LCH A congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker histiocytosis) is characterized by predominantly cutaneous involvement and spontaneous regression. Letterer-Siwe Disease is an acute disseminated form, which typically presents with hepatosplenomegaly, fever, thrombocytopenia and anemia. This is the form that this patient appears to have. A more chronic process is Hand-Schuller-Christian Disease, characterized by the classic triad of diabetes insipidus, exophthalmus and destructive skull lesions. Isolated Langerhans cell histiocytosis is termed eosinophilic granulomas when it involves bone or the lung. In the latter setting, this disease is associated with smoking. Importantly, the prognosis for Langerhans cell lesions is dependent on the clinical stage, not the histopathology. The presence of necrosis, atypia, and mitotic figures do not by themselves make Langerhans cell histiocytoses malignant. Adverse prognostic factors include disease onset at less than two years of age, and increasing number of involved dysfunctional organs.
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