OVERVIEW
OVERVIEW
Sinus histiocytosis with giant lymph node disease is a rare self-limiting disorder of acquired histiocytosis with giant lymph nodes.
Insured or not
Yes
Department of Medicine
Dermatology, Hematology
Synonyms
Rosai-Dorfman disease
Clinical Symptoms
Painless progressive superficial lymph node enlargement.
Hazards
Half of the patients may recover on their own, while a few may have a poor prognosis or even die due to the involvement of systemic organs.
Complications
Multiple organ damage.
Examination
Blood routine, blood sedimentation, polyclonal hyperimmunoglobulin test, ultrasonography of lymph nodes, histopathological examination of lymph nodes or skin.
Diagnosis
Diagnosis is made on the basis of the clinical features of painless progressive superficial lymph node enlargement, the features of skin lesions, as well as laboratory tests and pathologic examinations.
Treatment principle
Most patients can be cured on their own, and a few should be treated symptomatically with drugs or surgery.
Curability
The disease is self-limiting, but there are a few severe patients with poor prognosis.
Dietary advice
Eat more iron containing foods and foods rich in vitamin C.
Causes
Epidemiology
It can occur at any age, commonly between 10 and 20 years of age, the cutaneous form is common between 40 and 60 years of age, and is slightly more common in males than in females.
Etiology
The cause is unknown and may be related to human herpesvirus type 6 infection.
Symptoms and Diagnosis
Typical symptoms
1. Painless progressive enlargement of superficial lymph nodes, mainly in the cervical lymph nodes, followed by axillary and inguinal lymph nodes. 2. Accompanied by fever, anemia and weight loss. 3. 10% of patients have skin lesions, which may appear as yellowish-brown papules, pustules, or nodules, or erythematous spots or larger circular lesions. 4. It can also be accompanied by lesions of the upper respiratory tract, bone, genitourinary system, salivary glands, eyes and liver.
Diagnostic basis
1. Clinical manifestations: involvement of superficial lymph nodes and/or extra-nodal tissues, painless and progressive enlargement of lymph nodes; 2. Pathological examination: dilatation of lymphatic sinuses, infiltration of histiocytes within the sinuses, phagocytosis of intact lymphocytes; pathology of skin lesions shows phagocytosis of intact lymphocytes or plasma cells by histiocytes.3. Positive histiocyte S-100 protein, positive acid phosphatase, positive nonspecific esterases, and the absence of Birbeck granules.
Treatment
Treatment guidelines
Most patients can be cured by themselves, and a few should be treated with drug anti-inflammation to relieve symptoms or surgical excision.
Drug therapy
Glucocorticoids are applied to patients with autoimmune hemolytic anemia. Cytotoxic drugs, the efficacy is not certain.
Surgical treatment
Surgery is the most effective method for patients with limited lesions and compression symptoms that can be removed by surgery.
Prognosis
The disease is self-limiting, and there are a few patients with severe patients with poor prognosis.
Nursing care
Dietary management
Adequate nutrition, more food containing iron, more vegetables and fruits, etc.