Sinus histiocytosis - Roza-Dorfman's disease
Sinus histiocytosis among non-lensegan-type histiocytosis is one of the most rare diseases. The same disease is called the disease of Roza-Dorfman, after the names of the doctors who first described him in 1969.
This is a benign disease from the mononuclear blood cells of the phagocytic mononuclear system. It is characterized by accumulation of connective tissue cells - proliferating histiocytes. Accumulation occurs mainly in the lymph nodes, as a result of which a characteristic increase in the size of the node is observed.
Classification of sinus histiocytosis
The disease is classified as atypical cell disorders of the lymph nodes. In this case, atypia manifests itself in an abnormal increase in the concentration of proliferating histiocytes due to the expressed phagocytosis( absorption) of lymphoid cells by them.
The clinical picture is characterized by the predominance of enlargement of the cervical lymph nodes. Less common is the increase in lymphoid tissue of the nasopharynx. Bones or skin are even less likely to be affected.
The descriptions of the disease are very controversial due to its rarity. Specific histiocytic markers in this case are indeterminate, there are no unified clear methods for determining clonality. In addition, there are a number of close histological and clinical conditions of a tumor and reactive infectious disease. All this greatly complicates the diagnosis of the disease.
Etiology and course of the disease
In more than 50% of cases, oncological diseases are subsequently detected in patients with sinus histiocytosis. The aetiology of Rosa-Dorfman's disease is unknown. Although histological and clinical manifestations of the disease are similar to infectious data, with sinus histiocytosis, microorganisms have not yet been identified.
The disease predominantly proceeds continuously-recurrently. Pathohistologically, the course of the disease is manifested by proliferation of macrophages, signs of phagocytosis of plasma cells are revealed. Violation of the immune response is an important feature in the pathogenesis of the disease.
Diagnosis
To diagnose the disease, a diagnostic biopsy of the lymph nodes is performed, which allows to detect characteristic macrophages, foamy protoplasm, vacuized nuclei, giant lymphocytes and cells. The diagnosis is based on observations of the clinical picture, histological studies and laboratory tests.
Differential analysis is performed with lymphoma, other histiocytosis, atypical microbacterial infections, xanthomas, sarcoidosis.
Skin lesion is observed in the order of 7-10% of cases without affection of the lymph nodes. The dermal form appears in the form of a rash from a variety of red or yellow-brown papules or nodes up to 3- 4 in diameter. There is almost no lesion of the mucous membranes.
Treatment methods for sinus histiocytosis
The only clear treatment for sinus histiocytosis is not currently developed. The prognosis for patients is favorable in most cases, therefore the expectant management with active monitoring of the patient's condition is most often justified. In the presence of autoimmune complications, the use of corticosteroids is indicated. When an aggressive form of the disease occurs, treatment with combinations of alkylating chemotherapy and corticosteroids is used, but due to the extremely small number of observations, the effect of such treatment has been little studied.
Previously, chemotherapy with various effects of cyclophosphamide, vincristine, prednisol, and radiation therapy methods was used to treat Roza-Dorfman's disease. At the moment, the effect of the use of interferon preparations - reaferon and alphaferon in the amount of 1-3 million IU intramuscularly is being studied. The course is continued for 3-4 weeks, then repeated courses are applied.
There are a few cases of recovery of children after the use of 6merkapturin and methotrexate.
Achievements were recorded on the background of taking thalidomide remission of the cutaneous form of sinus histiocytosis.