Abstracts LymphForsch 2/2014

Classification and therapy of square toes in patients with chronic Lymphedema

G. Felmerer, J. Ernst, T. Bürger, A. Stepniewski, T. Aung
LymphForsch 2014; 18(2):60-65

The term „square toes“ is used to describe toes with box-like deformities of the dorsal skin. In reversible lymphedema stages, conservative treatment is indicated. Glove-like compressiongarments can provide long-term improvement of the condition. In irreversible stages, surgery provides cosmetically acceptable and functional results. A classification should be used toadapt treatment to obtain optimal results. Conservative treatment is indicated in reversible conditions (stage I). Advanced cases with irreversible changes can be treated either by gullwing door flaps (stage II, mostly square shaped, not fragmented) or by tangential excision (stage III, strongly fragmented and the interspaces filled by detritus). Histology shows tissue changes with massive enlargement of fibrous tissue, dilated lymphatic vessels and accumulation of plasma cells as a sign of sterile inflammation. In our study of 21 patients presented in this article, the lymphedema symptoms could be improved after treatment adapted according to the classification. Our results showed that surgery appears to be more appropriate as the standard treatment for more progressed cases of square toes.

Keywords: Lymphedema, elephatiasis, classification, Stemmer‘s sign, surgery, gull-wing door flap

Update lipedema 2014: Cologne lipedema study

M. E. Cornely, M. Gensior
LymphForsch 2014; 18(2):66-71

In many respects, lipedema of the arms and legs is still an underresearched disease within the lymphatic spectrum. It is clear that clinical symptoms frequently include symmetrical fat distribution in the arms and legs and pathognomonic tenderness in female family members. However, 75 years after the first descriptions provided by Allen and Hines, we still lack pathological evidence that would provide more insight than that offered by the theses proposed by Marsch and Brauer. We also lack information about hormonal influence on hyperplastic fatty tissue and the causes of obviously increased lymph formation in the fatty tissue in patients with lipohyperplasia dolorosa. Much more is known about the effects of combined decongestive therapy, which has been used since the 1960s. Moreover, since 1997, surgery has been used to successfully treat this disease presentation. The success rate in long-term observation (15 years) is 97%.

Keywords: Lipedema of arms and legs, lipohyperplasia dolorosa, lymphological liposculpture, sex hormones, Cologne Lipedema Study

Induction of blood and lymphatic vessels by endothelial progenitor cells and mesenchymal stem cells in the mouse

K. Buttler, G. Gross, J. Wilting, H. A. Weich
LymphForsch 2014; 18(2):72-77

Many therapeutic approaches are based on tissue reconstruction. Cell-based therapies for peripheral vascular diseases may be very helpful in the future. Specific interactions between mesenchymal stem cells (MSCs) and endothelial progenitor cells (EPCs) may provide such an option. To investigate the development of new blood and lymphatic vessels, we isolated MSCs from bone marrow and EPCs from the murine lung. Both cell types were co-injected subcutaneously in adult mice. In our studies we used immunocompetent mice in a syngeneic model. We first demonstrated de novo formation of both types of vessels. While the transplanted EPCs integrated into the endothelium of both blood and lymphatic vessels, grafted MSCs usually did not incorporate directly into the vessel wall. We then investigated the origin, quality and quantity of proteins, which are essential for vessel formation. Whereas vascular endothelial growth factor-A (VEGF-A) was secreted by MSCs in significant amounts, we did
not find lymphangiogenic growth factors in these cells. We assume that the neo-formation of lymphatics is induced by growth factors secreted from leukocytes, which were found in close association to the lymphatic vessels. Our studies demonstrate the general feasibility of cellular therapeutic approaches for neo-formation of blood and lymphatic vessels.

Keywords: endothelial progenitor cells, mesenchymal stem cells, blood and lymphatic vessels, lymphangiogenesis, immunocompetent mouse model

An exciting chapter in the history of medicine: Gerhard Domagk and the fight against tuberculosis

M. Hundeiker
LymphForsch 2014; 18(2):78-80

The dramatic history of the first tuberculostatic drugs appearing at the end of World War II is presented. Seventy years ago, tuberculosis vanished from Central Europe in a matter of years. Now, the disease is threatening to return with multiresistant bacteria from Eastern Europe, Asia and Africa. We need new drugs to fight this old disease. Except in coincidence with HIV, the incidence of clinically silent latent tuberculosis infections has not been studied; however, we do know that susceptibility to tuberculosis and the probability of contracting the disease is influenced by both environmental and genetic factors, as well as by other diseases.

Keywords: Gerhard Domagk, tuberculosis, chemotherapy, history

Gorham-Stout disease with lymphatic malformations and generalized lymphatic anomaly – a review

J. Rößler
LymphForsch 2014; 18(2):81-85

The new ISSVA (International Society for the Study of Vascular Anomalies) classification of vascular anomalies attempts to make a clear distinction between Gorham-Stout disease with lymphatic malformations and generalized lymphatic anomaly. However, it is still difficult to define the differences between the two diseases. In both types of vascular malformation, an increase of lymphatic vessels in the skeleton, the soft tissue and parenchymatous organs can be observed. In this review, the clinical picture as well as diagnostic measures for Gorham-Stout disease and generalized lymphatic anomaly are presented. Due to the rare nature of these lymphatic malformations, standard therapies are not available. Recent treatment strategies such as the use of the mTOR inhibitor rapamycin are discussed.

Keywords: vascular malformations, lymphatic malformations, Gorham-Stout disease, generalized lymphatic anomaly, rapamycin