr 2019 04 5:1-5. Epub 2019 Apr 5.

Department of Neurosurgery; and

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease process characterized by aberrant immune system activation and an exaggerated inflammatory response. Establishing the diagnosis may be challenging and is achieved by satisfying a number of clinical criteria, in addition to demonstrating tissue hemophagocytosis. This syndrome is rapidly fatal if prompt diagnosis and treatment are not achieved. Read More

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http://dx.doi.org/10.3171/2019.1.PEDS18591DOI Listing
April 2019
2 Reads

Epidemiology of primary cutaneous gamma/delta T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma in the United States from 2006-2015: A Surveillance, Epidemiology, and End Results-18 analysis.

Br J Dermatol 2019 Apr 1. Epub 2019 Apr 1.

Department of Dermatology, University of Minnesota, Minneapolis, MN.

Primary cutaneous gamma/delta T-cell lymphoma (pcGDTCL) is a rare, aggressive T-cell lymphoma that presents with ulcerated nodules, extranodal dissemination, constitutional symptoms, and often, hemophagocytic lymphohistiocytosis (HLH). pcGDTCL is so named given the gamma/delta phenotype of the T-cell receptor (TCR). This article is protected by copyright. Read More

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http://dx.doi.org/10.1111/bjd.17985DOI Listing
April 2019
15 Reads

A case of pulmonary tuberculosis diagnosed in a patient with manifestations of haemophagocytic lymphohistiocytosis.

Oxf Med Case Reports 2019 Mar 29;2019(3):omz013. Epub 2019 Mar 29.

Department of Hematology and Oncology, Kita-Harima Medical Center, Hyogo, Japan.

An 80-year-old woman was admitted with continuous fever, hepatic dysfunction and cytopaenia. The presence of hepatosplenomegaly, hyperferritinaemia, hypofibrinogenaemia and phagocytosis by macrophages in the bone marrow was consistent with a diagnosis of haemophagocytic lymphohistiocytosis (HLH). We suspected that HLH was induced by pre-existing tuberculosis, and antitubercular agents were started. Read More

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https://academic.oup.com/omcr/article/doi/10.1093/omcr/omz01
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http://dx.doi.org/10.1093/omcr/omz013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440261PMC
March 2019
6 Reads

Natural Killer Cell Degranulation Defect: A Cause for Impaired NK-Cell Cytotoxicity and Hyperinflammation in Fanconi Anemia Patients.

Front Immunol 2019 21;10:490. Epub 2019 Mar 21.

Department of Paediatric Immunology and Leukocyte Biology, National Institute of Iummunohematology (ICMR), KEM Hospital, Mumbai, India.

Fanconi anemia (FA) is a rare inherited syndrome characterized by progressive bone marrow failure (BMF), abnormal skin pigmentation, short stature, and increased cancer risk. BMF in FA is multifactorial and largely results from the death of hematopoietic stem cells due to genomic instability. Also, inflammatory pathology in FA has been previously reported, however the mechanism is still not clear. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2019.00490
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http://dx.doi.org/10.3389/fimmu.2019.00490DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6438155PMC
March 2019
6 Reads

Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report.

J Immunother Cancer 2019 Apr 3;7(1):97. Epub 2019 Apr 3.

Department of Pathology, Sapporo Medical University, School of Medicine, South 1, West 17, Chuo-ku, Sapporo, Hokkaido, 060-8556, Japan.

Background: Immune checkpoint inhibitors (ICIs) have provided more options in the treatment of lung cancer. However, ICIs can cause several unfavorable reactions generally referred to as immune-related adverse effects.

Case Presentation: In this report, we present the case of a 52-year-old woman with successful regression of pleomorphic carcinoma of the lung following nivolumab therapy. Read More

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http://dx.doi.org/10.1186/s40425-019-0582-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448268PMC
April 2019
1 Read

Hemophagocytic Lymphohistiocytosis (HLH) with Unusual Trigger.

J Assoc Physicians India 2019 Jan;67(1):101

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January 2019
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A Unique Case of a Pediatric Patient With Blastic Plasmacytoid Dendritic Cell Neoplasm, Guillain Barre Syndrome, and Hemophagocytic Lymphohistiocytosis.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Division of Pediatric Hematology/Oncology, Children's Hospital of Eastern Ontario (CHEO), Ottawa, ON, Canada.

Blastic plasmacytoid dendritic cell neoplasm is a rare hematopoietic malignancy with a poor prognosis that is seen primarily in the elderly population. We describe a pediatric patient with blastic plasmacytoid dendritic cell neoplasm who subsequently developed Guillain Barre syndrome followed by hemophagocytic lymphohistiocytosis. All 3 conditions are uncommon, particularly in the pediatric population. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001464DOI Listing
March 2019
3 Reads

Epstein-Barr Virus-Associated T and NK-Cell Lymphoproliferative Diseases.

Front Pediatr 2019 15;7:71. Epub 2019 Mar 15.

Institute of Pathology and Neuropathology and Comprehensive Cancer Center Tübingen, University Hospital Tübingen, Eberhard-Karls-University, Tübingen, Germany.

EBV-associated T and NK-cell lymphoproliferative diseases (EBV-T/NK LPDs) are characterized by the transformation and proliferation of EBV-infected T or NK cells. The 2016 revised World Health Organization classification recognizes the following EBV-positive lymphoproliferative disorders (LPD): chronic active EBV infection (CAEBV) of T- and NK-cell type (cutaneous and systemic forms), systemic EBV-positive T-cell lymphoma of childhood, aggressive NK-cell leukemia, extranodal NK/T-cell lymphoma, nasal type, and the new provisional entity primary EBV-positive nodal T/NK-cell lymphoma. EBV-associated hemophagocytic lymphohistiocytosis (HLH), although not included in the WHO classification because it is a reactive, inflammatory disease, is included in this review because it can be life-threatening and may have overlapping features with other EBV+ T/NK LPDs. Read More

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https://www.frontiersin.org/article/10.3389/fped.2019.00071/
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http://dx.doi.org/10.3389/fped.2019.00071DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6428722PMC
March 2019
6 Reads

Refractory Macrophage Activation Syndrome in the setting of Adult Onset Still's Disease with Hemophagocytic Lymphohistiocytosis Detected on Skin Biopsy treated with Canakinumab and Tacrolimus.

J Cutan Pathol 2019 Mar 30. Epub 2019 Mar 30.

Department of Dermatology, UT Southwestern Medical Center, Dallas, TX.

A 19-year-old Caucasian female with adult onset Still's disease (AOSD) presented for evaluation of an acute clinical decompensation and atypical annular papules and plaques with purpura on the lower extremities. A punch biopsy demonstrated histiocytes with engulfed degenerated erythrocytes and lymphocytes, consistent with hemophagocytic lymphohistiocytosis (HLH). HLH, clinically referred to as macrophage activation syndrome, is a rare complication of AOSD and is life threatening. Read More

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http://dx.doi.org/10.1111/cup.13466DOI Listing

Association of Hemophagocytic Lymphohistiocytosis and Programmed Death 1 Checkpoint Inhibitors.

J Thorac Oncol 2019 Apr;14(4):e77-e78

Division of Hematology, Oncology, Blood and Marrow Transplantation, Department of Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa. Electronic address:

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http://dx.doi.org/10.1016/j.jtho.2018.11.035DOI Listing

The inhibitory receptors on NK cells and CTLs are upregulated in adult and adolescent patients with secondary hemophagocytic lymphohistiocytosis.

Clin Immunol 2019 Mar 23;202:18-28. Epub 2019 Mar 23.

Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China. Electronic address:

Hemophagocytic lymphohistiocytosis (HLH) includes primary HLH (pHLH) and secondary HLH (sHLH). Mutations that cause abnormal functions in natural killer (NK) cells and cytotoxic T lymphocytes (CTLs) are frequently identified in pHLH. However, why NK cells and CTLs exhibit abnormal functions in sHLH remains unclear. Read More

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http://dx.doi.org/10.1016/j.clim.2019.03.006DOI Listing

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

Blood Cells Mol Dis 2019 Mar 19;77:17-22. Epub 2019 Mar 19.

Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO, USA.

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. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10799796193010
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http://dx.doi.org/10.1016/j.bcmd.2019.03.002DOI Listing
March 2019
4 Reads

Pathogenic Gene Mutations or Variants Identified by Targeted Gene Sequencing in Adults With Hemophagocytic Lymphohistiocytosis.

Front Immunol 2019 7;10:395. Epub 2019 Mar 7.

Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.

Hemophagocytic lymphohistiocytosis (HLH) can be classified into primary HLH and secondary HLH. Primary HLH usually occurs in infants and children with an underlying genetic defect, and there are also teens and occasional adults with primary HLH. Most cases with secondary HLH are adult patients with secondary triggers including infections, malignancies, and autoimmune diseases. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00395DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6416222PMC
March 2019
2 Reads

Effect of Serum Deprivation Stress on Signal Induction Regulatory Protein-Alpha (SIRP-Alpha)-Mediated Erythrophagocytosis by Macrophages.

Med Sci Monit Basic Res 2019 03 21;25:100-106. Epub 2019 Mar 21.

Department of Urology, Texas Tech University Health Sciences Center, Lubbock, TX, USA.

BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome that involves loss of macrophages' self-cells recognition resulting in auto-phagocytosis of erythrocytes, leukocytes, and platelets and leading to multi-system effects. The pathogenesis of HLH is unclear but can be explained by malfunction of the physiologic inhibitory pathway through interaction between macrophage SIRP-alpha and erythrocyte CD 47. The goal of the present study was to evaluate if erythrocytes phagocytosis occurs as a result of altered macrophage SIRP-alpha expression during inflammatory/stressful conditions as seen in HLH. Read More

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http://dx.doi.org/10.12659/MSMBR.912946DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6441304PMC
March 2019
1 Read

Immune myopathy with large histiocyte-related myofiber necrosis.

Neurology 2019 Apr 20;92(15):e1763-e1772. Epub 2019 Mar 20.

From the Departments of Neurology (A.P., C.L., R.B.) and Pathology and Immunology (A.P., N.S., R.S.), Washington University School of Medicine, Saint Louis, MO; and Department of Neurology (Z.A.), King Saud University, Riyadh, Saudi Arabia.

Objective: To describe the features of a new, pathologically distinctive, acquired myopathy with an unusual pattern of scattered necrotic muscle fibers that are neighbored, surrounded, or invaded, by large, often multinucleated, histiocytic cells.

Methods: Retrospective review of records and muscle pathology of 4 patients.

Results: Clinical features common to our patients included muscle pain and proximal, symmetric, moderate to severe, weakness in the arms and legs progressing over 1-4 weeks. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007260DOI Listing
April 2019
1 Read

Cross-regulation of defective endolysosome trafficking and enhanced autophagy through TFEB in UNC13D deficiency.

Autophagy 2019 Mar 20:1-19. Epub 2019 Mar 20.

a Department of Molecular Medicine , The Scripps Research Institute , La Jolla , CA , USA.

Several lines of evidence support the occurrence of cross-regulation between the endocytic pathway and autophagy, but the molecular mechanisms regulating this process are not well-understood. Here, we show that the calcium sensor UNC13D regulates the molecular mechanism of late endosomal trafficking and endosomal maturation, and defects in UNC13D lead to macroautophagy upregulation. unc13d-null cells showed impaired endosomal trafficking and defective endocytic flux. Read More

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http://dx.doi.org/10.1080/15548627.2019.1596475DOI Listing
March 2019
1 Read

Diagnostic Dilemma of Disseminated Histoplasmosis Mimicking Hemophagocytosis Lymphohistiocytosis in Patient with Rheumatoid Arthritis on Anti-TNF Therapy: Case Report and Review of the Literature.

Case Rep Rheumatol 2019 12;2019:4169052. Epub 2019 Feb 12.

Assistant Professor, Division of Rheumatology, University of Kentucky, Lexington, USA.

Tumor necrosis factor inhibitors (TNFi) have become the cornerstone for the treatment of rheumatoid arthritis and other systemic autoimmune conditions. However, these biologic DMARDs can lead to various opportunistic infections such as viral infection, tuberculosis, and histoplasmosis. Furthermore, these biologics can also cause severe systemic inflammatory reactions known as hemophagocytosis lymphohistiocytosis (HLH) that can lead to multiorgan failure and high mortality. Read More

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https://www.hindawi.com/journals/crirh/2019/4169052/
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http://dx.doi.org/10.1155/2019/4169052DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390234PMC
February 2019
4 Reads

Hemophagocytic Lymphohistiocytosis in Pregnancy: A Case Series and Review of the Current Literature.

Case Rep Obstet Gynecol 2019 12;2019:9695367. Epub 2019 Feb 12.

Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that can be fatal in pregnancy. We report two cases of severe HLH that highlight etoposide use in pregnancy.

Case 1: 28-year-old G2P1 with lupus presented at 18 weeks with acute hypoxic respiratory failure, hepatic dysfunction, leukopenia, thrombocytopenia, and elevated ferritin. Read More

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http://dx.doi.org/10.1155/2019/9695367DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390238PMC
February 2019
1 Read

Severe skin disease in lupus associated with hemophagocytic lymphohistiocytosis: case reports and review of the literature.

BMC Rheumatol 2019 8;3. Epub 2019 Feb 8.

3Division of Rheumatology, Department of Medicine, University of Calgary, Calgary, Alberta Canada.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a severe clinical entity associated with high mortality in the adult population. HLH has been associated with infections, malignancy and autoimmune conditions such as Systemic Lupus Erythematosus (SLE), however this is often in the context of a disease flare. Currently, there are limited reports of inaugural SLE manifesting as HLH with a lack of consensus on treatment and management of these patients. Read More

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http://dx.doi.org/10.1186/s41927-019-0055-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390523PMC
February 2019
1 Read

Plcγ2/Tmem178 dependent pathway in myeloid cells modulates the pathogenesis of cytokine storm syndrome.

J Autoimmun 2019 Mar 15. Epub 2019 Mar 15.

Department of Orthopaedic Surgery, Musculoskeletal Research Center, Washington University School of Medicine, St. Louis, MO, USA; Shriners Hospitals for Children, St. Louis, MO, USA. Electronic address:

Cytokine storm syndrome (CSS) is a life-threatening condition characterized by excessive activation of T cells and uncontrolled inflammation, mostly described in patients with familial hemophagocytic lymphohistiocytosis and certain systemic auto-inflammatory diseases, such as systemic juvenile idiopathic arthritis (sJIA). Defects in T cell cytotoxicity as a mechanism for uncontrolled inflammation following viral infections fail to represent the whole spectrum of CSS. Evidence implicates dysregulated innate immune responses, especially activation of monocytes and macrophages, in patients with CSS. Read More

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http://dx.doi.org/10.1016/j.jaut.2019.02.005DOI Listing
March 2019
1 Read

Hepatitis A infection related haemophagocytic syndrome: a case report and systematic review.

Trop Doct 2019 Mar 14:49475519834804. Epub 2019 Mar 14.

2 Professor, Department of Gastroenterology, PGIMER, Chandigarh, India.

Haemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of excessive inflammation and tissue destruction owing to abnormal immune activation. We report an unusual case of haemophagocytosis associated with hepatitis A virus (HAV) infection in a 21-year-old man. This was further complicated by haemolysis secondary to G-6-PD deficiency and fungal sepsis. Read More

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http://dx.doi.org/10.1177/0049475519834804DOI Listing
March 2019
4 Reads

Systemic Epstein-Barr Virus-positive T-Cell Lymphoma of Childhood Presentation With Hemophagocytosis.

J Pediatr Hematol Oncol 2019 Mar 12. Epub 2019 Mar 12.

Department of Anatomy and Cell Biology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan.

A 2-year-old Asian girl presented to our facility for the evaluation of thrombocytopenia. She was treated with intravenous immunoglobulin under the impression of immune thrombocytopenia. However, her body temperature spiked and progressive pancytopenia, hepatosplenomegaly, abnormal liver function, coagulopathy, and pulmonary infiltration developed. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001452DOI Listing
March 2019
3 Reads

Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis.

Case Rep Crit Care 2019 4;2019:3580796. Epub 2019 Feb 4.

Department of Critical Care Medicine, University of Calgary, Alberta, Canada.

Background: We describe a case of secondary Hemophagocytic Lymphohistiocytosis (HLH) from autoimmune hepatitis mimicking severe sepsis in a man admitted to the intensive care unit.

Case Presentation: A 34-year-old Pakistani male with a prior history of biopsy-proven autoimmune hepatitis presented to a regional hospital with severe fever, cytopenias, hyperferritinemia, hypertriglyceridemia, splenomegaly, and a bone marrow biopsy showing hemophagocytosis. After ruling out mimicking conditions, a diagnosis of HLH was made using the HLH-2004 diagnostic criteria. Read More

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https://www.hindawi.com/journals/cricc/2019/3580796/
Publisher Site
http://dx.doi.org/10.1155/2019/3580796DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378761PMC
February 2019
5 Reads

Reactivation of latent and disseminated cytomegalovirus in a returning traveller with ulcerative colitis.

JMM Case Rep 2018 Dec 23;5(12):e005170. Epub 2018 Nov 23.

The Royal London Hospital, Whitechapel Road, Whitechapel, London E1 1BB, UK.

Introduction: We describe a case of progressive disseminated histoplasmosis (PDH) and disseminated cytomegalovirus (CMV) with development of haemophagocytic lymphohistiocytosis in a 62-year-old man of Bangladeshi origin living in the UK.

Case Presentation: The patient had a background of ulcerative colitis for which he took prednisolone and azathioprine. He presented with fever, lethargy, cough, weight loss and skin redness, and was initially treated for bacterial cellulitis and investigated for underlying malignancy. Read More

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http://dx.doi.org/10.1099/jmmcr.0.005170DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6412033PMC
December 2018
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The Performance of Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis in Critically Ill Patients.

J Intensive Care Med 2019 Mar 12:885066619837139. Epub 2019 Mar 12.

4 Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, Cleveland, OH, USA.

Objective:: The diagnostic criteria for secondary hemophagocytic lymphohistiocytosis (HLH) have not been validated in the critically ill adult population. We set out to evaluate the performance of diagnostic criteria and determine the ferritin cutoff in critically ill adults.

Design:: A retrospective single-center study. Read More

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http://dx.doi.org/10.1177/0885066619837139DOI Listing

Hemophagocytic lymphohistiocytosis as a harbinger of aggressive lymphoma: a case series.

Int J Hematol 2019 May 8;109(5):553-562. Epub 2019 Mar 8.

Davidoff Cancer Center, Rabin Medical Center, Institute of Hematology, Beilinson Hospital, 49100, Petah Tikva, Israel.

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome, which can manifest either secondary to a variety of underlying causes, or due to a primary genetic defect. Malignancy is the most common underlying disease in adults with HLH, with lymphomas being the most common malignancy. Lymphoma-associated hemophagocytic syndrome (LAHS) typically follows a rapidly progressive clinical course and is associated with poor prognosis. Read More

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http://dx.doi.org/10.1007/s12185-019-02623-zDOI Listing
May 2019
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Rare cause of Hemophagocytic Lymphohistiocytosis due to mutation in PRF1 and SH2D1A genes in two children - a case report with a review.

BMC Pediatr 2019 Mar 8;19(1):73. Epub 2019 Mar 8.

FRIGE's Institute of Human Genetics, FRIGE House, Jodhpur Gam Road, Satellite, Ahmedabad, Gujarat, 380015, India.

Background: Hemophagocytic Lymphohistiocytosis (HLH) is a rare, complex, life-threatening hyper-inflammatory condition due to over activation of lymphocytes mediated secretory cytokines in the body. It occurs as a primary HLH due to genetic defect that mostly occurs in the childhood and associated with early neonatal death. Secondary HLH is triggered by secondary to infection and can occur at any age. Read More

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http://dx.doi.org/10.1186/s12887-019-1444-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6407181PMC
March 2019
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[Cytomegalovirus meningoencephalitis in a diffuse large B-cell lymphoma patient undergoing salvage chemotherapy].

Rinsho Ketsueki 2019;60(2):124-129

Department of Hematology/Oncology, Wakayama Medical University.

A 63-year-old woman was admitted to our hospital to receive a fourth course of modified rituximab-ESHAP chemotherapy for relapsed primary breast diffuse large B-cell lymphoma. She developed hemophagocytic lymphohistiocytosis (HLH) 20 days after admission. Polymerase chain reaction (PCR) detected cytomegalovirus (CMV) DNA in her peripheral blood; therefore, she was diagnosed with CMV-associated HLH and consequently treated with foscarnet (FCN). Read More

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http://dx.doi.org/10.11406/rinketsu.60.124DOI Listing
January 2019
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Ceftriaxone-Induced Immune Hemolytic Anemia: In Vitro Reversal with Peptide Inhibitor of Complement C1 (PIC1).

Case Rep Hematol 2019 30;2019:4105653. Epub 2019 Jan 30.

Department of Pediatrics, Eastern Virginia Medical School, 700 West Olney Road, Norfolk, VA 23507, USA.

We report a case of ceftriaxone-induced immune hemolytic anemia in a 10-year-old with chronic active Epstein-Barr virus disease and hemophagocytic lymphohistiocytosis. After chemotherapy, she became febrile and received ceftriaxone. She rapidly developed respiratory failure and anemia. Read More

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http://dx.doi.org/10.1155/2019/4105653DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374879PMC
January 2019
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A Case of Congenital Anaplastic Large Cell Lymphoma in a Very Preterm Low-Birth Weight Neonate.

J Pediatr Hematol Oncol 2019 Feb 28. Epub 2019 Feb 28.

Cleveland Clinic, Cleveland, Ohio.

A premature infant male was born at 30 weeks' gestation with severe coagulopathy and thrombocytopenia. Over the first days of his life, the patient developed evidence of immune hyperactivation with adenopathy, hepatosplenomegaly, and elevated ferritin. Although the patient met diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH), flow cytometric based assays were not consistent with primary HLH. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001450DOI Listing
February 2019
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Pembrolizumab-induced Autoimmune Hemolytic Anemia and Hemophagocytic Lymphohistiocytosis in Non-small Cell Lung Cancer.

Intern Med 2019 1;58(5):699-702. Epub 2019 Mar 1.

Department of Respiratory Medicine, National Hospital Organization Okayama Medical Center, Japan.

We herein report a 78-year old man with squamous cell carcinoma of the lungs treated with pembrolizumab. At 10 days after the administration of pembrolizumab, he showed progressive anemia and increased levels of bilirubin. Because the findings of a direct coombs test and cold hemagglutinin were positive, we diagnosed the patient with autoimmune hemolytic anemia and treated him with prednisolone. Read More

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http://dx.doi.org/10.2169/internalmedicine.1001-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443545PMC
April 2019
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Critically Severe Case of Neonatal Herpes with High Viral Load and Hemophagocytic Syndrome.

Tohoku J Exp Med 2019 03;247(3):149-152

Department of Pediatrics, The University of Tokyo Hospital.

Neonatal disseminated herpes simplex virus (HSV) infection is a severe disease with high mortality and morbidity; yet, the pathophysiology remains unclear. Here, we report a male infant with disseminated HSV type 1 (HSV-1) infection, complicated by hemophagocytic lymphohistiocytosis (HLH) and multiple organ failure. The infant, born at 39 weeks of gestation by normal delivery, developed fever (38. Read More

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http://dx.doi.org/10.1620/tjem.247.149DOI Listing
March 2019
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Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis.

Pediatr Hematol Oncol 2019 Mar 1:1-16. Epub 2019 Mar 1.

a Department of Pediatrics , Dr. Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital , Ankara , Turkey.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation. Secondary HLH syndrome develops as a complication of infection, drugs, rheumatologic conditions, or malignancy. The main objectives of this work were to identify the etiology of secondary HLH and prognostic factors associated with mortality. Read More

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http://dx.doi.org/10.1080/08880018.2019.1572253DOI Listing
March 2019
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Senescence-accelerated mice (SAMP1/TA-1) treated repeatedly with lipopolysaccharide develop a condition that resembles hemophagocytic lymphohistiocytosis.

Haematologica 2019 Feb 28. Epub 2019 Feb 28.

Nihon University School of Medicine.

Hemophagocytic lymphohistiocytosis is a life-threatening systemic hyperinflammatory disorder with primary and secondary forms. Primary hemophagocytic lymphohistiocytosis is associated with inherited defects in various genes that affect the immunological cytolytic pathway. Secondary hemophagocytic lymphohistiocytosis is not inherited, but complicates various medical conditions including infection, autoinflammatory/autoimmune disease, and malignant disease. Read More

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http://dx.doi.org/10.3324/haematol.2018.209551DOI Listing
February 2019
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