Hypereosinophilic syndrome

Classification according to ICD-10
D47.5	Chronic eosinophilic leukemia [hypereosinophilic syndrome]
D72.1	Eosinophilia
ICD-10 online (WHO version 2019)

The hypereosinophilic syndrome (HES) is a special form of hypereosinophilia and refers to a group of rare diseases (multi-system disease) defined by a pronounced, more than 6 months existing eosinophilia in peripheral blood of more than 1.5 x 10 ⁹ / L (or Bone marrow eosinophilia with no demonstrable cause) and significant organ dysfunction in eosinophilic tissue infiltration .

Synonyms are: hypereosinophilic syndrome; Eosinophilic leukemoid; Eosinophilic reticulosis; English Idiopathic hypereosinophilic syndrome

distribution

The incidence is 1 - 9 indicated to 100,000 men are in the ratio of 9 to 1 much more frequently affected than women, the disease usually occurs between 20 and 50 years of age on.

causes

→ Main article : "Causes" in the article Hypereosinophilia

The causes are just as diverse as the clinical manifestations, as already expressed in the term 'syndrome'. Acquired genetic disorders are the cause in some of the cases. In the so-called secondary forms, it is assumed that the symptoms are cytokine-mediated, e.g. B. suspected by increased production of interleukin IL-5 by a clonally expanded population of T lymphocytes (CD3-CD4 + phenotype).

Classification

The following forms can be distinguished:

• Idiopathic HES
• Myeloproliferative HES
• Lymphocytic HES
• Familiar HES
• HES overlap syndrome (eosinophilic esophagitis , eosinophilic pneumonia , hypereosinophilic dermatitis ).

Clinical manifestations

The extent of organ changes as a result of eosinophilia can vary widely. In over 50% the skin , heart , lungs and nervous system are involved. Hepato- and / or splenomegaly , eosinophilic gastroenteritis and coagulation disorders can occur. Clinical criteria are:

• General symptoms such as weight loss , fever , loss of appetite
• various skin conditions
• Heart involvement in 60%, most common cause of death
• Pulmonary infiltrates with cough , eosinophilic pleural effusions
• neurological changes, cerebral thromboembolism , peripheral neuropathies
• gastrointestinal involvement with abdominal complaints and hepatosplenomegaly
• Muscle weakness

diagnosis

The HES is a diagnosis of exclusion. Must be:

• Blood eosinophilia> 1500 / μl; for more than 6 months
• To rule out other causes of eosinophilia
• Evidence of organ involvement causing complaints.

Differential diagnosis

Other reasons for eosinophilia such as allergies , parasitism and neoplasias , malignant tumors or leukemia , eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) or HTLV infection must be excluded from the differential diagnosis .

therapy

The therapeutic care depends on the severity and cause of the disease. In the case of the genetic, acquired forms, very effective tyrosine kinase inhibitors can be given, in the case of the others, corticosteroids or hydroxycarbamide , interferon-alpha first . Recent evidence suggests that mepolizumab, an anti-IL-5 antibody, is an effective corticosteroid sparing agent for F / P negative cases. Good results are also described under PUVA therapy. Oral anticoagulation is recommended to prevent embolism .

history

The first description comes from 1919 by HZ Griffin .

literature

• Otto Braun-Falco, Helmut Heinrich Wolff: Dermatology and Venerology 2005, ISBN 978-3-540-40525-2 , 539

Individual evidence

1. ↑ ^{a b c d e} Orphanet Hypereosinophilic Syndrome
2. ↑ ^{a b c d e f g h} Online encyclopedia Dermatology
3. ↑ Hypereosinophilic syndrome, idiopathic, resistant to imatinib.  In: Online Mendelian Inheritance in Man . (English)
4. ↑ HZ Griffin: Persistent eosinophilia with hyperleuko-cytosis and splenomegaly. In: American Journal of Medicine , Vol. 158, 1919, 618-629

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !