Mediterranean lymphoma
| Classification according to ICD-10 | |
|---|---|
| C88.3 | Immunoproliferative disease of the small intestine. Muco-associated lymphoma |
| ICD-10 online (WHO version 2019) | |
As Mediterranean lymphoma a special form of will MALT lymphoma in the small intestine called. MALT lymphoma ( m ucosa- a ssociated l ymphoid t issue ) are malignant lymphomas that their output from the mucous membranes of the internal organs ( stomach , small intestine, colon ) take. The Mediterranean lymphoma is mainly found in the southern Mediterranean and the Middle East . It was first found in Oriental Jews and Arabs.
definition
It is a primarily extranodal non-Hodgkin lymphoma of the B-cell type.
root cause
Mediterranean lymphoma is caused by an α-chain disease (α-HCD), which can therefore be considered a precancerous stage ( precancerous disease ). Infection is assumed to be the cause. Although a large number of infectious pathogens could be detected in the affected patients, it has so far not been possible to identify a specific agent responsible for the disease .
Pathogenesis
The starting tissue of this neoplasm is the lymph tissue of the small intestinal mucosa. It is therefore one of the MALT lymphomas . It mainly affects young people. Lymphoma tends to infiltrate the entire small intestine and beyond.
Immunohistochemistry shows the secretion of IgA with a defective heavy chain. Mediterranean lymphoma and the associated α-chain disease are grouped under the term immunoproliferative disease of the small intestine , English: IPSID - immunoproliferative small intestinal disease, to form a common entity.
Symptoms
Cardinal symptom of the Mediterranean lymphoma is a chronic diarrhea ( diarrhea ), sometimes in combination with fatty stools ( steatorrhoea ). Another typical symptom is abdominal cramps. More rarely, tar and bloody stools (see melena and hematochezia ) based erosive intestinal inflammation. The disease leads to significant swelling of the mesenteric lymph nodes and, in the further course, to a pronounced malabsorption syndrome .
Diagnosis
The diagnosis is confirmed by biopsy with subsequent immunohistochemical processing of the tissue. In the blood and digestive secretions of many patients one finds high levels of abnormally configured IgA (shortened heavy α-chains with in some cases complete absence of the light chains). The origin of the pathological α-chains is not completely clear. The secretion of pathological immunoglobulins is most likely due to plasma cells that have infiltrated into the small intestinal tissue. The laboratory values show a clear increase in the rate of sedimentation of the blood cells .
course
The course of this disease is characterized by alternating phases of aggravation and improvement ( exacerbation and remission ), although overall chronic disease progression can be observed. In the course of the disease, many patients develop pronounced digestive disorders with subsequent tumor cachexia and often fatal outcome.
therapy
In the early stages, the administration of tetracyclines can bring about an improvement in the clinical situation that may last for years, and even freedom from symptoms. Significant healing of the histological lesions can also be observed. In the late stages of the disease, chemotherapy (e.g. CHOP scheme) is the therapeutic approach of choice. Relatively good results have been reported with a combination of antibiosis and chemotherapy .
source
- Manfred Dietel, Norbert Suttorp, Martin Zeitz (eds.): Harrison's internal medicine. 16th edition. Abw Wissenschaftsverlag, Berlin 2005, ISBN 3-936072-29-9 .
