hemophagocytic lymphohistiocytosis (hlh) is one of the complications of epstein-barr virus (ebv) infection. although the patients who have developed hlh following ebv have normal immune system, there are a few patients with ebv-induced immune deficiency who develop hlh as well. here, we describe the case of a 10-year-old girl with neurological complications caused by ebv-induced hlh. the patien...

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The evaluation of hematologic disorders after solid organ transplantation (SOT) must take into account issues unique to the post-transplant setting that influence the development of anemia and single or multi-lineage cytopenias. Attention to the time of onset of cytopenia(s) is important, because the disorders of passenger lymphocyte syndrome, transplant-related thrombotic microangiopathy, hemo...

BACKGROUND Hemophagocytic lymphohistiocytosis is an aggressive life-threatening syndrome of excessive immune activation. Hemophagocytic lymphohistiocytosis due to systemic lupus erythematosus is described as acute lupus hemophagocytic syndrome. Acute lupus hemophagocytic syndrome presenting with negative antinuclear antibody is uncommon. CASE PRESENTATION A 57-year-old Sri Lankan woman presen...

Autoimmune lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of apoptosis. It usually presents with chronic lymphadenopathy, splenomegaly, and symptomatic cytopenia in a child. Herein, we report a 14-year-old boy with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis who was treated before ALPS was diagnosed for the patient. This case should alert pediatricians to consi...

Acquired hemophagocytic lymphohistiocitosis (HLH) syndrome can be a complication of visceral leishmaniasis (VL). A multicenter prospective study was conducted to determine the frequency of HLH syndrome in children with VL. Twenty-four children with VL were identified, and 10 (41%) developed HLH syndrome. VL should be ruled out in all children with HLH criteria living in or coming from endemic a...

Macrophage activation syndrome is a form of secondary hemophagocytic lymphohistiocytosis in the setting autoimmune diseases. It’s rare, hyperinflammatory complication requiring prompt institution therapy to prevent organ dysfunction and death. We report young female presenting with macrophage multiorgan failure diffuse alveolar hemorrhage inaugural systemic lupus erythematosus. The patient was ...

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potential life-threatening clinical syndrome that results from uncontrolled activation of the immune system. Secondary HLH, more commonly observed in adult patients, is seen in the context of underlying triggering conditions. Epstein-Barr virus (EBV) has been recognized as the leading infectious cause and is associated with a poor outcome. ...

Background: Patients with Coronavirus Disease 2019 (COVID-19) frequently experience a hyperinflammatory syndrome leading to unfavorable outcomes. This condition resembles Secondary Hemophagocytic Lymphohistiocytosis (sHLH) described in neoplastic, rheumatic and other infectious diseases. A scoring system (HScore) that evaluates underlying immunosuppression, temperature, organomegaly, cytopenias...

OBJECTIVE To describe the clinical and laboratory presentation of hemophagocytic lymphohistiocytosis in children treated at a referral institution. METHODS A retrospective descriptive study was carried out of seven children diagnosed with hemophagocytic lymphohistiocytosis between 2010 and 2012. The criteria for diagnosis were those proposed by the Histiocyte Society. When indicated, immunoch...