Hemophagocytic lymphohistiocytosis (HLH), is an uncommon, life-threatening hyperinflammatory syndrome caused by severe hypercytokinemia with excessive activation of lymphocytes and macrophages due to a highly stimulated but ineffective immune process. We report a case of Hemophagocytic Lymphohistiocytosis in a 15 year old boy presenting with fever, lymphadenopathy and pancytopenia due to infect...

A patient from Southeast Asia was diagnosed with systemic lupus erythematosus. One year later, she experienced exacerbation of skin lesions and was diagnosed with erythema nodosum leprosum. Upon treatment, the patient developed hemophagocytic lymphohistiocytosis with multi-organ failure and died from invasive fungal infection. Hemophagocytic lymphohistiocytosis has to our knowledge, not previou...

Hemophagocytic lymphohistiocytosis (HLH) is the uncontrolled reaction of the immune system against a triggering pathogen and inability of the immune system to elliminate this triggering factor, which ends up with hypercytokinemia and hemophagocytosis. Hemophagocytic lymphohistiocytosis is classified into two major groups as genetic (primary) and acquired (secondary). The condition has high mort...

Fig. 1. (A–C) Features of the bone marrow (BM) aspirate and biopsy showing hemophagocytic lymphohistiocytosis (HLH) and cytomegalovirus (CMV) staining. (A, B) BM aspirate showing histiocytic hyperplasia and prominent hemophagocytosis by activated histiocytes. Arrowheads indicate features suggestive of ongoing endocytosis (Wright-Giemsa stain, ×1,000). (C) BM core biopsy result showing positive ...

Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here,...

Various manifestations of brain involvement for patients with virus-associated hemophagocytic syndrome have been reported. Here, we report on the sequential magnetic resonance (MR) findings of acute demyelination of the entire brain with subsequent brain atrophy in a follow-up study of a 25-month- old boy who was admitted with fever and then diagnosed with infectious mononucleosis and EBV-assoc...

Hemophagocytic syndrome is a macrophage disorder that may develop as a result of immunological activation, such as that seen in severe infection. It has been described in persons with HIV/AIDS and in those with a variety of HIV-associated opportunistic infections, including those caused by Histoplasma capsulatum. If present, this disorder may portend a poorer prognosis. We describe an HIV-posit...

A 13-yr-old female was admitted to our hospital with fever, seizure, and cervical lymphadenopathy. Laboratory data showed pancytopenia, elevation of serum transaminase, lactate dehydrogenase, triglyceride, and ferritin levels. Lymph node biopsy revealed features of Kikuchi's disease and there were signs of histiocytosis and hemophagocytic phenomenon in bone marrow. She recovered after treatment...

perforin gene (prf1) mutations have been reported in 20-30% of patients with familial hemophagocytic lymphohistiocytosis (fhl), an immune disorder of infancy and early childhood. cytotoxic t and natural killer (nk) cell activities are remarkably reduced or ab-sent in fhl patients. we report the first cases of familial hemophagocytic lymphohistiocy-tosis in an iranian family with two siblings. e...

typically found in darkly pigmented women aged 30 to 39 years. It can also be associated with systemic features including meningismus, hearing changes, and skin manifestations. To our knowledge, this is the first case report of a patient with hemophagocytic syndrome with features resembling ocular Vogt-KoyanagiHarada disease. It is possible that the hyperinflammatory state of hemophagocytic syn...