Go Letterer — Ziva disease (E. Letterer, S. Siwe)

Letterera — Ziva disease

Letterera — Ziva disease (E. Letterer, S. Siwe; synonym: aleukemic reticulosis, non-lipoid reticulohistiocytosis, reticuloendotheliosis, histiocytosis) is a systemic disease with the formation of foci of growth of reticular cells in internal organs, bones, skin, mucous membranes.

The etiology of the disease is not clear; it is believed that Letterer — Ziva disease should be considered as a neoplasm; others tend to believe that Letterer's disease — Ziva has an infectious, possibly viral, origin. A number of authors refer to Letterer's disease — Ziva, in contrast to Gaucher disease (see) and Niemann - Pick disease (see), to reticuloendotheliosis without disturbing lipoid metabolism and finds common features between Letterer — Ziva disease, Henda - Schüller - It is characterized by a disease ( see Hyundai - Schüller –Crischen disease) and eosinophilic granuloma bone (see Eosinophilic granuloma). These diseases can go one into another; more often, local processes become disseminated with damage to internal organs. On the basis of this, Liechtenstein [L. Lichtenstein (L. Lichten-stein)] combines these three diseases into one nosological form - histiocytosis X (see Histiocytosis). Letterer's disease — Ziva was considered to be unique only to young children, but in recent years it has also been described in adults.


Diffuse reticular proliferation in the lymph node in Letterer's disease - Ziva in a girl 2.5 months.

Pathological anatomy . The essence of Letterer — Ziva disease lies in the pathological proliferation of reticular cells of a diffuse or nodular character (Fig.), In contrast to reticuloleukemia, without washing them into peripheral blood. Hence the name - aleukemic reticulosis. Macroscopically very characteristic papular-hemorrhagic rash on the skin with necrosis of the elements of the rash. Lymph nodes are enlarged; on a section have a motley look. In the thymus, especially in infants, there are tumor-like bluish-red growths with a significant increase in organ weight. The spleen is enlarged, fleshy, with blackish areas of hemorrhage. Liver of clay type (fatty infiltration), moderately increased. In the lungs, there is either a diffuse seal of the type of interstitial pneumonia or granulomatous nodules; on the cut, they reveal air cavities (honeycomb type) resulting from the destruction of the lung carcass by proliferating reticular cells. In the long tubular and flat bones (often in the bones of the skull) lesions are seen in the form of cysts with pink-whitish tumor-like growths on the periphery. Endocrine glands, the lymphatic apparatus of the gastrointestinal tract, and soft tissues of the gums may also be affected. Histologically - the growth of reticular large light cells and cells of histiocytic type, there are giant cells like Langkhans, sometimes fields of eosinophils. In cases of transition to the Hend-Schüller-Crysten disease, the cytoplasm of the reticular cells takes on a foamy appearance. Between reticular cells, upon impregnation with silver, a network of argyrophil fibers is detected. Violent proliferation of reticular cells in the organs is accompanied by circulatory disorders with the formation of hemorrhages and necrosis. In the lymph nodes, the presence of erythrophagy is quite typical.

The clinical picture (symptoms and signs). The initial signs of the disease are manifested by general weakness, pallor, loss of appetite, headaches and fever, which can be subfebrile for a long time, over a period of several weeks, but sometimes increases to 38-39 °. Often, with Letterer's disease — Ziva, skin lesions are noted in the form of a papular-spotted rash and hemorrhage. A characteristic feature is a significant increase in the spleen, liver and lymph nodes. Gradually, progressive anemia develops, leukocytosis appears, as in septic diseases. In addition, bone damage is characteristic of Letterer – Ziva disease, accompanied by moderate tenderness and swelling of soft tissues at the site of the lesion; both tubular and flat bones are affected. An X-ray examination may show oval or irregular shapes and various sizes of well-defined bone defects. The prognosis is unfavorable, especially in young children, in whom the disease is acute and in a few weeks or months ends with a fatal outcome.

Treatment . Restorative means - blood transfusion, plasma, glucose, vitamin therapy. Hormones (corticosteroids) are also used in combination with antibiotics (broad-spectrum), general strengthening means and radiation therapy. Such a combined treatment causes remission of varying duration (up to 6 years).