Langerhans cell histiocytosis - Clinical signs

Symptoms cell histiocytosis Langherhans is diverse, it depends on the patient's age and number of affected organs. In the course of the disease may have affected a single organ or system, but there are cases when contained multiple organs and systems resulting in their dysfunction.Multisystem forms is manifested by fever, weight loss, disappearance of appetite, apathy.
In the system ETSO-articular injuries occur in 80% of cases. They can be single or multiple asimpotomatice discovered fortuitously in the conduct of a radiological examination. There are cases in which bone lesions are painful and can be with or without swelling of the soft parts. This is particularly the clinical picture in children older than 5 years. Headquarters bone lesions in the bones is often cap skull and other flat bones: ribs, vertebrae, is also found at the jaw and jaw with the effect of spontaneous loss of teeth.Affected vertebrae undergoes compaction is accompanied by local pain. Appear deformities of the spine, and scoliosis.Chronic or chronic otomastoidita Otoreea is a sign suggestive of temporal rock damage.
X-ray regions to target OSAS, bone lesions lititice appear well defined, with irregular edges.Single bone lesion called granuloma eosinophilia.
A second target organ is the skin disease manifestation. In 50% of cases it occurs in the skin lesions limited to capulu skin (scalp), making a common or seborrheic dermatitis can be extended to the face, trunk, limbs. Lesions consist of a papular eruption-eritemato, purple, covered with scales (exanthema petechiae, bleeding and squamous).In infants, the first manifestation of the disease can be represented by localized lesions cutranate only later evolving multisystemic. Old name of the clinical forms of the disease is the syndrome Letterer-Siwei.
The lympho-ganglionic cervical lymphadenopathy presenting to superficial palpation in 33% of cases, especially in large child.Solitary lesion shape is paote present as cervical or inguinal adenopathy in the region.
On abdominal palpation can highlight the increase in volume of the liver (hepatomegaly) in 40% of cases, especially in multisystem forms.Hepatomegaly is suggestive of general activation of immune system cells, which are locally deteremina Kupffer cell hypertrophy or hyperplasia them.Liver cell dysfunction, present in forms of infants is manifested by multisystem: hyperbilirubinemia, hypoproteinemia, abnormal clotting. And ascites may be associated sometimes. The presence of clinical manifestations at diagnosis adversely affects prognosis.Pacinetul may present an increase in volume of the spleen (hypersplenism). Both splenectomy and spleen irradiation is ineffective which will aggravate treating hypersplenism.
Pulmonary lesions is another manifestation of the disease. They occur in any age group but are more common in multisystem form of infants prcum and 3rd decade of life. The existence of lesions in the lungs will cause the clinical picture that includes: coughing, shortness of breath, chest pain, hemoptysis.Make a lung X-rays will reveal the presence of diffuse micronodular infiltrates in both lung fields, showing interstitial infiltrates. Evolution of lesions over time will wear the appearance of "figure of honey."The bronchoalveolar lavage cells Langherhans stand out. Their mere presence is insufficient to support the diagnosis. When Langherhans CD1a positive cell counts greater than 5% can be suspected diagnosis of cell lymphocytosis Langherhans.
Bone marrow involvement rarely occurs in forms of systemic, multiorgan. Langerhans cells there is usually the bone marrow.Excessive proliferation at this level will cause anemia, thrombocytopenia, pancitopeniei.
Events such as vomiting, diarrhea, slowdown affecting the gastrointestinal tract are suggestive. Ileum damage induced malabsorption and enteropathy installation resulting in loss of protein.
Langerhans cell infiltration of the thymus with nonspecific involution will cause damage, severe dysplasia and changes dismorfice.
A 40% of cases present and affecting the endocrine system. The most common manifestation of the cell histiocytosis Langherhans is diabetes insipidus, occurs frequently in patients with severe bone in the skull.Conversely, any child diagnosed with diabetes insipidus first will have X-ray examination and tomography to rule out lytic bone lesions. By carrying out laboratory investigations, Resonant magnetic (MRI) diagnosis of diabetes insipidus is issued when the hypothalamic-pituitary stem reveals narrowing of more than 2, 5 mm.
Systemic manifestations are present in about 30% of cases, young children and consist of fever, weight stagnation, anorexia, apathy.
Histiocitozelor known classification of Langerhans cell according to the number of organs affected.Langerhans cell histiocytosis, localized form includes:- Skin rash, skin biopsy confirmed, no other locations of the disease;- Monoostotice lesions, with or without diabetes insipidus associated with impaired ganglionic neighborhood rash- Poliostotice lesions affecting multiple bone or lytic lesions more than two to one bone, but without diabetes insipidus.Langerhans cell histiocytosis, extensive form includes:- Visceral, with or without associated bone lesions, diabetes insipidus, efectare ganglonara neighborhood and / or rash, but without signs of organic dysfunction;- Visceral, with or without associated bone lesions, diabetes insipidus, damage to nearby lymph nodes and / or rash, but associated with organic dysfunction of any of the following organs: lungs, liver, hematopoietic system.