Treatment Of Hemophagocytic Syndrome Research Articles

One Hemophagocytic Syndrome Relating to Adult Epstein-Barr Virus Infection and a Literature Review

Background: Adult hemophagocytic syndrome is a critical condition that is often difficult to diagnose and results in a bad prognosis because of the lack of effective and unified treatment. Aim: To investigate the clinical, diagnosis and treatment of hemophagocytic syndrome. Case Presentation: A 22-year-old female patient received a splenectomy, and the spleen was sent to the King Med Center of Medical Inspection. The results confirmed the diagnosis of hemophagocytic syndrome related to EB (Epstein-Barr) virus infection. Conclusion: Early diagnosis and early intervention are important factors for affecting the progression of the disease and improving its prognosis.

Reactive Hemophagocytic Syndrome in Adult Korean Patients with Systemic Lupus Erythematosus: A Case-Control Study and Literature Review

To determine the characteristics of hemophagocytic syndrome (HPS) in adult Korean patients with systemic lupus erythematosus (SLE). We reviewed the medical records of 1033 adult patients with SLE for a recent 14-year period and identified 15 patients who had developed HPS. Forty-two age- and sex-matched patients with SLE admitted for other manifestations were included as disease controls. Features of HPS in these patients were analyzed. Reactive HPS occurred from some distinct causes during the course of SLE. HPS was associated with SLE in 11 patients (4 at onset of SLE and 7 at SLE flare), infection in 3 patients (2 bacterial infection; 1 viral infection), and drug use (azathioprine) in 1 patient. Common clinical features included fever (93.3%), hepatomegaly (60.0%), and splenomegaly (60.0%). Steroid pulse therapy (46.7%), immunosuppressants (46.7%), and intravenous immunoglobulin (46.7%) were frequently used for treatment of HPS. One patient (6.7%) died. Compared with SLE patients without HPS, those with HPS showed a higher SLEDAI score (p = 0.003) and lower levels of plasma leukocytes (p < 0.001), hemoglobin (p = 0.013), and platelets (p < 0.001) as well as a higher serum C-reactive protein level (p = 0.039) and a lower serum albumin level (p = 0.004). HPS was observed in 1.5% of adult Korean patients with SLE. The occurrence of HPS was most frequently associated with the SLE disease activity. Profound pancytopenia, a high SLEDAI score, and notable changes in the level of acute-phase reactants can be the characteristics of SLE patients with HPS.

Diagnosis and treatment of hemophagocytic syndrome

Diagnosis and treatment of hemophagocytic syndrome

Traitement du syndrome d’activation macrophagique sévère associé à une leishmaniose viscérale

The association of hemophagocytic syndrome (HS) and visceral leishmaniasis is a frequent disorder during infancy in endemic areas such as Tunisia. The range of severity of HS secondary to visceral leishmaniasis includes both pure biological forms that resolve with antimicrobial therapy and life-threatening emergencies that require specific treatment. We describe 2 cases of severe HS secondary to visceral leishmaniasis. The diagnosis of HS was based on the HLH-2004 diagnostic criteria. Therapy involved pentavalent antimonial (Glucantime) in both cases. The combination of corticosteroids with immunoglobulins, used in the 1st case, but introduced late, led to an unfavorable course and death. In the 2nd case, the specific treatment of HS was based on immunochemotherapy including etoposide and corticosteroids. Progression was favorable with a follow-up of 24 months. Etoposide containing therapeutic regimens can be proposed in severe forms of HS associated with visceral leishmaniasis.

Progress in diagnosis and treatment of Epstein-Barr virus-associated hemophagocytic syndrome

Hemophagocytic syndrome (HS) is a syndrome characterized with complicated etiology and mechanism,the pathological hallmark of this syndrome is unregulated proliferation and activation of histio-cytes and phagocytosis. HS comprises the primary forms and secondary forms,the primary forms are related to the familial genetic defects,and the secondary forms are caused by acquired infections,especially the Epstein-Barr virus ( EBV) infection. EBV-associated HS is a rapidly progressive disease with a high rate of mortality. In this paper,we aim to review the recent progress in diagnosis and treatment of EBV-associated HS. Key words: Epstein-Barr virus; Hemophagocytic lymphohistiocytosis; Diagnosis; Treatment

Meet the expert: microbiology case presentation severe EBV infection in a 22-year-old male

A 22-year-old male with a history of Type 1 diabetes was admitted to a local hospital in March of this year with generalised malaise and headache. He was noted to have a mild transaminitis, and variant lymphocytes in his blood film. A Monospot test performed at the time was negative. A lumbar puncture was also entirely normal, and he was diagnosed with an unspecified viral illness, and discharged. He re-presented to hospital in early August with a history of headache, fever, dyspnoea and abdominal pain. He was found to have diffuse lymphadenopathy, and massive hepatosplenomegaly. He had variant lymphocytes, thrombocytopenia and severe hepatitis. A mononucleosis screen was positive, and his Epstein-Barr virus (EBV) serology was consistent with primary infection. He had multi-organ failure requiring ventilatory support, vasopressors, continuous haemofiltration, and had marked cholestatic liver function tests. Initial plasma EBV DNA PCR demonstrated 710 000 copies/mL. Testing for cytomegalovirus (CMV) and human herpes virus-6 (HHV-6) was negative. After correction of his coagulopathy and thrombocytopenia, a lymph node biopsy was performed; this showed reactive changes consistent with infectious mononucleosis lymphadenitis, and some evidence of haemophagocytosis. After discussion, it was decided that there were no features of lymphoma on the biopsy. Initial genetic testing for X-linked immunodeficiency was negative. A working diagnosis of EBV-associated haemophagocytic syndrome was made, and he was commenced on cyclosporine, etoposide and hydro-cortisone. Over the next fortnight, his EBV levels reduced slightly, he was able to maintain his own ventilation, and his hepatic function improved, although he remained in anuric renal failure, and was still profoundly pancytopenic. He was discharged to the ward, but developed a severe nosocomial pneumonia with a marked deterioration in his hepatic function. His bilirubin level was elevated at over 400 μmol/L, and he developed intractable seizures secondary to encephalopathy. Six weeks into his hospital admission he died. A post-mortem revealed haemophagocytosis in multiple organs, with high EBV levels throughout the body. Further results of testing for additional primary immunodeficiency disorders are pending. This case raised several issues for us: • spectrum of EBV infections and review of previous cases of fulminant EBV infection at our institution; • histological similarities between EBV lymphadenitis and lymphoma; • pathophysiology and treatment of haemophagocytic syndrome; • association of haemophagocytic syndrome with certain infections.

The successful treatment of haemophagocytic syndrome in patients with human immunodeficiency virus-associated multi-centric Castleman's disease

Both virus-associated haemophagocytic syndrome (HPS) and human immunodeficiency virus-associated multi-centric Castleman's disease (HIV-MCD) induced by human herpesvirus-8 (HHV-8) are extremely rare. We therefore wished to investigate their occurrence together, and establish the degree of cytokine activation present. From a prospective cohort of individuals with HIV-MCD, we investigated the incidence and outcomes of HPS and measured 15 inflammatory cytokines and the plasma HHV-8 viral loads before and during follow-up. Of 44 patients with HIV-MCD with an incidence of 4.3/10,000 patient years, four individuals (9%) were diagnosed with HPS. All are in remission (range 6-28 months) following splenectomy, etoposide and rituximab-based therapy. Plasma HHV-8 levels were raised markedly at presentation (median 3,840,000 copies/ml). Histological samples from spleen, splenic hilar lymph nodes and bone marrow demonstrated increased phagocytosis by histiocytes and presence of HHV-8-infected plasmablasts outside the follicles. Surprisingly, many known inflammatory plasma cytokines were not elevated, although interleukin (IL)-8 and interferon-gamma were increased in all cases and IL-6 levels were raised in three of four patients. HPS in the setting of HIV-MCD is common and treatment can be successful provided the diagnosis is made appropriately. Systemic activation of cytokines was limited, suggesting that immunosuppressive therapy with steroids is not indicated in HHV-8-driven HPS.

Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases

Haemophagocytic syndrome (HPS) is known as a relatively rare complication in autoimmune diseases. Here we analysed the clinical features of HPS in patients with systemic autoimmune diseases. One thousand and fourteen patients with systemic autoimmune diseases admitted to Hokkaido University Hospital from 1997 to 2007 were recruited [350 SLE, 136 RA, 98 polymyositis/dermatomyositis (PM/DM), 88 SSc, 91 vasculitis syndrome, 37 primary SS, 26 adult onset Still's disease (AOSD) and 188 other diseases]. Clinical features and treatment outcomes were retrospectively analysed. Thirty cases (3.0%) fulfilled HPS criteria (progressive cytopenia in two or more lineages and haemophagocytosis in reticuloendothelial systems). Underlying diseases were SLE (18), RA (2), PM/DM (2), SSc (2), vasculitis (1), SS (2) and AOSD (3). Nineteen patients were diagnosed as having autoimmune-associated HPS, eight infection-associated, one drug-induced and one developed HPS after haematopoietic stem cell transplantation. For the treatment of HPS, high-dose corticosteroid monotherapy was given in 26 cases, being effective in 12 (46%). Ten out of 15 patients with corticosteroid-resistant autoimmune-associated HPS were treated with CsA, cyclophosphamide or tacrolimus, leading to the remission in 80%. The overall mortality rate was 20%. Multivariate analysis showed that the presence of infections and CRP level >50 mg/l on HPS related with poor prognosis. The prevalence of HPS among in-hospital patients with systemic autoimmunity is not ignorable. Administration of immunosuppressants was effective in cases with autoimmune-associated HPS, whereas prognosis was poor in infection-associated HPS.

Understanding selective trafficking of lymphocyte subsets.

<h3>Objective.</h3> To determine the characteristics of hemophagocytic syndrome (HPS) in adult Korean patients with systemic lupus erythematosus (SLE). <h3>Methods.</h3> We reviewed the medical records of 1033 adult patients with SLE for a recent 14-year period and identified 15 patients who had developed HPS. Forty-two age- and sex-matched patients with SLE admitted for other manifestations were included as disease controls. Features of HPS in these patients were analyzed. <h3>Results.</h3> Reactive HPS occurred from some distinct causes during the course of SLE. HPS was associated with SLE in 11 patients (4 at onset of SLE and 7 at SLE flare), infection in 3 patients (2 bacterial infection; 1 viral infection), and drug use (azathioprine) in 1 patient. Common clinical features included fever (93.3%), hepatomegaly (60.0%), and splenomegaly (60.0%). Steroid pulse therapy (46.7%), immunosuppressants (46.7%), and intravenous immunoglobulin (46.7%) were frequently used for treatment of HPS. One patient (6.7%) died. Compared with SLE patients without HPS, those with HPS showed a higher SLEDAI score (p = 0.003) and lower levels of plasma leukocytes (p &lt; 0.001), hemoglobin (p = 0.013), and platelets (p &lt; 0.001) as well as a higher serum C-reactive protein level (p = 0.039) and a lower serum albumin level (p = 0.004). <h3>Conclusion.</h3> HPS was observed in 1.5% of adult Korean patients with SLE. The occurrence of HPS was most frequently associated with the SLE disease activity. Profound pancytopenia, a high SLEDAI score, and notable changes in the level of acute-phase reactants can be the characteristics of SLE patients with HPS.

Nasal angiocentric lymphoma with hemophagocytic syndrome.

ObjectivesHemophagocytic syndrome(HS) is a fatal complication of nasal angiocentric lymphoma (AL) and difficult to distinguish from malignant histiocyosis. Epstein-Barr virus(EBV)-associated HS is frequently observed in lymphoma of T-cell lineage and EBV is highly associated with nasal AL. Clinicopathologic features of 10 nasal ALs with HS were reviewed to determine the clinical significance and the pathogenetic association with EBV.MethodsTen patients of HS were identified from a retrospective analysis of 42 nasal ALs diagnosed from 1987 to 1996. Immunohistochemical study and in situ hybridization were performed on the paraffin-embedded tumor specimens obtained from 10 patients. Serologic study of EBV-Ab was performed in 3 available patients.ResultsFive patients had HS as initial manifestation, 3 at the time of relapse and 2 during the clinical remission of AL. Four patients were treated by combination chemotherapy (CHOP) and others had only supportive care. The median survival of all patients with HS was 4.1 months (range 2 days–36.5 months) and all had fatal outcome regardless of the treatment-modality. AH cases were positive for UCHL1 (CD45RO) and EBV by EBER in situ hybridization. The data of serologic tests indicated the active EBV infection.ConclusionsHS is a fatal complication of nasal AL and has a high association with EBV. Reactivation of EBV may contribute to HS and further investigation of predictive factors and effective treatment of HS should be pursued in the future.

Hemophagocytic syndrome and T-cell lymphoma after kidney transplantation: a case report

Abstract. Lymphoma in immunocompromised transplant patients is a feared cause of morbidity and mortality. Superimposed on the lymphoma and the transplantation immunosuppression is a rare condition: hemophagocytic syndrome (HS). HS is characterized by fever, hepatosplenomegaly and lymphade-nopathy, skin rashes, jaundice, coagulopathy, and phagocytosis of blood elements with pancytopenia. Here we describe a rare but fatal case of a kidney transplant patient who developed T-cell lymphoma and HS, without evidence of EBV replication. A short review of the diagnosis, treatment, and prognosis of HS is given.