Thursday, 16 May 2013

Lyme Disease Complicates Doctor-Patient Relationship


 June 29, 2012

BOSTON — Controversy still exists over the long-term diagnosis and treatment of Lyme disease. A small group of doctors and some sufferers say that long-term symptoms such as headache, fatigue and pain stem from an ongoing infection from Lyme bacteria that can persist even after antibiotics. They say that Lyme disease itself can be “chronic.”
However, many doctors and researchers in mainstream medicine reject the theory that the bacteria can persist. They agree that Lyme disease can have long-term effects, but blame lingering damage rather than active bacteria.
Dr. Mark Drapkin is one of those doctors. He is the associate chief of the Division of Infectious Diseases at Newton-Wellesley Hospital and a professor at Tufts University School of Medicine. He spoke with Morning Edition host Bob Oakes about the issue.
Dr. Mark Drapkin: After treating an infection and eradicating the organism, there can be a period during which the person still feels ill, during which various manifestations can occur. They can be immune kind of manifestations, for example a joint might still suffer the pain and swelling that may have accompanied the original infective arthritis. But now that is a damaged joint and the damage has a life of its own. But that does not necessarily imply that the bug is still there and that the problem is that the organism itself has not been eradicated.
Bob Oakes: Let me get you to respond to something said by another doctor, a specialist in Lyme. He’s Dr. Daniel Cameron, an internist based in Mount Kisco, N.Y., former president of the International Lyme and Associated Diseases Society. He says there needs to be an acknowledgement that people are suffering for months or years after being diagnosed with Lyme. And he also thinks that there should be a serious look by the medical establishment at some alternative strategies, such as long-term antibiotic treatment for patients:
I’ve been successful for over 20 years in practice at taking these complex patients and getting them better. I just need the research community to take these strategies and work with them.
Why has it been so hard to get the research community to dig deeper into treatments that some patients and some doctors say are working for patients?
Well, there have been some attempts to do that. With all due respect to the physician whom you have just quoted, when a person says, “I’ve been doing this for 20 years and it works,” I don’t listen very carefully because that’s not a research statement. These antibiotics are not benign. And I can match anecdote for anecdote if people have done well with antibiotics, they come to me when they have not done well with the antibiotics.
But you and I both know there are people on the other end of the radio who are suffering from Lyme who are going to say, “Well you know, I have a doctor who gives me antibiotic treatments and I feel that I’m doing much better with them.”
I sympathize with that person. I can’t argue with a person who feels better and I’m glad that the person’s feeling better. But does that mean that that person would feel any better or any worse if that individual received a placebo? I don’t know unless there is a trial that tells me that. I’m a physician. I’ve been in practice for 40 years now. Do I ever use drugs in ways that are not completely justified by medical science? Of course. We have to do things for patients sometimes that are not bolstered by excellent science. We know that. But with respect to Lyme disease, I would just ask that the same standards of testing, of investigation be applied to Lyme disease as are applied to any other infectious or other disease.
In a way we’re talking about, or maybe around, the story of Barbara MacLeod, a former reporter for New England Cable News who says she’s been battling Lyme disease for more than 20 years. She gives this assessment:
This is one of those diseases where you don’t get it until you get it. And the small group of doctors who will treat aggressively, and who believe the patients, they usually have some personal experience with Lyme. Where they can’t believe that their mother, sister, daughter, son or themselves are suffering the way they are, and can’t get answers and can’t get treatment.
So what do you say to someone like Barbara MacLeod, who has had great difficulty with Lyme disease for a couple of decades and has had trouble getting treatment for it?
Well, I hesitate to comment on a particular person’s illness.
Because you haven’t examined that person?
I haven’t examined the person. I haven’t gone over the medical record. I think it would be wrong for me to make any snap judgements about a particular individual’s diagnosis or treatment.
So what would you say to a person who’s feeling the symptoms — fatigue, headache, pain — and can’t find any answer?
I will be open-minded and I will not comment on an individual’s diagnosis until I have the data: the face-to-face talk time, the physical examination, the review of all prior laboratory testing. Those same symptoms could be early HIV infection. I would wonder has the person been HIV tested. Those same symptoms could be those of Lupus. I would wonder if that disease had been considered in the differential diagnosis. There are many, many instances where people have come to me thinking that they had one entity, and it turns out they have something else entirely.
Is it [in] some ways more difficult to treat patients because they come in with this preconceived notion that they think they have Lyme disease, because there’s so much information available about what many people call “chronic” Lyme disease, or others call “post-Lyme” disease syndrome?
Once you latch on to a diagnosis, it can be very hard to disabuse you of that notion. And if a patient has a list of symptoms and then sort of looks around and says, “OK, what matches my list?” And then starts looking for other lists and you come across a Lyme website, for example, that lists all of those symptoms, you say, “Oh my God. That’s me. That’s me. And that’s just what I've been experiencing.” And latches onto that diagnosis. It does cause difficulty. It does cause difficulty, because I am trying to look at this patient from a broader perspective and to try to keep my mind open. How much more difficult it is for a patient to keep his or her mind open when that person has recognized what seems to be a list of symptoms that matches his or hers exactly.

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Friday, 29 March 2013

Sarcoidosis is a condition where small bead like patches of inflamed cells called granulomas show up in the body

Sarcoidosis

Introduction:

Sarcoidosis is a condition where small bead-like patches of inflamed cells called granulomas show up in the body, usually in the lungs and nearby lymph nodes. Sarcoidosis can also affect other parts of the body, including the muscles, eyes, and skin.

Many people with sarcoidosis may have no symptoms at all. But in others, the condition can cause long-term organ damage. An example is the formation of fiber-like scar tissue in the lung, which can cause breathing problems. Sarcoidosis may develop over time and cause symptoms that last for years, or it may show up and go away quickly.

People who have a variation of sarcoidosis, called Lofgren's syndrome, may have symptoms that include swollen lymph nodes, fever, painful, reddened nodules, and joint pain. Lofgren's syndrome generally tends to clear up on its own within 1 - 2 years.

Sarcoidosis affects more African-Americans than Caucasians in the United States. About 36 in 100,000 African-Americans, while 11 in 100,000 Caucasians have the condition.

Signs and Symptoms:

Many people with sarcoidosis have no symptoms at all.

Some people with pulmonary (lung) sarcoidosis may have the following signs and symptoms:
When sarcoidosis affects areas of the body other than the lungs, symptoms can include:
  • Enlarged lymph nodes
  • Red bumps on arms, face, buttocks
  • Fever
  • Swelling and pain in the ankles and knees
  • Infections of the eye, including pink eye (conjunctivitis)
  • Enlarged or inflamed
  • Enlarged or inflamed liver

Causes:

Researchers don't know exactly what causes sarcoidosis. Some think it's due to an overactive immune system that responds too strongly to an invading organism. Other researchers have proposed that sarcoidosis may be inherited, caused by an infection, or caused by allergens that are breathed in or toxins found in the environment.

Risk Factors:

Anyone can develop sarcoidosis, although it's more common among the following:
  • People of Scandinavian, Irish, African, or Puerto Rican descent
  • People in their 30s or 40s
  • Women

Diagnosis:

Sarcoidosis can be hard to diagnose. You may have to see several different doctors, including a pulmonologist (lung specialist). To begin, your doctor will rule out other conditions that could be causing your symptoms, such as rheumatoid arthritis. The following tests may help diagnose the condition:
  • Chest x-ray
  • Lung function tests
  • Biopsy
  • Blood tests
  • Bronchoscopy -- examines the inside of your lungs
  • CT scan, MRI, or other imaging tests
  • ECG (electrocardiogram)

Treatment:

About half of all people with sarcoidosis get better without needing treatment. For others, medications such as corticosteroids may help reduce swelling, rashes, pain, fever, and lung problems. Some lifestyle changes may help control complications, such as kidney stones or other damage. While complementary therapies for sarcoidosis have not been well studied, they may help you feel better as part of an overall treatment plan.

Lifestyle

If you smoke, quit. Quitting smoking can ease lung symptoms.

Sarcoidosis can be a long-lasting disease, so it's important to eat a healthy diet filled with fruits, vegetables, and whole grains.

Exercise regularly. Be sure to ask your doctor before starting a new exercise program, especially if sarcoidosis leaves you short of breath.

Medications

Corticosteroids such as prednisone are considered the first-line treatment for lowering inflammation associated with sarcoidosis. Oral corticosteroids can have some serious side effects if taken in high doses for long periods. Side effects may include high blood pressure, diabetes, peptic ulcers, tuberculosis, or osteoporosis. Your doctor will likely suggest regular check-ups and tests.

Other medications sometimes used for sarcoidosis include those that suppress the immune system, such as methotrexate, azathioprine (Imuran), and infliximab (Remicade). However, there are no long-term studies on whether these medication are effective for treating sarcoidosis.

Other medications that may be used include:

Antimalarial drugs such as hydroxychloroquine -- used when the skin is affected. It may be toxic to the eyes.
Thalidomide -- being studied for sarcoidosis; used to improve lung function and treat skin problems.

Surgery and Other Procedures

Surgery, such as a lung or heart transplant, is only necessary in very severe cases.

Nutrition and Dietary Supplements

Although there is no evidence that any particular herb or supplement helps treat sarcoidosis, a comprehensive treatment plan may include complementary and alternative therapies. Ask your team of doctors about the best ways to bring these therapies into your overall treatment plan. Always tell your doctor about the herbs and supplements you are using or considering using, as some supplements may interfere with conventional treatments.

These nutritional tips will help your overall health:
  • Eat antioxidant foods, including fruits (such as blueberries, cherries, and tomatoes), and vegetables (such as squash and bell peppers).
  • Include foods rich in magnesium and low in calcium, such as barley, bran, corn, rye, oats, soy, brown rice, avocado, banana, and potato.
  • Avoid refined foods, such as white breads, pastas, and sugar.
  • Eat fewer red meats and more lean meats, cold-water fish, tofu (soy, if no allergy) or beans for protein.
  • Use healthy oils, such as olive oil or vegetable oil.
  • Reduce or eliminate trans-fatty acids, found in commercially baked goods such as cookies, crackers, cakes, French fries, onion rings, donuts, processed foods, and margarine.
  • Avoid caffeine, alcohol, and tobacco.
  • Drink 6 - 8 glasses of filtered water daily.
  • Exercise at least 30 minutes daily, five days a week. Ask your doctor before starting a new exercise routine.
The following supplements may also help overall health:
  • A daily multivitamin, containing the antioxidant vitamins A, C, E, the B-complex vitamins, and trace minerals such as magnesium, calcium, zinc, and selenium.
  • Omega-3 fatty acids, such as fish oil, 1 - 2 capsules or 1 - 3 tablespoonfuls oil, one to three times daily. Fish oil seems to help reduce inflammation throughout the body. Cold-water fish, such as salmon or halibut, are good sources to add to your diet. If you take supplements, check with your doctor because they may increase the risk of bleeding, especially if you take blood thinners such as warfarin (Coumadin) or aspirin. Eating fish doesn' t cause the same risk.
  • Bromelain, a mixture of enzymes derived from pineapple, 500 mg per day. Bromelain may also help reduce inflammation in the body. Ask your doctor before taking it, because it can increase the risk of bleeding and may interact with other medications.
  • Probiotic supplement (containing Lactobacillus acidophilus), 5 - 10 billion CFUs (colony forming units) a day. These "friendly" bacteria help maintain gastrointestinal health. You should refrigerate your probiotic supplements for best results.

Herbs

Herbs are generally a safe way to strengthen and tone the body's systems. As with any therapy, you should work with your health care provider to get your problem diagnosed before starting any treatment. You may use herbs as dried extracts (capsules, powders, teas), glycerites (glycerine extracts), or tinctures (alcohol extracts). Unless otherwise indicated, you should make teas with 1 tsp. herb per cup of hot water. Steep covered 5 - 10 minutes for leaf or flowers, and 10 - 20 minutes for roots. Drink 2 - 4 cups per day. You may use tinctures alone or in combination as noted.

Studies haven' t found any herbs that specifically treat sarcoidosis. However, the following herbs may help overall health. Talk to your doctor before taking any herb or supplement if you have sarcoidosis.
  • Turmeric (Curcuma longa) standardized extract, 300 mg three times a day, may help reduce inflammation. Turmeric may increase the risk of bleeding and interact with other medications, so ask your doctor before taking it.
  • Cat's claw (Uncaria tomentosa) standardized extract, 20 mg three times a day, may help reduce inflammation. People with leukemia should not take cat' s claw. Cat' s claw interacts with some medications, so ask your doctor before taking it.

Homeopathy

A few case reports suggest that homeopathic remedies may improve the general well-being of individuals with sarcoidosis. An experienced homeopath can prescribe a regimen for treating sarcoidosis that is designed specifically for each individual. The primary remedies used by individuals who reported improvements in their symptoms include:
  • Tuberculinum bovinum
  • Beryllium
Other homeopathic remedies that have been used clinically for the condition are as follows:
  • Carcinosin
  • Euphrasia
  • Graphites
  • Leuticum (Syphilinum)
  • Bacillinum
  • Sepia
  • Phosphorus
  • Arsenicum album

Other Considerations:


Pregnancy

Women with sarcoidosis can still get pregnant. But if you are thinking about having a baby, talk to your doctor. Some of the medications used to treat sarcoidosis, such as methotrexate, can be harmful to the baby. Up to 65% of women may see their symptoms improve during pregnancy, while 5% may find symptoms get worse. Some women may have a flare of symptoms after giving birth. Pregnant women with sarcoidosis should avoid being exposed to x-rays.

Prognosis and Complications

Complications from sarcoidosis usually happen in only the most serious cases, and can include heart, kidney, and lung damage. Long-term use of corticosteroids may cause ulcers, diabetes, high blood pressure, osteoporosis, and infections, such as tuberculosis.

The prognosis for most people with sarcoidosis is good. Only 15% of those with sarcoidosis have symptoms that get worse. About 5% develop severe lung problems, which increases the risk of death. Almost half of all people with sarcoidosis get better without any therapy. Treatments used today, such as corticosteroids, often help ease the inflammation associated with the condition.
  • Reviewed last on: 9/3/2010
  • Steven D. Ehrlich, NMD, Solutions Acupuncture, a private practice specializing in complementary and alternative medicine, Phoenix, AZ. Review provided by VeriMed Healthcare Network.

Supporting Research

Boots AW, Drent M, Swennen EL, Moonen HJ, Bast A, Haenen GR. Antioxidant status associated with inflammation in sarcoidosis: A potential role for antioxidants. Respir Med. 2009 Mar;103(3):364-72.
Cox CE, Donohue JF, Brown CD, Kataria YP, Judson MA. Health-related quality of life of persons with sarcoidosis. Chest. 2004 Mar;125(3):997-1004.
Dambro MR. Griffith's 5-Minute Clinical Consult. Baltimore, Md: Lippincott Williams & Wilkins, Inc.; 2006.
Doty JD, Mazur JE, Judson MA. Treatment of sarcoidosis with infliximab. Chest. 2005 Mar;127(3):1064-71.
Dryden GW Jr, Deaciuc I, Arteel G, McClain CJ. Clinical implications of oxidative stress and antioxidant therapy. Curr Gastroenterol Rep. 2005;7(4):308-16.
Fauci AS, Braunwald E, Hauser SL, et al, eds. Harrison's Principles of Internal Medicine. 17th ed. New York, NY: McGraw-Hill; 2008.
Funk JL, Oyarzo JN, Frye JB, et al. Turmeric extracts containing curcuminoids prevent experimental rheumatoid arthritis. J Nat Prod. 2006;69(3):351-5.
Goldman L, Ausiello DA, et al, eds. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: W.B. Saunders; 2007.
Gonclaves C, Dinis T, Batista MT. Antioxidant properties of proanthocyanidins of Uncaria tomentosa bark decoction: a mechanism for anti-inflammatory activity. Phytochemistry. 2005;66(1):89-98.
Goroll AH, ed. Primary Care Medicine, 5th ed. Philadelphia, Pa: Lippincott Williams & Wilkins Publishers; 2007.
Krieg C. Role of diet in the prevention of common kidney stones. Urol Nurs. 2005;25(6):451-7.
Lichtenstein AH, Russell RM. Essential nutrients: food or supplements? Where should the emphasis be? JAMA. 2005;294(3):351-8.
Massey L. Magnesium therapy for nephrolithiasis. Magnes Res. 2005;18(2):123-6.
Nguyen YT, Dupuy A, Cordoliani F, Vignon-Pennamen MD, Lebbé C, Morel P, Rybojad M. Treatment of cutaneous sarcoidosis with thalidomide. J Am Acad Dermatol. 2004 Feb;50(2):235-41.
Rakel RE, ed. Conn's Current Therapy 2008. Philadelphia, Pa: W.B. Saunders; 2008.
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Tuesday, 5 March 2013

A Case of Abdominal Sarcoidosis in a Patient with Acute Myeloid Leukemia

Bone marrow aspirate showing acute myeloid leu...
Bone marrow aspirate showing acute myeloid leukemia. Several blasts have Auer rods. (Photo credit: Wikipedia)

Case Reports in Hematology
Volume 2013 (2013), Article ID 379898, 3 pages
http://dx.doi.org/10.1155/2013/379898

1Department of Haematology, Pilgrim Hospital, Sibsey Road, Boston, PE21 9QS, Lincolnshire, UK
2Department of Oncology, Pilgrim Hospital, Sibsey Road, Boston, PE21 9QS, Lincolnshire, UK
Received 7 January 2013; Accepted 3 February 2013
Academic Editors: S. Aref, E. Arellano-Rodrigo, and P. Tsirigotis
Copyright © 2013 Vadsala Baskaran et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The allogeneic bone marrow transplantation usually preceded by induction chemotherapy, in fit patients, represents the gold standard in the acute myeloid leukaemia. In the last years, many trials have been set up with the view of improving the number of remissions during the induction by adding new drugs. Several early or late side effects have been described in the literature. We herein present a patient with acute myeloid leukaemia patient who, after chemotherapy, developed ascites that turned out to be abdominal sarcoidosis.

1. Case Report

A 68-year-old Caucasian lady presented to the Haematology Department with pancytopenia and palpitations in March 2009. She had a past medical history of uterine fibroids necessitating hysteroannessectomy, diverticulitis, oesophageal reflux, and hypercholesterolemia, as well as a family history of familial Polyposis coli for which she was under surveillance. She was taking ranitidine and simvastatin regularly.
Bone marrow biopsy revealed hypercellular bone marrow with architectural distortion of haemopoiesis, a reduction in mature granulocytes and infiltration of myeloblasts (which made up 30–40% of all cells seen within the biopsy) consistent with acute myeloid leukaemia (AML NOS M1 category, cytogenetics 46 XX). She was commenced on the AML16 trial and treated with Daunorubicin, Clofarabine, Gemtuzumab, and nine months maintenance Azacytidine. Allogenic bone marrow transplant was considered but was not pursued at the patient’s request. On completion of five months of treatment (October 2010), the patient was asymptomatic and a repeat bone marrow biopsy demonstrated full remission.
Three months later (January 2011), the patient was admitted with abdominal discomfort and distension. On examination, there was evidence of ascites with normal renal and liver function, haemoglobin 12.6, white cell count 3.9, platelet count 316, and an elevated CA125 at 320. Echocardiogram demonstrated good biventricular function. Imaging of the abdomen and pelvis confirmed gross ascites with no focal liver lesion, but there was evidence of fat stranding in the omentum with small omental deposit on the computed tomography of the abdomen and pelvis with contrast (Figure 1). Subsequent MRI showed omental cake and mesenteric deposits raising the possibility of peritoneal neoplasia or mesothelioma. Abdominal paracentesis was performed and 4 litres of straw coloured fluid was drained. The serum-ascites albumin gradient (SAAG) was 1 with LDH 95, white cell count 923 (90% lymphocytes) and no malignant cells seen. Flow cytometry of the ascitic fluid confirmed no evidence of AML. A total body CT scan was performed to rule out any primary solid tumour and there was no evidence of any solid tumour. The patient continued to require frequent ascitic drainage every 3-4 weeks with approximately 4 litres of fluid drained on each occasion.
379898.fig.001
Figure 1: CT scan of the abdomen and pelvis with contrast showing heterogeneous fat/soft-tissue density within the greater omentum (see white arrow). There is also extensive ascites.
A laparoscopic peritoneal biopsy was arranged; macroscopically there was evidence of some miliary deposits on the dome of the bladder and along the peritoneum surface (Figure 2). This was followed by a cystoscopy which did not demonstrate any abnormalities.
379898.fig.002
Figure 2: Laparoscopic image of glistening nodules on the peritoneal surface.
Histopathology for the biopsies taken from the peritoneal nodules reported the presence of chronic inflammation and noncaseating granulomata (Figure 3). There were no atypical lymphoid cells or immature granulocytes visualised; no evidence of malignancy or vasculitis and no fungi or acid fast bacilli were grown on extended culture with Grocott and Ziehl-Neelson staining. The patient was admitted 6 times with an interval of 7–10 days to have abdominal paracentesis, every time aspirating 4-5 litres. QuantiFERON test was performed to rule out abdominal tuberculosis and it turned out to be negative.
379898.fig.003
Figure 3: Biopsy of peritoneal nodules showing noncaseating histiocytic granulomas.
Based on these results, the patient was commenced on an empirical dose of 25 mg prednisolone and subsequently her symptoms fully resolved. Residual omental thickening was not present in the repeat MRI scan and she no longer required any further paracentesis.
Having excluded the more common causes of granulomata including tuberculosis, fungal infection, autoimmune disease, and neoplasia, this left us with a likely diagnosis of abdominal sarcoidosis. Serum angiotensin-converting enzyme level was normal (54).

2. Discussion

Sarcoidosis typically presents with lung involvement or the classic tetrad of fever, bilateral hilar lymphadenopathy, erythema nodosum, and arthralgia; however, 10% of patients present with extrathoracic involvement, most often hepatic [1]. Ascites can occur and is most often transudate, secondary to pulmonary hypertension or portal hypertension due to granulomatous obstruction. However, there are a small number of case reports (a search of the Medline database returned a total of 28) reporting transudative or exudative ascites associated with peritoneal or serosal sarcoid studding [2]. This is associated with a significant elevation in serum CA125, as seen in our patient. Isolated ascites in sarcoidosis is usually benign and highly responsive to steroids, a feature again seen in our patient.
ACE is produced by sarcoid granulomas, and serum ACE levels are used to correlate with disease load [2]. Elevated serum ACE occurs in approximately 60% of patients with sarcoidosis. However, having a normal ACE level as in our patient does not exclude the diagnosis.
There has been several previous case reports of sarcoidosis associated with a diagnosis of AML. Of these, only three reported AML preceding the diagnosis of sarcoidosis, with intervals ranging from 11 months to 17 years [35]. The case presented here adds to list, with an interval of 8 months between the diagnosis of AML and sarcoidosis. To our knowledge this is the only case report of peritoneal sarcoidosis following a diagnosis of AML. There have been no previous cases revealed on extensive literature searches.
The exact relationship between AML and sarcoidosis is unclear. It has been hypothesised that the granulomatous inflammation observed in sarcoidosis may occur in reaction to tumour-associated antigens that are widespread in AML. In addition, the transmission of sarcoidosis or sarcoidosis-inducing pathogens, for example, via bone marrow transplantation, has been considered [6]. Our patient did not receive a bone marrow transplant, but she was treated with several chemotherapeutic agents as part of the clinical trial. Granulomatous disease has been associated with exposure to chemotherapy agents, for example, capecitabine, interferon beta, and particularly heavy metals or methotrexate [78]. It is not clear whether this was a factor in the case presented here. There have been no similar cases in the AML16 study cohort to our knowledge, but this has been raised as a potential adverse event with the trial centre. The relationship between AML and sarcoidosis is an area that requires further study.

3. Conclusion

The distinctiveness of this case is the uncommon presentation of sarcoidosis, in association with a preexisting diagnosis of AML. As mentioned in the discussion, our patient is a participant of a clinical trial and a possible conjecture is whether one of the chemotherapeutic drugs used could have influenced the development of granulomas in our patient. However no similar symptoms have been reported in other trial patients to our knowledge.
We have highlighted this case as a possible late adverse event to the trial centre and to the competent authority.

References

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