Long-term survival of patients with acute differentiated histocytosis (letterersiwe disease)

Abstract

A case is reported in which a patient with the typical clinical and pathologic features of Letterer‐Siwe disease survived more than 15 years from the onset of symptoms. A search of the literature revealed only four other instances of similarly long survivals. An attempt to correlate clinical features and treatment data with length of survival in 96 adequately documented cases collected from the literature showed few clear‐cut trends. Lack of pulmonary involvement and the absence of thrombocytopenia seem to be of favorable prognostic significance. Of the patients 18.9% survived 2 years or longer; all but one of these were still alive at the time of the last follow‐up. Survivals of 2 years or longer were significantly higher in patients who had received corticosteroids than in those who had not. The number of reported long‐term survivals is too small to permit one to detect a common denominator that would make it possible to predict such a favorable clinical course.

Letterer-Siwe disease in a young adult

“…So much is this so that Doede & Rappaport (1967), in a review of ninety-six adequately documented cases of this disease in world literature, made an age of less than 3 years one of their diagnostic criteria. So much is this so that Doede & Rappaport (1967), in a review of ninety-six adequately documented cases of this disease in world literature, made an age of less than 3 years one of their diagnostic criteria.…”

“…Letterer-Siwe disease in its classical presentation is seen almost exclusively in children. So much is this so that Doede & Rappaport (1967), in a review of ninety-six adequately documented cases of this disease in world literature, made an age of less than 3 years one of their diagnostic criteria.…”

Letterer-Siwe disease: a study of thirteen cases over a 21-year period

“…Four children have survived; (16 years, 7 years, 5 years and 9 months since diagnosis) and two of these had pulmonary involvement. DISCUSSION We were able to find only one large study of Letterer-Siwe disease alone which did not embrace the other forms of histiocytosis X (Doede & Rappaport, 1967). In this, they reported one long term survivor of 15 years, and reviewed ninety-six other cases of Letterer-Siwe disease.…”

“…However, Lahey (1975b) and Komp et al (1977) support the concept that organ dysfunction is more significant prognostically and is independent of age. Doede & Rappaport (1967) found pulmonary involvement to be a poor prognostic sign. In our study, seven of the eight children presenting with pulmonary infiltration have died.…”

Letterer-Siwe disease in the adult

“…However, the disease is not always fatal. In one series 177% of ninety-six patients were well 3 years after the onset ofthe disease (Doede & Rappaport, 1967) and in another seven of eighteen patients with seven or more organs involved survived (Lahey, 1975).…”