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      Cytomegalovirus associated haemophagocytic lymphohistiocytosis in the immunocompetent adult managed according to HLH-2004 diagnostic using clinical and sero logical means only

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          Abstract

          Haemophagocytic lymphohistiocytosis (HLH) describes a rare, poorly recognised and under-diagnosed immunopathological syndrome whereby there is a highly stimulated yet ineffective multisystem inflammatory response [1]. I present the first case in English literature of Cytomegalovirus (CMV) associated HLH diagnosed by clinical and serological means, and the fourth case of CMV associated haemophagocytic lymphohistiocytosis in an immunocompetent adult, according to HLH-2004 diagnostic guidelines. I include a literature review of CMV associated HLH in adults and raise awareness of checking serum ferritin in patients who present with a sepsis like syndrome. Additionally, this article discusses the merits of HLH-2004 diagnostic work-up without bone marrow biopsy, using clinical and serological means only. I support the reclassification of HLH alongside the other hyperinflammatory syndromes of SIRS, sepsis, septic shock, and MODS to improve understanding and recognition.

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          Most cited references15

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          Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis.

          Nineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection. High fever, constitutional symptoms, liver function, and coagulation abnormalities and peripheral blood cytopenias were characteristic findings. Hepatosplenomegaly, lymphadenopathy, bilateral pulmonary infiltrates, and skin rash were often present. Fourteen of the patients were immunosuppressed. Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1. The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes. Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs. Thirteen patients recovered. Lymph node biopsy and autopsy material showed generalized histiocytic hyperplasia with hemophagocytosis. The relationship of this disorder to familial hemophagocytic reticulosis, familial erythrophagocytic lymphohistiocytosis, histiocytic medullary reticulosis, and malignant histiocytosis is discussed. Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus-associated syndrome.
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            Viral infections associated with haemophagocytic syndrome

            Abstract Haemophagocytic syndrome (HPS) or haemophagocytic lymphohistiocytosis (HLH) is a rare disease caused by a dysfunction of cytotoxic T cells and NK cells. This T cell/NK cell dysregulation causes an aberrant cytokine release, resulting in proliferation/activation of histiocytes with subsequent haemophagocytosis. Histiocytic infiltration of the reticuloendothelial system results in hepatomegaly, splenomegaly, lymphadenopathy and pancytopenia ultimately leading to multiple organ dysfunctions. Common clinical features include high fevers despite broad spectrum antimicrobials, maculopapular rash, neurological symptoms, coagulopathy and abnormal liver function tests. Haemophagocytic syndrome can be either primary, i.e. due to an underlying genetic defect or secondary, associated with malignancies, autoimmune diseases (also called macrophage activation syndrome) or infections. Infectious triggers are most commonly due to viral infections mainly of the herpes group, with EBV being the most common cause. HPS can be fatal if untreated. Early recognition of the clinical presentation and laboratory abnormalities associated with HPS and prompt initiation of treatment can be life saving. HPS triggered by viral infections generally does not respond to specific antiviral therapy but may be treated with immunosuppressive/immunomodulatory agents and, in refractory cases, with bone marrow transplantation. Copyright © 2010 John Wiley & Sons, Ltd.
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              Hemophagocytic lymphohistiocytosis: a potentially underrecognized association with systemic inflammatory response syndrome, severe sepsis, and septic shock in adults.

              Hemophagocytic lymphohistiocytosis (HLH) was originally described as a genetic disorder of immune regulation, presenting in neonates with protracted fever, hepatosplenomegaly, and cytopenia. A secondary form of HLH, triggered by serious infections, was subsequently described in adults.

                Author and article information

                Journal
                1886
                122234
                European Journal of Microbiology and Immunology
                EuJMI
                Akadémiai Kiadó, co-published with Springer Science+Business Media B.V., Formerly Kluwer Academic Publishers B.V.
                2062-509X
                2062-8633
                1 March 2013
                : 3
                : 1
                : 81-89
                Affiliations
                [ 1 ] Hull and East Yorkshire National Health Service Trust, Castle Hill Hospital, Castle Road, Cottingham, East Yorkshire, HU16 5JQ, UK
                [ 2 ] 5 Montcalm Road, Charlton, SE7 8QG, London, UK
                Author notes
                [* ] (44) 0203 556 7066/(44) 07599565623, margaret.oluk@ 123456gmail.com
                Article
                12
                10.1556/EuJMI.3.2013.1.12
                3832083
                24265923
                6c69e3e2-8072-472c-be1e-e77a7a1fe5e0
                History
                : 3 October 2012
                : 4 October 2012

                Medicine,Immunology,Health & Social care,Microbiology & Virology,Infectious disease & Microbiology
                inflammation,cytomegalovirus,infection associated haemophagocytic lymphohistiocytosis,CMV,HLH,haemophagocytic lymphohistiocytosis,haemophagocytosis,IAHS

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