Histiocytosis X is a rare disorder with no particular predilection for race, age or sex. Since its discovery by Hand in 1893, the etiology has remained unknown, although viruses, bacteria and genetic factors have been implicated. Familial occurrence of this disease is very rare, and only a handful of such cases have been reported. The present study adds further evidence to support the influence...

A case of histiocytosis X involving the thyroid and hypothalamus is reported. A 16 year old female presented with amenorrhoea and diabetes insipidus. She subsequently developed a painful goitre with biochemical hypothyroidism, and stridor. The stridor and goitre responded to cyclophosphamide. Previous publications on the use of cytotoxics in histiocytosis X involving the thyroid are reviewed. W...

Langerhans’ cell histiocytosis may cause irreversible respiratory failure due to progressive destruction of lung parenchyma and widespread cystic change. Transplantation oVers a therapeutic option. A case is described of recurrence of Langerhans’ cell histiocytosis which was associated with deterioration in lung function four years following bilateral lung transplantation. Patients transplanted...

Fibreoptic broncho-alveolar lavage was used in four patients; the diagnosis of histiocytosis X had been established by lung biopsy in three and was suggested on clinical grounds in the remaining patient. Characteristic cells with an ultrastructural cytoplasmic marker (X body) were found in the washes of all four patients. In the patient without biopsy confirmation, the findings in the broncho-a...

Histiocytic syndromes in children are divided into 3 classes: (i) Langerhans-cell histiocytosis (LCH) or Histiocytosis-X (HX), (ii) Hemophagocytic syndromes—Familial erythrophagocytic lymphohistiocytosis (FEL) and infection-associated hemophagocytic syndrome (IAHS) due to viruses (VAHS), and occasionally bacterial, fungal or parasitic, and (iii) Malignant histiocytic disorders(l). The lesional ...