Saturday, October 17, 2009

Arm and Leg Swelling After Malt Lymphoma

Arm and Leg Swelling After Malt Lymphoma

With the advent of better and more effective cancer treatments, the survival rate for all cancers has risen dramatically. With this progress, a new and often misunderstood and misdiagnosed complication has arisen.

Many cancer survivors , having overcome cancer, find themselves with sudden and often unexplained swelling, usually of the arms or of the legs.

This swelling occurs because of one of several factors.

First, the swelling begins after lymph nodes have been removedfor cancer biopsies.

Second, the swelling may start as a result of radiation damage to either the lymph nodes and/or the lymph system.

Due to either the removal of lymph nodes or damage to the lymph system, your body is no longer able to rid itself of excess fluids. The fluids collect in the limbs effected and swelling begins.

This swelling is called lymphedema and it can effect either your leg or your arm. The swelling that occurs is permanent, and while it is not curable it is treatable.

Hopefully, in the future with radiological scans becoming more sensitive and with increased use of such techniques such as the small needle biopsy, we can put an end to this epidemic of secondary lymphedema from cancer biopsy.

Permanent Leg or Arm Swelling

In the situation of any permanent leg swelling whether the cause is known or unknown, the diagnoses of lymphedema must be considered

There are several groups of people who experience leg or arm swelling from known causes, but it doesn't go away or unknown causes where the swelling can actually get worse as time goes by.

Group One

This group includes those who have had the injuries, infections, insect bites, trauma to the leg, surgeries or reaction to a medication. When this swelling does not go away, and becomes permanent it is called secondary lymphedema.

Group Two

Another extremely large group that experiences permanent leg or arm swelling are cancer patients, people who are morbidly obese, or those with the condition called lepedema. What causes the swelling to remain permanent is that the lymph system has been so damaged that it can no longer operate normally in removing the body's waste fluid.

In cancer patients this is the result of either removal of the lymph nodes for cancer biopsy, radiation damage to the lymph system, or damage from tumor/cancer surgeries.

This is also referred to as secondary lymphedema.

Group Three

Group three consists of people who have leg or arm swelling from seemingly unknown reasons. There may be no injury, no cancer, no trauma, but for some reason the leg simply is swollen all the time.

The swelling may start at birth, it may begin at puberty, or may begin in the 3rd, 4th or even 5th decade of life or sometimes later.

This type of leg or arm swelling is called primary lymphedema. It can be caused by a genetic defect, malformation or damage to the lymph system while in the womb or at birth or be part of another birth condition that also effects the lymph system.

This is an extremely serious medical condition that must be diagnosed early, and treated quickly so as to avoid painful, debilitating and even life threatening complications. Treatment should NOT include the use of diuretics.

WHAT IS LYMPHEDEMA?

Lymphedema is defined simply as an accumulation of excessive protein rich fluid in the tissues of the leg. The accumulation of fluid causes the permanent swelling caused by a defective lymph system.

A conservative estimate is that there may be 1-2 million people in the United States with some form of primary lymphedema and two to three million with secondary lymphedema.

WHAT ARE THE SYMPTOMS OF LYMPHEDEMA?

If you are an at risk person for arm lymphedema there are early warning signs you should be aware of. If you experience any or several of these symptoms, you should immediately make your physician aware of them.

1.) Unexplained aching, hurting or pain in the arm or leg

2.) Experiencing “fleeting lymphedema.” This is where the limb may swell, even slightly, then return to normal. This may be a precursor to full blown arm lymphedema or leg lymphedema.

3.) Localized swelling of any area. Sometimes lymphedema may start as swelling in one area, for example the hand, or between the elbow and hand. This is an indication of early lymphatic malfunction.

4.) Any arm inflammation, redness or infection.

5.) You may experience a feeling of tightness, heaviness or weakness of the arm.

The symptoms for leg lymphedema are very similar to ones for the arm.

HOW IS LYMPHEDEMA TREATED?

The preferred treatment today is decongestive therapy. The forms of therapy are complete decongestive therapy (CDT) or manual decongestive therapy (MLD), there are variances, but most involve these two type of treatment.

It is a form of massage therapy where the leg is very gently massaged to actually move the fluid out of the leg and into an area where the lymph system still functions normally.

With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down and/or is taught to use compression wraps to maintain the leg size.

WHAT ARE SOME OF THE COMPLICATIONS OF LYMPHEDEMA?

1. Infections such as cellulitis, lymphangitis, erysipelas. This is due not only to the large accumulation of fluid, but it is well documented that lymphodemous limbs are localized immunocompromised.

2. Draining wounds that leak lymphorrhea which is very caustic to surrounding skin tissue and acts as a port of entry for infections.

3. Increased pain as a result of the compression of nerves usually caused by the development of fibrosis and increased build up of fluids.

4. Loss of Function due to the swelling and limb changes.

5. Depression - Psychological coping as a result of the disfigurement and debilitating effect of lymphedema.

6. Deep venous thrombosis again as a result of the pressure of the swelling and fibrosis against the vascular system. Also, can happen as a result of cellulitis, lymphangitis and infections.

7. Sepsis, Gangrene are possibilities as a result of the infections.

8. Possible amputation of the limb.

9. Pleural effusions may result if the lymphatics in the abdomen or chest are to overwhelmed to clear the lung cavity of fluids.

10. Skin complications such as splitting, plaques, susceptibility to fungus and bacterial infections.

11. Chronic localized inflammations.

CAN LYMPHEDEMA BE CURED?

No, at the present time there is no cure for lymphedema. But it can be treated and managed and most of the complications can be avoided. Life with lymphedema can still be active and full, with proper treatment, patient education, and patient life style adaptation.

For extensive information on lymphedema, please visit our home page:

Lymphedema People

Sunday, October 11, 2009

Zevalin Active for Treatment of Extranodal Marginal-zone Lymphoma

Zevalin Active for Treatment of Extranodal Marginal-zone Lymphoma


Researchers from Italy have reported that patients with relapsed or refractory extranodal marginal-zone lymphoma have a high response rate following treatment with Zevalin® (90Yttrium-ibritumomab tiuxetan). The details of this study were presented at the Joint ECCO 15 – 34th ESMO Multidisciplinary Congress in Berlin, September 20-24, 2009.

Marginal zone lymphomas are a relatively uncommon type of B-cell non-Hodgkin’s lymphoma (NHL), comprising approximately 2-4% of all cases. There are about 61,000 new cases of NHL diagnosed annually, of which 1,000-2,000 are marginal zone. Some of these marginal zone lymphomas are in extranodal sites including the spleen (splenic marginal lymphomas). Marginal zone lymphomas also fall into the category of mucosal associated lymphoid tissue (MALT lymphomas). MALT lymphomas are frequently associated with gastric Helicobacter Pylori infection. Marginal-zone lymphomas are included in the “low grade” category, and treatments are similar to patients with chronic lymphocytic leukemia (CLL) and follicular lymphoma. This generally means Rituxan® (rituximab)-based therapy.

Zevalin is a radioimmunotherapeutic agent currently approved in the United States for the treatment of patients with relapsed or refractory, low-grade, or follicular B-cell NHL, including those refractory to Rituxan® (rituximab). Zevalin is also indicated, under accelerated approval, for the treatment of relapsed or refractory, Rituxan-naive, low-grade and follicular NHL.
The current study involved 24 patients with relapsed or refractory marginal-zone lymphoma that was present in extranodal sites and treated with Zevalin between 2004 and 2009. Ten of the 24 cases had Helicobacter Pylori-negative gastric MALT NHL, and 14 had extra-gastric marginal-zone lymphoma. Twenty-one of these 24 patients had disseminated disease. Twenty-one patients were evaluable for response, and the median follow-up was 22 months:
  • 76% had a complete response.
  • Duration of remission was three to 59 months, with six of 16 patients remaining in remission for greater than three years.
  • Survival was 100%.
These authors concluded: “The high rate of complete remission (76%) and the duration of response are clearly superior to those observed with conventional systemic approaches [CT, monoclonal antibody alone or in combination with CT].”

Comments: These and other studies suggest that Zevalin is one of the most effective agents available for the treatment of B-cell NHL of any type, but especially low-grade NHL.

Reference:
[1] Vanazzi A, Pruneri G, Crosta C, et al. Efficacy of 90Yttrium-ibritumomab tiuxetan in extranodal marginal-zone lymphoma. European Journal of Cancer Supplements, Vol 7 No 2, September 2009, page 562, abstract O-9207.

Tuesday, September 29, 2009

MALT lymphoma of the floor of the mouth: a case report.

MALT lymphoma of the floor of the mouth: a case report.


MALT (mucosa-associated lymphoid tissue) lymphomas are low-grade extranodal B-cell lymphomas that may involve various sites in the head and neck including the thyroid, salivary, and lacrimal glands. Development of MALT lymphoma in the head and neck is often associated with autoimmune diseases such as Sjögren syndrome or Hashimoto thyroiditis. Here we report a case of a MALT lymphoma of the floor of the mouth that likely arose in the sublingual gland. The patient was successfully treated with external-beam radiation therapy and remained disease-free at the 4-year follow-up.

Sunday, September 20, 2009

Systemic lupus erythematosus in a patient with primary MALT lymphoma of the larynx.

Systemic lupus erythematosus in a patient with primary MALT lymphoma of the larynx.
Ear Nose Throat J. 2009 Aug

Gonzàlez N, Xicoy B, Olivé A, Jové J, Ribera JM, Feliu E.
Clinical Hematology Department, Institut Català d'Oncologia, Hospital Germans Trias i Pujol, Badalona, Universitat Autònoma de Barcelona, Spain.


Primary lymphomas of the larynx are extremely rare. Even more unusual is their association with rheumatologic diseases. Some cases of lymphoma have developed after chemotherapy for rheumatologic diseases, suggesting that the treatment may contribute to the development of lymphoma. The converse can also occur-the development of rheumatologic disease after a primary laryngeal lymphoma-but that is even rarer. We report a case of primary extranodal marginal zone B-cell MALT (mucosa-associated lymphoid tissue)lymphoma of the larynx in a 42-year-old woman treated with involved-field radiotherapy who developed systemic lupus erythematosus (SLE) 1 year after the lymphoma diagnosis. Currently, the patient remains in remission 8 years after her diagnosis. The SLE, for which she is receiving maintenance therapy, is stable.

PMID: 19688705 [PubMed - in process]

Thursday, September 17, 2009

The gastric MALT lymphoma. An unforeseen model for studying the pathogenesis of the limphomas

The gastric MALT lymphoma. An unforeseen model for studying the pathogenesis of the lymphomas

Recenti Prog Med. 2009 Jun

Savio A.
Unità Funzionale di Istopatologia, Laboratorio di Patologia Clinica ed Istocitopatologia, Ospedale S. Orsola Fatebenefratelli, Brescia.
antonellasavio@bresciaonline.it

MALT lymphoma is a low-grade lymphoma originating from the mucosa associated lymphoid tissue. It is the third most frequent non-Hodgkin B-cell lymphomas. One third of the cases is primary gastric. The causal association between Helicobacter pylori infection and gastric MALT lymphoma is proved by numerous clinical and experimental studies. The most dramatic evidence supporting a pathogenetic role for H. pylori in gastric MALT lymphoma is remission of the tumour following eradication with antibiotic therapy. However, the evolution of a H. pylori infection towards lymphoma is an exceptional event which takes place probably due to the concurrence of host, environmental and bacterial strain factors. Gastric MALT lymphoma is currently the only malignant neoplasia that can be cured by a simple antibiotic therapy. In early stage cases the Helicobacter pylori eradication is the first line therapy as it obtains stable remissions in most of the cases. Surgical treatment of this disease has been completely abandoned. The role of chemio and radiotherapy is limited to the few cases non-responders to the antibiotic therapy. This paper offers a wide and updated review of the literature about gastric MALT lymphoma. Practical points for the clinical management are also given, in keeping with the European Guidelines that will be shortly published.

PubMed

Primary Low-grade and High-grade Gastric MALT-lymphoma Presentation: A Systematic Review.

Primary Low-grade and High-grade Gastric MALT-lymphoma Presentation: A Systematic Review.
J Clin Gastroenterol. 2009 Sep 9

Zullo A, Hassan C, Andriani A, Cristofari F, Cardinale V, Spinelli GP, Tomao S, Morini S.
*Gastroenterology and Digestive Endoscopy daggerHaematology, "Nuovo Regina Margherita" Hospital double daggerDepartment of Experimental Medicine, "La Sapienza" University, Rome, Italy.


GOALS: To assess the clinical and endoscopic presentation of primary gastric lymphoma.

BACKGROUND: Remission rate and long-term survival in patients with gastric lymphoma mainly depend on disease stage at diagnosis. Series reporting clinical and endoscopic presentation of gastric lymphoma are generally small and heterogeneous.

STUDY: Systematic review with pooled-data analysis assessing clinical and endoscopic presentation of primary gastric lymphoma.

RESULTS: Data regarding 2000 patients were collected. Overall, males were slightly more prevalent, alarm symptoms were absent in near half of the patients, lymphoma was diagnosed in a stage less then I in one-third of the patients, and Helicobacter pylori infection was present in 88.8% of considered patients. At endoscopy, the ulcerative type was the most frequent presentation, although low-grade lymphoma was diagnosed on normal/hyperemic gastric mucosa in 9% of cases. Patients with high-grade lymphoma presented alarm symptoms (anemia and/or melena and/or hemorrhage, persistent vomiting, weight loss), an exophytic or ulcerative lesion, a stage III-IV, and a H. pylori negative status more frequently than low-grade lymphoma cases.

CONCLUSIONS: Our pooled-data analysis showed that gastric lymphoma is still disappointingly diagnosed in an advanced stage in a large number of patients. This is probably due to presence of nonspecific symptoms at initial clinical presentation and/or a normal appearing mucosa at endoscopic observation in the early stages.


Lippincott, Williams & Wilkins

Monday, June 8, 2009

Primary gastric lymphoma: a retrospective series of 35 cases

Primary gastric lymphoma: a retrospective series of 35 cases

Tunis Med. 2008 May
Ennibi K, Mikdame M, Rabhi M, Jroundi I, Benkirane A, Chaari J, Toloune F.
Service de Médecine interne A. Hôpital Militaire d'Instruction Mohammed V. Rabat. Maroc.


BACKGROUND: Primary gastric non Hodgkin's lymphoma (PGNHL) is the most common site of extranodal malignant lymphoma. It is a rare subtype of malignancy, for which no consensus exists about treatment.

AIM: The purpose of This paper was to assess the managemnt of PGNHL.

METHODS: Between January 1992 and December 2001, 35 patients were retrospectively evaluated.

RESULTS: Of the 35 patients, with a mean age of 44 years old Signs and symptoms are unspecific. 20 (57.1%) were in stage IE/IIE1. 15 patients (42.8%) were in stage IIE2,IIIE,IVE. These patients were treated with primary surgery with or without chemotherapy (11; 31.4%); primary chemotherapy (CT) alone with surgery in one patient (21; 60%) and three patients with gastric MALT lymphoma were treated by Helicobacter pylori eradication. The 5 year survival rates of the 35 patients are 62, 86%. There was no significant difference in the 5 year survival rate between the patients with low grade lymphoma and the patients with large grade lymphoma (75% versus 60%, P = 0.467). The 5-year survival rates for stage I/IIE1 and IIE2/III/IV patients were 80%, 53.3% respectively (p less then p =" 0.05).

CONCLUSIONS: This study suggested that primary surgical resection may be important factor predicting the long-term survival of patients with primary gastric NHL. H. pylori eradication therapy was an effective first-line treatment for patients with gastric MALT lymphoma.

PubMed