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LYMPHEDEMA IMMUNODEFICIENT LIMB

Localized Immunodefiency in a Lymphovenous Limb

The Lymphedema Limb - Immunocompromised - Immunodeficient - Cell Mediated Immune Response

Discussion - My opinion

A serious complication of lymphedema is that the affected limb becomes a locale that can be classified as immunosuppressed or immunodeficient.

Definition: Immunosupressed is described as a person whose immune response is not adequate. Localized is that the condition exists in one specific site of the body.

The protein-rich fluid that accumulates in massive amounts in the lymphedema limb is the perfectly ideal medium for the growth of bacteria and the hosting of subsequent bacterial infections.

The lymph system which is the body's front line defense mechanism against bacterial infections is damaged and thus unable to mount an adequate defense once infection occurs. Furthermore, the remaining viable lymphatics are overwhelmed in attempting to respond. It is also over whelmed because the amount of "space" it is attempting to defend (due to the swelling) is often many times that which it is designed to defend and because it is severely impaired due to the damaged lymphatics.. Because of the fibrosis and tissue changes that occur in lymphedema, it becomes increasingly difficult for even antibiotics to attack the invading bacteria.

Therefore, it is imperative that treating doctors, whether they be a PCP or an emergency room physician absolutely understand this concept in the treatment of cellulitis, lymphangitis, erysipelas, impetigo or any other infection in a lymphedema patient.

There must be a different protocol for treating infections in lymphedema patients than in treating infections in "normal" individuals.

Infections in (see: Stages of lymphedema) stage 2 and stage 3 lymphedema patients often require extended periods of antibiotic therapy. This includes necessary IV antibiotic therapy, often for extended periods of time for stage 3 patients.

There is mounting evidence as well that the reason for a person acquiring secondary lymphedema is that they were born with an all ready "at-risk" lymph system.  All that is needed for lymphedema to present is a triggering event.

In conjunction with this, I know of several hereditary lymphedema patients who have been diagnosed with idiopathic leukopenia because it was found their CD4/CD8 levels are severely suppressed,  Generally, this is discovered through blood work used for diagnosing cancers.  It may well be that hereditary patients are also already experiencing a suppressed cell-mediated immune reponse capability.  This would explain to me why so many of us are highly susceptible to chronic or recurrent infections. 

Not understanding this very basic concept will or may kill the patient or cause additional complications from which they are never able to recover from.

* I am not a doctor, but I have lived with lymphedema for 56 years. The above is my opinion.
My experience in addition to the experience of hundreds of other lymphedema patients sharing in the online support groups adds support and weight to my personal opinion on this topic. I welcome any dissenting discussion or reply from the medical community. Just be well prepared in your dissent.*

Pat O'Connor
03/10/04

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Abstracts and Clinical Studies

Lymphedema: an immunologically vulnerable site for development of neoplasms

Ruocco V, Schwartz RA, Ruocco E.

Department of Dermatology, 2nd University of Naples, Naples, Italy.

Abstract 

Lymphedema is the result of accumulation of protein-rich interstitial fluid (lymph stasis) caused by a failure of lymph drainage in the face of a normal capillary filtration. Whether the origin is congenital or acquired from infection, radiation, trauma, or surgery, chronic lymph stasis impairs local immune surveillance by disrupting trafficking of the immunocompetent cells in the lymphedematous district and stimulates vicarious angiogenesis by promoting development of a collateral lymphatic and hematic network in the lymphedematous district. When the local mechanisms of immune surveillance begin to fail, the lymphedematous region becomes an immunologically vulnerable area, predisposed to malignancy, chiefly vascular tumors such as Stewart-Treves syndrome and Kaposi's sarcoma, because of the continual angiogenic stimulus. (J Am Acad Dermatol 2002;47:124-7.)

Elsevier

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Kaposi's sarcoma on a lymphedematous immunocompromised limb.

Int J Dermatol. 1984

Ruocco V, Astarita C, Guerrera V, Lo Schiavo A, Moscariello CG, Satriano RA, Pisani M.

Kaposi's sarcoma appeared on a chronically lymphedematous leg of a 75-year-old man. Immunologic investigations revealed a lack of cellular immune response confined to the involved lower limb. Regional disorders of immune surveillance are thought to play an important role in the early stages of Kaposi's sarcoma.

PubMed

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Evidence for altered cell-mediated immunity in postmastectomy lymphoedema.

Br J Dermatol. 1997 Dec

n E, Powell S, Mortimer P, Ryan TJ.

Department of Dermatology, Churchill Hospital, Oxford, U.K.

Patients with chronic lymphoedema are prone to develop chronic infections and various tumours in the lymphoedematous limb, suggesting that regional immune surveillance is impaired. To test the hypothesis that cutaneous cell-mediated immunity is impaired, 35 women with postmastectomy lymphoedema were investigated using dinitrochlorobenzene to test the afferent and efferent loops of the allergic contact immune response. The results support the role of lymphatics as an important component of the immune response to allergens by the demonstration of impairment of both the afferent and efferent loops of the allergic contact dermatitis reaction, and confirm that there is suppression of immune competence in a lymphoedematous limb.

PMID: 9470909 [PubMed - indexed for MEDLINE]

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Primary cutaneous monomelic B-cell lymphoma

Ann Dermatol Venereol. 1997

Marquart-Elbaz C, Lipsker D, Cribier B, Meyer M, Heid E.

Clinique Dermatologique, des Hôpitaux Universitaires de Strasbourg.

BACKGROUND: Cutaneous B cell lymphomas, especially when appearing as a monomelic papulonodular eruption, are rare. P

PATIENT: Ms H. 87-year-old, consulted for a papulonodular eruption of the left lower limb which developed during the past 5 months. This limb had been the site of a lymphedema since a traumatism 8 years ago. Histopathological analysis and immunostaining of a nodule showed that it was a large cell lymphoma of follicular stem cells. There was no extracutaneous involvement and the patient was successfully treated with radiotherapy. Two months after the completed radiotherapy a cutaneous relapse on the trunk and the upper limbs was treated with cyclophosphamide-vincristine-prednisone chemotherapy. 

DISCUSSION: Lymphedema probably played a role in the genesis of this lymphoma presumably by reducing the local immune response. It may have harmed endothelial cells and maintained an antigenic stimulation leading first to lymphocyte hyperplasia and eventually to a true lymphoma, in the same way this has been proved for some MALT lymphomas.

Massonfr

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Cutaneous diffuse large B-cell lymphoma of the leg associated with chronic lymphedema.

Int J Dermatol. 2008 Feb

González-Vela MC, González-López MA, Val-Bernal JF, Fernández-Llaca H.

Department of Anatomical Pathology, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain.

Development of malignant tumors is a rare but well known complication in chronic lymphedema (CL). We report herein a cutaneous diffuse large B-cell lymphoma of the leg associated with CL. An 89-year-old man presented with multiple cutaneous lesions on his right limb that showed a CL. Dermatological examination disclosed multiple violaceous, firm, slightly infiltrated nodules on the anterior aspect of the leg and the dorsum and sole of the foot. A biopsy of one nodule of the leg disclosed a diffuse large B-cell lymphoma, type of the legs. There was no evidence of lymphadenopathy on computed tomography (CT) scans of the chest, abdomen, and pelvis. A bone marrow aspiration and biopsy showed normal results. The patient was treated with local radiotherapy at a dose of 40 Gy, obtaining a highly significant, almost complete, clinical remission. A literature search identified 11 additional cases of primary cutaneous lymphoma associated with CL. An inadequate lymphatic drainage may make the lymphedematous region an immunologically vulnerable area, predisposing to neoplasia.

Blackwell Synergy

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External Links

The Adaptive Immune System

http://faculty.ccbcmd.edu/courses/bio141/lecguide/unit5/cellular/cmioverview/cmioverview.html

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Inflammation, lymphatic function, and dendritic cell migration.

Lymphat Res Biol. 2006

Mary Ann Liebert Publishers

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Cryptococcal cellulitis in congenital lymphedema.

I1990 Jan-Feb

PubMed

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Cell Mediated Immunity

http://users.rcn.com/jkimball.ma.ultranet/BiologyPages/C/CMI.html

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Cell Mediated Unity Cell Mediated immunityCell Mediated Immunity

http://users.rcn.com/jkimball.ma.ultranet/BiologyPages/C/CMI.html

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Join us as we work for lymphedema patients everywehere:

Advocates for Lymphedema

Dedicated to be an advocacy group for lymphedema patients. Working towards education, legal reform, changing insurance practices, promoting research, reaching for a cure.

http://health.groups.yahoo.com/group/AdvocatesforLymphedema/

Subscribe: AdvocatesforLymphedema-subscribe@yahoogroups.com

Pat O'Connor

Lymphedema People / Advocates for Lymphedema

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For information about Lymphedema

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For information on Infections Associated with Lymphedema

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For information on Lymphedema in Children

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Lymphedema Glossary

http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing

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Lymphedema People - Support Groups

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Children with Lymphedema

The time has come for families, parents, caregivers to have a support group of their own. Support group for parents, families and caregivers of chilren with lymphedema. Sharing information on coping, diagnosis, treatment and prognosis. Sponsored by Lymphedema People.

http://health.groups.yahoo.com/group/childrenwithlymphedema/

Subscribe: childrenwithlymphedema-subscribe@yahoogroups.com

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Lipedema Lipodema Lipoedema

No matter how you spell it, this is another very little understood and totally frustrating conditions out there. This will be a support group for those suffering with lipedema/lipodema. A place for information, sharing experiences, exploring treatment options and coping.

Come join, be a part of the family!

http://health.groups.yahoo.com/group/lipedema_lipodema_lipoedema/?yguid=209645515

Subscribe: lipedema_lipodema_lipoedema-subscribe@yahoogroups.com

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MEN WITH LYMPHEDEMA

If you are a man with lymphedema; a man with a loved one with lymphedema who you are trying to help and understand come join us and discover what it is to be the master instead of the sufferer of lymphedema.

http://health.groups.yahoo.com/group/menwithlymphedema/

Subscribe: menwithlymphedema-subscribe@yahoogroups.com

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All About Lymphangiectasia

Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.

http://health.groups.yahoo.com/group/allaboutlymphangiectasia/

Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com

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Lymphatic Disorders Support Group @ Yahoo Groups

While we have a number of support groups for lymphedema... there is nothing out there for other lymphatic disorders. Because we have one of the most comprehensive information sites on all lymphatic disorders, I thought perhaps, it is time that one be offered.

DISCRIPTION

Information and support for rare and unusual disorders affecting the lymph system. Includes lymphangiomas, lymphatic malformations, telangiectasia, hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of information available through sister site Lymphedema People.

http://health.groups.yahoo.com/group/lymphaticdisorders/

Subscribe: lymphaticdisorders-subscribe@yahoogroups.com

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Lymphedema People New Wiki Pages

Have you seen our new “Wiki” pages yet?  Listed below are just a sample of the more than 140 pages now listed in our Wiki section. We are also working on hundred more.  Come and take a stroll! 

Lymphedema Glossary 

http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing 

Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema 

Arm Lymphedema  

http://www.lymphedemapeople.com/wiki/doku.php?id=arm_lymphedema 

Leg Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=leg_lymphedema 

Acute Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=acute_lymphedema 

The Lymphedema Diet 

http://www.lymphedemapeople.com/wiki/doku.php?id=the_lymphedema_diet 

Exercises for Lymphedema  

http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema 

Diuretics are not for Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=diuretics_are_not_for_lymphedema 

Lymphedema People Online Support Groups 

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_people_online_support_groups 

Lipedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=lipedema 

Treatment 

http://www.lymphedemapeople.com/wiki/doku.php?id=treatment 

Lymphedema and Pain Management 

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pain_management 

Manual Lymphatic Drainage (MLD) and Complex Decongestive Therapy (CDT)

http://www.lymphedemapeople.com/wiki/doku.php?id=manual_lymphatic_drainage_mld_complex_decongestive_therapy_cdt 

Infections Associated with Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema 

How to Treat a Lymphedema Wound 

http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound 

Fungal Infections Associated with Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=fungal_infections_associated_with_lymphedema 

Lymphedema in Children 

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children 

Lymphoscintigraphy 

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphoscintigraphy 

Magnetic Resonance Imaging 

http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging 

Extraperitoneal para-aortic lymph node dissection (EPLND) 

http://www.lymphedemapeople.com/wiki/doku.php?id=extraperitoneal_para-aortic_lymph_node_dissection_eplnd 

Axillary node biopsy 

http://www.lymphedemapeople.com/wiki/doku.php?id=axillary_node_biopsy

Sentinel Node Biopsy 

http://www.lymphedemapeople.com/wiki/doku.php?id=sentinel_node_biopsy

 Small Needle Biopsy - Fine Needle Aspiration 

http://www.lymphedemapeople.com/wiki/doku.php?id=small_needle_biopsy 

Magnetic Resonance Imaging 

http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging 

Lymphedema Gene FOXC2

 http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_foxc2

 Lymphedema Gene VEGFC

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_vegfc

 Lymphedema Gene SOX18

 http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_sox18

 Lymphedema and Pregnancy

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pregnancy

Home page: Lymphedema People

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Page Updated: Dec. 1, 2011