Download Email Template - National Lymphedema Network

Newsletter Article Reprint
Volume 11, No. 2
April–June 1999
Lymphedema Through The Ages
Steven I. Hajdu, M.D.
This short summary of the history of lymphedema is written
for those who are interested in where we were, how we got here,
where we are and where we might be going.
The first recorded cases of lymphedema date back to well
before Biblical time. Celsus (30 B.C.–50 A.D.) was the first to
use the term “elephantiasis” for lymphedema. Others such as
Haley Ben Abbas (930– 994) and Avicenna (980–1037) used the
same term and set the tone for its usage for the next thousand
years. During the middle ages, very little happened. It is true, as
somebody once said, that the history of medicine (including, of
course, the history of lymphedema) in the middle ages is
immensely boring.
The cause of lymphedema was not known, but its endemic
occurrence in Asia, Africa and Egypt was noted and by 1874 in
Dunglison's Medical Dictionary published in Philadelphia,
"elephantiasis" was listed according to geographic types such as
Indian, Italian, Greek, Arabian et.al.
Leprosy-associated lymphedema was known for ages. It is
believed that leprosy was brought to the Mediterranean area by
soldiers of Alexander the Great returning from India in 326 B.C.
Although its contagious nature was first recognized by a monk,
Anglicus, in 1246, identification of the organism, a bacillus that
causes it, had to wait for another 600 years. According to the
World Health Organization, there are over 15 million lepers
worldwide and, at a certain point and time, all develop
lymphedema.
Another condition, filariasis, an endemic disease in India,
Southern Asia, Egypt and South America commonly producing
lymphedema, was suspected as an infectious disease from the
time it was first described in the Ebers Papyrus in 1550 B.C. But
it was Patrick Mason, a Scottish physician who, while working in
Taiwan, demonstrated in 1877 that the lymphedema was caused
by embryos of a parasite (Filaria bancrofti) transmitted by
mosquitos. An estimated 90 million people are infected
throughout the world and most of them develop lymphedema of
the extremities.
.
Other conditions reported to induce development of
lymphedema are: breast cancer (Horne, 1805 and Druitt, 1850);
Milroy disease, a congenital hereditary lymphedema (Milroy,
1892 — a native of New York City and Professor of Medicine
and Hygiene at Omaha Medical College); terminal liver cancer
in children (Keating, 1890); trauma (Secretan, 1901); axillary
dissection (Halsted, 1921 — a pioneer in the late 1800s of
radical operation for breast cancer [mastectomy with axillary
node dissection] at Johns Hopkins in Baltimore); neurofibromatosis (Abt, 1923); Turner syndrome, so called "prune babies"
(1960) as the result of arrest in the development of the jugular
and iliac lymphatic sacs during embryonic life; cat scratch
disease (Filler, 1964); and Maffucci syndrome (Kerr, 1991).
Lymphedema was suspected as a precursor of cancer for
centuries. Although Pigray is credited with the first description
of a lymphedema-associated cancer in 1649, reports of malignant
transformations were scarce prior to the paper on
lymphangiosarcoma in a congenital lymphedematous leg written
by Kettle in 1918. I added a further case to the literature in 1971.
Kaposi's sarcoma was described in a chronically
lymphedematous extremity the first time by Hansson in 1940.
But the landmark paper was on malignant transformation of
lymphedematous tissues to lymphangiosarcoma in six patients,
written in 1948 by my teacher Dr. Stewart in collaboration with
Dr. Treves from the Memorial Sloan-Kettering Cancer Center.
Report of cutaneous malignant lymphoma secondary to
lymphedema was published by Waxman in 1984 and I reported
with my colleagues two cases of lymphangiosarcoma following
filarial infection associated lymphedema in 1981 and in 1987.
The classification of lymphedemas by Telford and Simmons
in 1938 as noted below set the ground for present day attempts in
both prevention and treatment.
ETIOLOGIC CLASSIFICATION OF LYMPHEDEMAS
A. LYMPHEDEMA CAUSED BY CONGENITAL OR GENETIC
DEFECTS
B. LYMPHEDEMA CAUSED BY TRAUMA OR DISEASES
C. LYMPHEDEMA CAUSED BY TREATMENT OF DISEASES
There were many attempts to treat lymphedema through the
ages, but, most often, a do-nothing, wait-and-see approach
prevailed. The opinion of some of the most eminent physicians
was expressed by Samuel Gross of Philadelphia in his book on
surgery in 1866, "the treatment of elephantiasis (lymphedema)
is, at best, very unsatisfactory." By the late 1800s, application of
leeches, scarification, punctures of the skin and bloodletting
were abandoned, but it was not until the turn of the century that
new therapeutic modalities such as keeping the edematous
extremity in an elevated position, well-bandaged and
mechanically compressed were put into practice. As a last resort,
if everything failed, ligation of arteries and amputation of limbs
were accepted therapeutic measures and are practiced to present
day.
No lasting progress can be made in prevention and
treatment of lymphedema without a clear-cut understanding of
the patho-mechanism of its development. Very promising results
were obtained over the last 20 years through the use of complex
physical therapy, including manual lymph drainage, compression
and exercises.
As to prevention of surgically induced lymphedema, some
progress has been made during recent years, but very little is
known about the prevention of malignant transformation of
lymphedematous tissues. However, we know that tissues altered
by chronic lymphedema are immunologically privileged,
immunodeficient sites and constantly must be protected against
trauma, infections and ulcerations, because local tissue changes
may initiate a series of cellular alterations that may lead to the
appearance of lymphangiosarcoma, an affliction for which there
is no effective therapy at the present time.
Dr. Steven Hajdu is Chief of the Division of Surgical Pathology and
Cytopathology, and Professor of Pathology, North Shore University
Hospital, Manhasset, New York. Dr. Hajdu is a member of the NLN
Medical Advisory Committee.
National Lymphedema Network • Latham Square • 1611 Telegraph Avenue • Suite 1111 • Oakland, CA 94612-2138 • Tel: 800-541-3259 •
Fax: 510-208-3110