Cutaneous histopathologic, immunohistochemical, and clinical manifestations in patients with hemophagocytic syndrome. Military Medical Consortium for Applied Retroviral Research (MMCARR)

1992 ◽  
Vol 128 (2) ◽  
pp. 193-200 ◽  
Author(s):  
K. J. Smith
Keyword(s):  
Hemophagocytic Syndrome ◽  
Clinical Manifestations

scholarly journals Clinical Warning of Hemophagocytic Syndrome Caused by Epstein-barr Virus

2020 ◽  
Author(s):  
Jinjin Shi ◽  
Chu Chu ◽  
Min Yu ◽  
Dandan Zhang ◽  
Yuqin Li ◽  
...  
Keyword(s):  
Laboratory Tests ◽  
Epstein Barr Virus ◽  
Hemophagocytic Syndrome ◽  
Clinical Manifestations ◽  
Prognostic Indicator ◽  
Barr Virus ◽  
Ebv Infection ◽  
Ebv Dna ◽  
Epstein Barr ◽  
D Dimer

Abstract Objectives This study aimed to compare the clinical features and laboratory tests of infectious mononucleosis (IM) and hemophagocytic syndrome (HLH) caused by Epstein-Barr virus (EBV) in 1-3-year-old children and to explore the risk factor of HLH caused by EBV (EBV-HLH). Methods The clinical data of 92 children with EBV infection admitted in our hospital from 2011 to 2019 were collected; 61 cases were diagnosed as EBV-IM, and 31 cases were diagnosed as EBV-HLH. The subjects’ clinical manifestations and laboratory tests were analyzed retrospectively. Results Compared with EBV-IM patients, EBV-HLH patients had longer durations of fever, both before hospitalization and overall, and a higher probability of hepatomegaly. The levels of ALT, AST, LDH, TG, SF, D-Dimer and the plasma EBV DNA load of EBV-HLH patients were significantly higher than those of EBV-IM patients. The absolute values of CD3+, CD4+, CD8+, NK, and CD3-CD19+ cells and IgA and IgM levels of EBV-HLH patients were significantly lower than those of EBV-IM patients. The plasma EBV DNA load was positively correlated with the PT, TT, α-HBDH, AST, LDH, CK, Scr, BUN, UA, TG, and CRP levels in EBV-HLH patients, and the plasma EBV DNA load was positively correlated with the D-Dimer level in the EBV-IM patients. Among the 10 different potential markers, at the cut-off point of 1721.500 µg/L, the sensitivity and specificity of D-Dimer was 88.90% and 90.20%, respectively. Conclusion The D-Dimer level may be a good prognostic indicator of EBV-HLH caused by EBV.

scholarly journals Clinical Characteristics of Hemophagocytic Syndrome: 46 Cases Report and Literature Re-View

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5916-5916
Author(s):  
Weibin Zhuo ◽  
Ya Gao ◽  
Chunyan Yang ◽  
Ying Xu ◽  
Yintian Zhang ◽  
...  
Keyword(s):  
Survival Rate ◽  
Hemophagocytic Syndrome ◽  
Conflicts Of Interest ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Survival Group ◽  
Β2 Microglobulin ◽  
Short Period ◽  
Death Group

Abstract Objective HPS is caused by a variety of diseases, the most common causes including infections, malignancies and autoimmune diseases. It has an acute onset, a dangerous condition, rapid progress, and a high mortality rate. The main clinical manifestations are persistent fever, hepatosplenomegaly, hematocytopenia and tissue cell phagocytosis.Most of the current research is focused on children, and there are few reports on adult HPS.And lack of multi-center, prospective research, its pathogenesis, diagnosis, treatment is still poorly understood. Methods To analyze the clinical manifestations,laboratory data, therapy and prognosis in patients with hemophagocytic syndrome, 46 patients (over 18 years old) with HPS in our hospital from June 2011 to September 2017 were analyzed.The scope of the study included the patient's age, gender, etiology, clinical manifestations, laboratory indicators, treatment and prognosis, and analyzed its clinical, laboratory and prognostic characteristics. Result Of the 46 patient cases, 19 cases were secondary to cancer and 11 cases secondary to infection,10 cases were considered as malignant lymphoma by PET- CT,other 6 cases of unknown etiology.A total of 31 patients with EBV infection were diagnosed by EBV-DNA in all patients.Forty-six patients had various clinical manifestations, all had fever, mostly persistent irregular hyperthermia, and antibiotic treatment was not effective.There are also splenomegaly, serous effusion, respiratory symptoms, hemorrhage, central nervous system symptoms, renal dysfunction。According to HPS -2004 criteria,the coincidence rate of indices were: fever (100%), high level of serun ferritin (100% ),cytopenias (93.48% ),splenomegaly (91.30% ), hemophagocytosis in bone marrow,spleen or lymph nodes (84.78% ),hypofibrinogenemia (67.39% ), hypertriglyceridemia (54.05% ). Among cancer,infection and unknown etiology group,there were statistically differences on Ferritin and β2 microglobulin (P<0.05), It can be seen that the levels of Ferritin and β2 microglobulin in the tumor-associated HPS group were significantly higher than those in the unknown etiology and infection-related HPS group. The overall survival rate of the 46 patients was 11.1%.Causes of death included respiratory and circulatory failure (36.4%), cardiac arrest (18.2%), multiple organ failure (18.2%), hemorrhage, cerebral palsy (18.2%), and liver failure (9.1%).Comparing the laboratory indicators of the survival group and the death group at the initial diagnosis, the Lactic dehydrogenase and Triglyceride levels in the death group were higher than those in the survival group, while the Fibrinogen level in the death group was lower than that in the survival group. Conclusion The secondary HPS occurs from various underlined disease,many associated with Epstein- Barr virus infection.Cancer,especially NK/T- cell lymphoma is the main cause.Adult HPS progresses rapidly due to the disease, which makes it difficult to confirm the diagnosis in time. Some patients have deteriorated before the diagnosis is perfected.Increased sensitivity indicators such as sCD25 and NK cell activity can help early diagnosis. The 1-month survival rate of the chemotherapy group was higher than that of the anti-infective group alone, indicating that chemotherapy and controlled hemorrhage are meaningful for controlling the disease in a short period of time, while the simple anti-infection effect is poor.Adult HPS has only gradually gained attention in recent years. How to identify the underlying cause of HPS and to formulate a treatment strategy based on different incentives and prognostic factors is essential. Disclosures No relevant conflicts of interest to declare.

scholarly journals Clinical warning of hemophagocytic syndrome caused by Epstein-Barr virus

2020 ◽  
Author(s):  
Jinjin Shi ◽  
Chu Chu ◽  
Min Yu ◽  
Dandan Zhang ◽  
Yuqin Li ◽  
...  
Keyword(s):  
Laboratory Tests ◽  
Epstein Barr Virus ◽  
Hemophagocytic Syndrome ◽  
Clinical Manifestations ◽  
Prognostic Indicator ◽  
Barr Virus ◽  
Ebv Infection ◽  
Ebv Dna ◽  
Epstein Barr ◽  
D Dimer

Abstract Objectives: This study aimed to compare the clinical features and laboratory tests of infectious mononucleosis (IM) and hemophagocytic syndrome (HLH) caused by Epstein-Barr virus (EBV) in 1-3-year-old children and to explore the risk factor of HLH caused by EBV (EBV-HLH). Methods: The clinical data of 92 children with EBV infection admitted in our hospital from 2011 to 2019 were collected; 61 cases were diagnosed as EBV-IM, and 31 cases were diagnosed as EBV-HLH. The subjects’ clinical manifestations and laboratory tests were analyzed retrospectively. Results: Compared with EBV-IM patients, EBV-HLH patients had longer durations of fever, both before hospitalization and overall, and a higher probability of hepatomegaly. The levels of ALT, AST, LDH, TG, SF, D-Dimer and the plasma EBV DNA load of EBV-HLH patients were significantly higher than those of EBV-IM patients. The absolute values of CD3 + , CD4 + , CD8 + , NK, and CD3-CD19 + cells and IgA and IgM levels of EBV-HLH patients were significantly lower than those of EBV-IM patients. The plasma EBV DNA load was positively correlated with the PT, TT, α-HBDH, AST, LDH, CK, Scr, BUN, UA, TG, and CRP levels in EBV-HLH patients, and the plasma EBV DNA load was positively correlated with the D-Dimer level in the EBV-IM patients. Among the 10 different potential markers, at the cut-off point of 1721.500 µg/L, the sensitivity and specificity of D-Dimer was 88.90% and 90.20%, respectively. Conclusion: The D-Dimer level may be a good prognostic indicator of EBV-HLH caused by EBV.

scholarly journals Hemophagocytic Syndrome Associated with Immune Reconstitution Inflammatory Syndrome in a Patient with AIDS Related Burkitt’s Leukemia/Lymphoma

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Metin Kanıtez ◽  
Mahir Kapmaz ◽  
Nilufer Alpay ◽  
Fatih Selcukbiricik ◽  
Atahan Çağatay ◽  
...  
Keyword(s):  
Immune Function ◽  
Immune Reconstitution Inflammatory Syndrome ◽  
Highly Active Antiretroviral Therapy ◽  
Immune Reconstitution ◽  
Hemophagocytic Syndrome ◽  
Opportunistic Infections ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
High Dose ◽  
Inflammatory Syndrome

Highly active antiretroviral therapy (HAART) has markedly decreased human immunodeficiency virus- (HIV-) related mortality and the incidence of opportunistic infections. The dramatic reduction in HIV-1 RNA and increase in CD4 lymphocyte count mean a recovery in immune function. This restoration in immune function may be associated with paradoxical deterioration in subclinical opportunistic infections in some patients, a condition called immune reconstitution inflammatory syndrome (IRIS). IRIS, a “paradoxical” inflammatory response to either previously treated or subclinical infections or noninfectious diseases, can manifest during the restoration phase of immunity hemophagocytic syndrome (HS) which is a very rare complication in patients with acquired immune deficiency syndrome (AIDS). We describe a case of hemophagocytic syndrome associated with IRIS in a patient with AIDS related Burkitt’s leukemia/lymphoma (BL). IRIS was probably the cause of hemophagocytosis for our patient. Zoster infection may facilitate to IRIS. With the increasing number of people with HIV infection and the accompanying use of HAART, much more clinical manifestations of IRIS will be experienced especially in patients given high dose chemotherapy, just like in our case.

scholarly journals Clinical warning of hemophagocytic syndrome caused by Epstein-Barr virus

2020 ◽  
Author(s):  
Jinjin Shi ◽  
Chu Chu ◽  
Min Yu ◽  
Dandan Zhang ◽  
Yuqin Li ◽  
...  
Keyword(s):  
Laboratory Tests ◽  
Epstein Barr Virus ◽  
Hemophagocytic Syndrome ◽  
Clinical Manifestations ◽  
Prognostic Indicator ◽  
Barr Virus ◽  
Ebv Infection ◽  
Ebv Dna ◽  
Epstein Barr ◽  
D Dimer

Abstract Objectives: This study aimed to compare the clinical features and laboratory tests of infectious mononucleosis (IM) and hemophagocytic syndrome (HLH) caused by Epstein-Barr virus (EBV) in 1-3-year-old children and to explore the risk factor of HLH caused by EBV (EBV-HLH).Methods: The clinical data of 92 children with EBV infection admitted in our hospital from 2011 to 2019 were collected; 61 cases were diagnosed as EBV-IM, and 31 cases were diagnosed as EBV-HLH. The subjects’ clinical manifestations and laboratory tests were analyzed retrospectively.Results: Compared with EBV-IM patients, EBV-HLH patients had longer durations of fever, both before hospitalization and overall, and a higher probability of hepatomegaly. The levels of ALT, AST, LDH, TG, SF, D-Dimer and the plasma EBV DNA load of EBV-HLH patients were significantly higher than those of EBV-IM patients. The absolute values of CD3+, CD4+, CD8+, NK, and CD3-CD19+ cells and IgA and IgM levels of EBV-HLH patients were significantly lower than those of EBV-IM patients. The plasma EBV DNA load was positively correlated with the PT, TT, α-HBDH, AST, LDH, CK, Scr, BUN, UA, TG, and CRP levels in EBV-HLH patients, and the plasma EBV DNA load was positively correlated with the D-Dimer level in the EBV-IM patients. Among the 10 different potential markers, at the cut-off point of 1721.500 µg/L, the sensitivity and specificity of D-Dimer was 88.90% and 90.20%, respectively.Conclusion: The D-Dimer level may be a good prognostic indicator of EBV-HLH caused by EBV.

scholarly journals Hemophagocytic syndrome after recovery from SARS-Cov-2 infection: a Case Report

2021 ◽  
Author(s):  
Marco Meazza Prina ◽  
Francesca Martini ◽  
Federico Bracchi ◽  
Daniela Di Mauro ◽  
Anna Fargnoli ◽  
...  
Keyword(s):  
Blood Count ◽  
Low Dose ◽  
Hemophagocytic Syndrome ◽  
Immunosuppressive Treatment ◽  
Clinical Manifestations ◽  
High Dose ◽  
Cytokine Storm ◽  
Case Presentation ◽  
Caucasian Female ◽  
Colchicine Therapy

Abstract Background Hemophagocytic syndrome (HPS) or hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory disease, whose diagnosis is based on the HLH-2004 criteria. In secondary forms of HLH (sHLH), the primary goal is treating the triggering factors such as SARS-CoV-2-19 infection. The link between the cytokine storm related to SARS-CoV-2-19infection and development of sHLH has already been reported since the onset of pandemic (1), but little is known about clinical manifestations of HLH which develop after patient’s recovery from SARS-CoV-2-19 infection. Case presentation A 56-year-old caucasian female was diagnosed with sHLH according to HLH-2004 criteria, after recovery from a mild symptomatic SARS-CoV-2-19 infection and received immunosuppressive treatment (high-dose steroids, IVIG, low dose Ruxolitinib and Etoposide. Antiviral (acyclovir), antibiotic (sulfamethoxazole / trimethoprim) and heparin prophylaxes were administered. Colchicine therapy was added considering the pericarditis. Improvement in patient symptoms and normalization of blood count as well as fibrinogen and ferritin values was observed.Conclusion Our report suggests that HLH-like syndrome might be secondary to SARS CoV-2-19 infection, even after the patient completely recovered from the mildly symptomatic viral infection. In addition, we underline the treatment with low dose ruxolitinib plus etoposide as a potential choice for SARS-CoV-2-19 related HLH.

scholarly journals Clinical warning of hemophagocytic syndrome caused by Epstein-Barr virus

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Jinjin Shi ◽  
Chu Chu ◽  
Min Yu ◽  
Dandan Zhang ◽  
Yuqin Li ◽  
...  
Keyword(s):  
Laboratory Tests ◽  
Epstein Barr Virus ◽  
Hemophagocytic Syndrome ◽  
Clinical Manifestations ◽  
Prognostic Indicator ◽  
Barr Virus ◽  
Ebv Infection ◽  
Ebv Dna ◽  
Epstein Barr ◽  
D Dimer

Abstract Objectives This study aimed to compare the clinical features and laboratory tests of infectious mononucleosis (IM) and hemophagocytic syndrome (HLH) caused by Epstein-Barr virus (EBV) in 1–3-year-old children and to explore the risk factor of HLH caused by EBV (EBV-HLH). Methods The clinical data of 92 children with EBV infection admitted in our hospital from 2011 to 2019 were collected; 61 cases were diagnosed as EBV-IM, and 31 cases were diagnosed as EBV-HLH. The subjects’ clinical manifestations and laboratory tests were analyzed retrospectively. Results Compared with EBV-IM patients, EBV-HLH patients had longer durations of fever, both before hospitalization and overall, and a higher probability of hepatomegaly. The levels of ALT, AST, LDH, TG, SF, D-Dimer and the plasma EBV DNA load of EBV-HLH patients were significantly higher than those of EBV-IM patients. The absolute values of CD3+, CD4+, CD8+, NK, and CD3-CD19+ cells and IgA and IgM levels of EBV-HLH patients were significantly lower than those of EBV-IM patients. The plasma EBV DNA load was positively correlated with the PT, TT, α-HBDH, AST, LDH, CK, Scr, BUN, UA, TG, and CRP levels in EBV-HLH patients, and the plasma EBV DNA load was positively correlated with the D-Dimer level in the EBV-IM patients. Among the 10 different potential markers, at the cut-off point of 1721.500 μg/L, the sensitivity and specificity of D-Dimer was 88.90 and 90.20%, respectively. Conclusion The D-Dimer level may be a good prognostic indicator of EBV-HLH caused by EBV.

scholarly journals LINFOHISTIOCITOSIS HEMOFAGOCÍTICA ASOCIADA AL DENGUE GRAVE

2021 ◽  
Vol 6 (4) ◽  
pp. 65
Author(s):  
Nereida Josefina Valero ◽  
Lisbeth Georgina Polanco Peláez ◽  
Marcos Jacob Sacan Arriola
Keyword(s):  
Dengue Virus ◽  
Natural Killer ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Hemophagocytic Syndrome ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Severe Dengue ◽  
Timely Diagnosis ◽  
Palabras Clave ◽  
Revisión Sistemática

La linfohistiocitosis hemofagocítica (LHH) es una enfermedad que afecta al sistema inmunitario, a través de la activación macrofágica descontrolada, con falla en las funciones de las células asesinas natural killer (NK) y los linfocitos T citotóxicos. El dengue es la arbovirosis de mayor impacto en salud pública, que se presenta en áreas tropicales del mundo. El objetivo de esta investigación fue analizar la asociación de la linfohistiocitosis hemofagocítica a la severidad del dengue, síntomas y diagnóstico. Se aplicó una metodología con diseño documental con revisión sistemática de la bibliografía en las bases de datos científicas y buscadores PubMed, Medline, Scielo, Elsevier y Google Académico, utilizando las palabras clave: síndrome hemofagocítico, linfohistiocitosis hemofagocítica, virus dengue, dengue grave, natural killer e inflamación. Se seleccionaron 85 artículos bajo criterios de inclusión y exclusión, tanto en inglés como en español, publicados entre los años 2010-2021. El síndrome hemofagocítico es el responsable de los síntomas que en determinado momento prolongan o complican el dengue grave, se caracteriza por una activación inmune patológica, con signos y síntomas de inflamación excesiva, donde sus principales manifestaciones clínicas son fiebre, citopenias, esplenomegalias, hemofagocitosis, hipertrigliceridemia e hipofibrinogenemia, teniendo encuentra que el problema principal en esta patología es el diagnóstico oportuno, dado que su presentación generalmente esta enmascarada por la enfermedad viral que usualmente se convierte en el desencadenante, y la persistencia o progresión de los síntomas suele ser pasada por alto. La LHH asociada al dengue se encuentra descrita como una forma inusual y grave, lo que da como resultado una tormenta de citocinas liberadas durante la enfermedad. La asociación entre ambas entidades causa manifestaciones clínicas amplias que pueden iniciar desde lo menos grave hasta lo hemorrágico, el cuadro febril, dolor abdominal y dificultad respiratoria, entre otras manifestaciones clínicas, resaltan, lo cual debe orientar al diagnóstico oportuno y evitar la muerte del paciente. Palabras clave: síndrome hemofagocítico linfohistiocitosis, virus dengue, dengue grave, dengue severo, natural killer.   ABSTRACT Hemophagocytic lymphohistiocytosis (HHL) is a disease that affects the immune system, through uncontrolled macrophage activation, with failure of natural killer (NK) cells and cytotoxic T lymphocytes. Dengue is the arbovirus with the greatest impact on public health, occurring in tropical areas of the world. The objective of this research was to analyze the association of hemophagocytic lymphohistiocytosis with the severity of dengue, symptoms and diagnosis. A documentary design methodology was applied with a systematic review of the bibliography in the scientific databases and search engines PubMed, Medline, Scielo, Elsevier and Google Scholar, using the key words: hemophagocytic syndrome, hemophagocytic lymphohistiocytosis, dengue virus, severe dengue, natural killer and inflammation. 85 articles were selected under inclusion and exclusion criteria, both in English and Spanish, published between 2010-2021. Hemophagocytic syndrome is responsible for the symptoms that at a certain time prolong or complicate severe dengue, it is characterized by pathological immune activation, with signs and symptoms of excessive inflammation, where its main clinical manifestations are fever, cytopenias, splenomegaly, hemophagocytosis, hypertriglyceridemia and hypofibrinogenemia, having found that the main problem in this pathology is timely diagnosis, since its presentation is generally masked by the viral disease that usually becomes the trigger, and the persistence or progression of symptoms is often overlooked. Dengue-associated LHH is described as an unusual and severe form, resulting in a storm of cytokines released during the disease. The association between both entities causes wide clinical manifestations that can start from the less severe to the hemorrhagic, the feverish picture, abdominal pain and respiratory distress, among other clinical manifestations, they highlight, which should guide the timely diagnosis and avoid the death of the patient. Keywords: hemophagocytic lymphohistiocytosis syndrome, dengue virus, severe dengue, severe dengue, natural killer.

scholarly journals Hemophagocytic syndrome secondary to SARS-Cov-2 infection: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Marco Meazza Prina ◽  
Francesca Martini ◽  
Federico Bracchi ◽  
Daniela Di Mauro ◽  
Anna Fargnoli ◽  
...  
Keyword(s):  
Case Report ◽  
Viral Infection ◽  
Low Dose ◽  
Hemophagocytic Syndrome ◽  
Clinical Manifestations ◽  
Cytokine Storm ◽  
Case Presentation ◽  
Triggering Factors

Abstract Background Hemophagocytic syndrome (HPS) is a severe hyperinflammatory disease, whose diagnosis is based on the HLH-2004 criteria. In secondary forms of HLH (sHLH), the primary goal is treating the triggering factors such as COVID-19 (Coronavirus disease 2019). The link between the cytokine storm related to COVID-19 and development of sHLH has already been reported since the onset of pandemic, but little is known about clinical manifestations of HLH which develop after the patient’s recovery from mild symptomatic or asymptomatic Sars-CoV-2 infection. Case presentation We describe the case of a woman diagnosed with sHLH related to previous Sars-CoV-2 infection and successfully treated with steroids, colchicine, etoposide and ruxolitinib. Conclusions Our report suggests that HLH-like syndrome might be secondary to Sars-CoV-2 infection, even if the patient utterly recovered from the mildly symptomatic viral infection. In addition, we underline the treatment with low dose ruxolitinib plus etoposide as a potential choice for Sars-CoV-2 infection related HLH.

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