Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This article will introduce a neonate with HLH in Iran. We report a case of HLH presenting with respiratory distress and fever, hepatosplenomegaly, jaundice and pancytopenia on the second day of life....

Background Langerhans cell histiocytosis (LCH) is a rare disease in which monoclonal migration and proliferation of specific dendritic cells is seen. The disease primarily affects the bones and skin, but there is a possibility that involves other organs or appears as a multi-systemic disease. Case Report In oral examination of a nine-month girl, two deep wounds with a yellow membrane with appro...

In this issue of Blood, Hervier et al has identified that cooccurrence of Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) in the same patient is not a rare event. Mixed histiocytosis (MH) highlights existence of a link between distinct groups of histiocytic disorders and suggests presence of a common progenitor cell. Today, histiocytic disorders are classified into 3 group...

Langerhans cell histiocytosis (LCH) is a rare disorder characterised by increased production of Langerhans-type histiocytes. It more common in the pediatric age group with predilection for osseous involvement, though any organ may be involved. A 10-year male child was brought to neurosurgical clinic slow growing painful tender mass on head. Initial attempt biopsy lesion failed due excessive ble...

Members of four generations of a family had a defect of serum opsonization for yeast phagocytosis consistent with dominant inheritance. 2 were healthy, one had chronic osteomyelitis, and the fourth developed a fatal illness in infancy characterized by exfoliative dermatitis, diarrhoea, multiple bacterial infections, and failure to thrive, which resembled the two prevously reported cases with th...

We reviewed clinical and pathologic characteristics of 18 cases of malignant histiocytosis including 2 autopsy cases with special interest on their immunohistochemical characteristics. We report 3 cases of unusual immunohistochemical finding and postulate these cases may be a supportive evidence of the view that dendritic cells share common precursor with those of mononuclear phagocytic system....

Smoking-induced diffuse interstitial lung processes include respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease (RBILD), desquamative interstitial pneumonia (DIP) and Langerhans’ cell histiocytosis. The histological, radiological and clinical features of respiratory bronchiolitis, RBILD and DIP are reviewed, with particular reference to management issues; L...

Histiocytosis represents a group of rare disorders characterized by nonneoplastic proliferation of histiocytes and solitary cutaneous reticulohistiocytosis (SCR) is a form of non-Langerhans histiocytosis. SCR consists of small papule, usually single, varying in color from yellow to brownish-red, more frequent in young adults, and uncommon in childhood. We report a case of SCR in an 11-year-old ...