Hemophagocytic lymphohistiocytosis (HLH) is a rare cause of cytopenia and is often associated with lymphoma. The occurrence of HLH in the course of lymphoma treatment could be an indicator for refractory disease.

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...

Hemophagocytic lymphohistiocytosis (HLH) or hemophagocytic syndrome is a life threatening hyperinflammatory condition caused by immune dysregulation and resulting in hemophagocytosis and organ damage by activated macrophages and histiocytes. The characteristic clinical features include fever, splenomegaly, cytopenias, liver dysfunction, and hyperferritinemia. HLH can be either primary (genetic)...

INTRODUCTION Systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease is an extremely rare disorder and classically arises following primary acute or chronic active Epstein-Barr virus infection. It is characterized by clonal proliferation of Epstein-Barr virus-infected T-cells with an activated cytotoxic phenotype. This disease has a rapid clinical course and is more fr...

We describe an unusual presentation of fatal infection due to Rhizomucor pusillus bloodstream infection in a 12-year old pediatric patient recently diagnosed with hemophagocytic lymphohistiocytosis. R. pusillus was isolated from one blood culture drawn on Day 11 of hospitalization.

Diagnosis of hemophagocytic syndrome remains a challenge in particular during pregnancy. Concomitant presence of clinical and biological signs, for example, fever, pancytopenia, hyperferritinemia, and hypertriglyceridemia, should alert clinicians to suspect HPS and proceed to prompt treatments.

Missense mutations in genes involved in familial hemophagocytic lymphohistiocytosis can delay the onset of this life-threatening disease. In children and adults, early recognition of aspecific features as neurological symptoms is crucial as urgent treatment is required.

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition marked by uncontrolled histiocyte proliferation and activation, phagocytosis of normal hematopoietic cells. This disease rare, concurrent presentation with acute polymyositis rare. A 14-year-old male was admitted to Chattogram Medical College Hospital high-grade fever for 25 days, generalized severe body aches, multiple large j...

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 ...