Infections associated with haemophagocytic syndrome

Rouphael, Nadine; Talati, Naasha J; Vaughan, Camille P.; Cunningham, Kyle W.; Moreira, Roger K.; Gould, Carolyn V.

doi:10.1016/s1473-3099(07)70290-6

Cited by 512 publications

(527 citation statements)

References 69 publications

(61 reference statements)

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“…[2][3][4][5] The central pathogenesis involves dysregulated Th1 cytokine secretion. This results in an uncontrolled accumulation of activated T-lymphocytes and histiocytes in various organs including the liver, spleen and bone marrow.…”

Section: Discussionmentioning

confidence: 99%

“…6 Viruses are the most common infectious triggers of HLH, particularly EBV, and lymphoma is the most common associated malignancy. [2][3][4][5] It is hypothesized that EBV can interfere with normal lymphocyte signaling pathways leading to the aforementioned over-expression of Th1 cytokines, which can then trigger HLH. 7 The diagnosis of HLH is based on a combination of clinical and laboratory parameters as outlined in Table 1.…”

Section: Discussionmentioning

confidence: 99%

“…The loss of immune function likely allowed the reactivation of Epstein-Barr virus which triggered HLH and his fulminant presentation. [3][4][5]9 Indeed, both the liver and bone marrow samples showed evidence of EBV reactivation as evidenced by the presence of EBER. The diagnosis of Hodgkin lymphoma was made from a liver biopsy specimen rather than bone marrow examination.…”

Section: Discussionmentioning

confidence: 99%

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Elements of confusion

Chakrabarti

Booth

Detsky

et al. 2010

Journal of Hospital Medicine

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The approach to clinical conundrums by an expert clinician is revealed through presentation of an actual patient's case in an approach typical of morning report. Similar to patient care, sequential pieces of information are provided to the clinician who is unfamiliar with the case. The focus is on the thought processes of both the clinical team caring for the patient and the discussant. A 23-year-old man presented to his family physician's office with a 2-week history of fever, chills, night sweats, anorexia, and fatigue. This was associated with a 4-month history of a nonproductive cough and a 20-pound involuntary weight loss. He denied shortness of breath, chest pain, headaches, abdominal pain, vomiting, diarrhea, dysuria, and rash. There was no recent travel, sick contacts, or animal exposures. This patient's symptoms could represent an underlying infectious, neoplastic, or inflammatory process. I would ascertain any relevant personal or family history and explore whether the patient has risk factors for human immunodeficiency virus (HIV) infection or tuberculosis (TB). On physical examination, I would listen for a heart murmur and look for lymphadenopathy, hepatosplenomegaly, and arthritis. Investigations including cultures, urinalysis, and a chest radiograph would be indicated at this time.During the 2 weeks after his initial presentation, he experienced persistent fever, and further weight loss. He was admitted to the hospital to determine the etiology of his symptoms. The patient had no previous medical problems. On initial examination, his temperature was 102 degrees Fahrenheit, blood pressure was 100/65 mmHg, heart rate was 105 per minute, respiratory rate was 22 breaths per minute and oxygen saturation was normal on ambient air. He appeared cachectic. He was oriented to person, place, and time. Head and neck examination revealed no intraoral pathology, lymphadenopathy or scleral icterus, but did reveal conjunctival pallor. The chest was clear to auscultation, and the cardiovascular examination revealed a normal apical impulse and heart sounds with no murmurs. There was peripheral edema to the level of the mid-shins bilaterally. The abdomen was soft and non-tender with no appreciable hepatosplenomegaly. There were no stigmata of chronic liver disease. There was no axillary or inguinal lymphadenopathy. The remainder of the examination was normal. A complete blood count showed a hemoglobin concentration of 5.2 g/dL with a mean corpuscular volume (MCV) of 89fL, white blood cells were 1,400 cells/ mm 3 with an absolute neutrophil count (ANC) of 800 cells/mm 3 and a platelet count of 90,000 cells/mm 3 The serum sodium was 124 mmol/L, The patient is clearly very unwell and requires admission to the hospital for treatment and further investigation. Emergent management includes administration of intravenous fluids to correct his electrolyte abnormalities, empiric broad spectrum antibiotics (given his relative neutropenia and fever), and a transfusion for his profound anemia. I would be very concerned that he ha...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Elements of confusion

Chakrabarti

Booth

Detsky

et al. 2010

Journal of Hospital Medicine

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“…Another form of the disease occurs secondary to conditions like infections, malignancies, autoimmune diseases or immunosuppression [4]. Most common infectious etiologies include EBV among virus and VL among parasites [5]. The HLH syndrome, which is similar in both forms, is diagnosed as per HLH-2004 [6] guidelines that includes eight criteria-fever, splenomegaly, cytopenia of at least two cell lines, hypofibrinogenemia or hypertriglyceridemia, demonstrable hemophagocytosis, ferritin level [500 lg/l, low/absent NK cell activity and soluble CD25 [2400 U/ml.…”

Section: Casementioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis Associated with Visceral Leishmaniasis: Varied Presentation

Ranjan

Kumar

Ganguly

et al. 2015

Indian J Hematol Blood Transfus

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Visceral leishmaniasis (VL) is endemic in many parts of India. Rarely, it may be complicated by hemophagocytic lymphohistiocytosis (HLH) that has varied presentation and course. We describe two cases of VL complicated by HLH that were markedly different in clinical presentation, course and management. First case presented with Fever of unknown origin whereas second case had fever with severe bleeding manifestations. VL was diagnosed by bone marrow aspiration and serum rk39 immunodiagnostic test respectively in these cases. HLH was diagnosed by HLH 2004 diagnostic criteria. VL was treated by intravenous amphotericin B in both cases. HLH was managed by treating primary disease in the first case whereas steroid was given for management in the second case. High index of suspicion is crucial for early diagnosis of HLH to reduce morbidity and mortality.

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“…This uncontrolled and inappropriate immune response leads to hypersecretion of proinflammatory cytokines and uncontrolled hemophagocytosis throughout the reticuloendothelial system. 2 Diagnosis of HLH relies on specific clinical, laboratory, and histopathological findings, proposed by the Histiocyte Society in 1991 and updated in 2004. 3 The diagnosis can be established if five of the eight criteria are fulfilled, namely, clinical criteria (fever for more than 7 days and splenomegaly), laboratory criteria (bicytopenia without marrow hypoplasia, hypertriglyceridemia, and/or hypofibrinemia, hyperferritinemia, low/absent NK cell activity, increased soluble CD25 levels), and histological criteria (hemophagocytosis which can be seen in any organ, but is particularly common in bone marrow, lymph nodes, liver, and spleen).…”

Section: To the Editormentioning

confidence: 99%