Letterer-Ziv disease

Letterer-Ziv disease (E. Letterer, S. Siwe; synonym: leikemicakie retikulez, nelipovichis reticulohistiocytosis, reticuloendotheliosis, Langerhans) is a systemic disease with the formation of pockets of growth reticular cells in the internal organs, bones, skin, mucous membranes.
The etiology of the disease is not clear; there is an opinion that Letterer-Ziv disease should be considered as a new formation; others tend to assume that L-3. B. has the infection, can be of viral origin. A number of authors considers L-3. B., unlike Gaucher disease (see) and Niemann - pick disease (see), to reticuloendotheliosis without disturbance of lipoid metabolism and finds similarities between Letterer-Ziv disease, Hand - shyullera - Christian disease (see Hand - shyullera - Christian disease) and eosinophilic granuloma bones (see Eosinophilic granuloma). These diseases can move from one to the other; often local processes will be disseminated with damage to internal organs. Based on this Liechtenstein [Lichtenstein (L. Lichten-stein)] brings together these three diseases in one nosological form - histiocytosis X (see Langerhans). L-3. B. it was considered inherent exclusively to young children, but in recent years are described In adults.

Diffuse reticular proliferation in the lymph node when illness Letterer - Ziv the girl 2.5 months.

Pathological anatomy. The essence Letterer-Ziv disease is abnormal proliferation reticular cells or diffuse nodular character (Fig.), unlike reticulocytosis, without washing them in the peripheral blood. Hence the name - leikemicakie retikulez. Macroscopically very typical papular-hemorrhagic skin rash with necrosis of rash elements. Lymph nodes are enlarged; on a cut have a mottled appearance. In the thymus gland, especially infants, are observed granulomas bluish-red growth with a significant increase in body weight. The spleen is enlarged, fleshy, with blackish areas of hemorrhage. Liver clay type (fat infiltration), moderately increased. In light note or diffuse seal on the type of interstitial pneumonia, or granulomatous nodules; on the cut in them detect cavities (kind of honeycombs), resulting from the destruction of the framework proliferating light reticular cells. In the long tubular and flat bones (often in the bones of the skull) are visible lesions in the form of cysts with a pink-white-like growths on the periphery. May be affected and the endocrine glands, lymph apparatus, gastrointestinal tract, soft tissue of the gums. Histologically - proliferation reticular large light cells gistiotitarnaya type, there are giant cell type Langhans, sometimes the fields of eosinophils. In cases of conversion to the disease Hand - shyullera - Christian cytoplasm reticular cells becomes frothy. Between reticular cells during the impregnation silver detected network argyrophilic fibers. The rapid proliferation reticular cells in the organs accompanied by disorders of blood circulation with the formation of hemorrhage and necrosis. In the lymph nodes is quite typical is the presence of erythropygia.
The clinical picture. The initial symptoms of the disease are shown weakness, paleness, loss of appetite, headaches and fever, which is a long time in a few weeks, may be low grade, but sometimes it rises to 38 - 39 degrees. Often when L - 3. B. there are skin lesions in the form papular-spotted rash and hemorrhage. Characteristic feature is the significant increase in the spleen, liver and lymph nodes. Gradually develops progressive anemia, appears leukocytosis, as in septic diseases. In addition, for the disease Letterer-Ziv characterized by loss of bone accompanied by moderate pain, and swelling of the soft tissues in the place of the hearth; affects both tubular and flat bones. X-ray examination can be observed oval or irregular shape and sizes well defined bone defects. The prognosis is poor, especially in children of early age at which the disease is flowing urgently and in a few weeks or months lethal outcome.
Treatment. Tonic - transfusion, plasma glucose, vitamin therapy. Also used hormonal drugs (corticosteroids) in combination with antibiotics (broad spectrum), restorative means and radiation therapy. This combination treatment is remission of different duration (up to 6 years).