Hemophagocytic syndrome associated with cytomegalovirus infection in a severely immunocompromised AIDS patient: case report

Nogueira, Marcus V.F.; Vidal, Laira; Terra, Bruno; Pagot, Thiago; Salluh, Jorge I.; Soares, Márcio

doi:10.1590/s1413-86702009000100016

Cited by 8 publications

(15 citation statements)

References 7 publications

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“…Familial HLH has higher prevalence in infants and children where genetic mutations play a role in the pathogenesis [ 6 , 10 - 12 , 17 , 18 ]. Secondary HLH is seen in adults more frequently where autoimmune disorders [ 1 , 8 ], malignancies [ 4 , 19 , 20 ], infectious agents including bacteria [ 21 , 22 ], viruses [ 23 , 24 ], and immunosuppression [ 7 ] are involved in the pathogenesis. Macrophage activation syndrome (MAS) is a variant of HLH especially when it occurs in association with autoimmune disorders [ 1 , 2 ].…”

Section: Discussionmentioning

confidence: 99%

“…This syndrome has been reported frequently in infants and children compared to adults [ 4 - 6 ]. Infectious agents, autoimmune disorders and malignancies have been associated with HLH in adults [ 7 , 8 ] whereas genetic mutations play a major role in infants and children [ 9 - 12 ]. Cutaneous T-cell lymphomas in the setting of latent Epstein-Barr virus (EBV) infection have been reported in the literature [ 13 , 14 ].…”

Section: Introductionmentioning

confidence: 99%

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Hemophagocytic Lymphohistiocytosis in Association With Clostridium difficile Infection and Cutaneous T-Cell Lymphoma

et al. 2011

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Hemophagocytic lymphohistiocytosis (HLH) or Macrophage Activation Syndrome (MAS) is a potentially life threatening disorder that presents with fever, suppressed blood cell counts, hepatosplenomegaly and multi-organ failure. HLH has been reported in association with genetic mutations, infections, autoimmune disorders, and various malignancies. However to the best of our knowledge, HLH in association with Clostridium difficile infection has never been reported. We present a case of HLH in a patient with Epstein-Barr virus (EBV) positive natural killer T (NKT) cell cutaneous lymphoma and active Clostridium difficile infection. A 35-year-old male with recently diagnosed EBV positive NKT cell lymphoma was admitted for Clostridium difficile associated diarrhea. During the course of hospitalization he gradually developed pancytopenia and multi-organ failure leading to death. Post-mortem examination confirmed the diagnosis of hemophagocytic lymphohistiocytosis.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Association With Clostridium difficile Infection and Cutaneous T-Cell Lymphoma

et al. 2011

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“…The former is more common [26] and includes cytopenias caused by post-autoimmune hepatitis cirrhosis and autoimmune lymphoproliferative syndrome [29,30]. The latter, also known as basic immune thrombocytopenia, is an acquired immune disease common in chronic lymphocytic leukemia [31,32], variable immunodeficiency disease [33,34], and hemophagocytic syndrome [35], presenting with abnormal bleeding due to decreased platelet counts. Thrombocytopenia due to immunodeficiency occurs in cases of increased antibody-mediated platelet destruction coupled with decompensation due to bone marrow defect [36].…”

Section: Immunodeficiencymentioning

confidence: 99%

Non-Hypersplenism Causes of Peripheral Cytopenias in Patients with Cirrhotic Portal Hypertension: A Review

Lv¹,

Lau²,

Deng³

et al. 2016

J Hypertens (Los Angel)

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Hypersplenism and non-hypersplenism factors, either alone or in combination, can cause peripheral cytopenias in patients with cirrhotic portal hypertension. Although non-hypersplenism factors account for only a small proportion of patients, they do exist. When peripheral cytopenias do not improve, or fail to improve adquately, or even become worse after splenectomy in these patients, non-hypersplenism factors should be considered. This review aims to provide an overview of non-hypersplenism factors.

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“…The Hemophagocytic lymphohistiocytosis syndrome (HLH) was first described by Farquhar and Claireaux in 1979 in immunosuppressed patients with viral infections [1,2,3].…”

Section: Introductionmentioning

confidence: 99%

Hemophagocytic Syndrome in Patients Living with HIV

2019

Journal of HIV/AIDS & Infectious Diseases

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Hemophagocytic syndrome is a clinical condition characterized by non-malignant proliferation and activation of lymphocytes and macrophages (histiocytes); hemophagocytosis in the reticuloendothelial system; and the clinical findings of fever, hepatosplenomegaly, cytopenias, and hyperferritinemia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening and rapidly progressive condition caused by excess activation of the immune system. This syndrome is classified as familial or acquired; the latter is more frequent and is associated with diverse conditions, such as infections, malignancies, and rheumatic diseases. HLH associated with human immunodeficiency virus (HIV) is rare, most often being described in those with chronic HIV infection or with the presence of concomitant opportunistic infections. In immunocompromised patients, HLH is an aggressive condition and a fulminant syndrome with high rates of mortality. Patients presenting with a clinical picture of fever and highly elevated inflammatory markers should raise the suspicion for HLH. Treatment usually requires chemotherapy and even with standard treatments prognosis is poor with approximately 50% survival. Rapid diagnosis and treatment of the underlying disorder is necessary to prevent progression to multiorgan failure and death.

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Hemophagocytic syndrome associated with cytomegalovirus infection in a severely immunocompromised AIDS patient: case report

Cited by 8 publications

References 7 publications

Hemophagocytic Lymphohistiocytosis in Association With Clostridium difficile Infection and Cutaneous T-Cell Lymphoma

Hemophagocytic Lymphohistiocytosis in Association With Clostridium difficile Infection and Cutaneous T-Cell Lymphoma

Non-Hypersplenism Causes of Peripheral Cytopenias in Patients with Cirrhotic Portal Hypertension: A Review

Hemophagocytic Syndrome in Patients Living with HIV

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