Immunoglobulin M deficiency

IgM deficiency - Immunoglobulin M deficiency: Description, Causes and Risk Factors:IgMIgM antibodies are the largest type of antibody. They are found in the bloodstream and lymph fluid. The IgM antibodies are the first antibodies that are produced in response to an infection. They also stimulate other immune system cells to produce compounds that can destroy invading cells. IgM antibodies normally make up about 5-10% of all the antibodies in the body.Selective immunoglobulin M deficiency (SIgMD) is a rare form of primary immunodeficiency with a reported prevalence of 0.03% to 3%. Selective IgM deficiency can be asymptomatic or present symptomatically with infections caused by encapsulated bacteria and viruses, some of which can be serious and even life-threatening. These vary from pneumonia to septicemia and meningitis.Selective IgM deficiency is a heterogeneous disorder with no known genetic component, and may occur as a primary or a secondary condition. Primary in children may present with severe life-threatening infections, whereas in adults it is usually associated with autoimmune diseases and malignant neoplasm. Secondary selective IgM deficiency is much more common than primary selective IgM deficiency and may be seen in association with malignancy, autoimmune disease, gastrointestinal disease, and immunosuppressive treatment.The pathogenesis of primary selective IgM deficiency is unknown. A number of defects have been reported, including intrinsic B-cell defect in plasma cell differentiation, increased T-cell suppressor activity, which may be specific to IgM isotype or isotype-nonspecific, and decreased helper T-cell activity. This suggests that selective IgM deficiency is a heterogeneous disorder, which requires further studies to elucidate the predominant mechanisms involved in its pathogenesis.The cause of SIgM deficiency is unknown, and no clear pattern of inheritance has been suggested. The stability of the finding of selective deficiency of IgM versus progression to deficiency of other immunoglobulin isotypes, has not been well characterized.Patients with malignant neoplasms (eg, clear cell sarcoma, Bloom syndrome, promyelocytic leukemia), autoimmune diseases (eg, rheumatoid arthritis, Hashimoto thyroiditis, systemic lupus erythematosus, autoimmune hemolytic anemia), infections (eg, Brucella), or those given immunosuppressive agents may develop secondary SIgM deficiency.Patients with selective immunoglobulin M deficiency are susceptible to recurrent sepsis and overwhelming infection with encapsulated bacteria (eg, Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae). They may also have autoimmune disease including glomerulonephritis and osteomyelitis from which organisms are not recoverable, as well as malignancies, chronic dermatitis, diarrhea, and upper respiratory infections (UTIs).Symptoms:Patients may be asymptomatic, while others may experience prolonged or life-threatening infections, especially during infancy. Recurrent infections, including, sinusitis and pneumonia, are caused by bacteria. Patients may also experience atopic or chronic dermatitis, allergic rhinitis (hay fever), wheezing, and/or diarrhea.Diagnosis:A nephelometry blood test may be performed to diagnose SIgM deficiency. The disorder is diagnosed after significantly decreased IgM are observed in the patient's blood. During the procedure, a sample of blood is taken from the patient. Anti-immunoglobulins are added to the blood sample. A medical instrument then measures the movement of particles in a substance that is caused by the interaction between immunoglobulins and anti-immunoglobulins. The test quickly and accurately measures the amount of IgM in the patient's blood.Treatment:Infections should be treated promptly with appropriate antibiotics, depending on the suspected pathogen. Empiric broad-spectrum therapy may be necessary before specific organisms are isolated. Some patients with selective IgM deficiency may also have increased susceptibility to fungal infections, so excessive use of broad spectrum antibiotics should be avoided, and antifungals should be added when appropriate. Intravenous immunoglobulin is a therapeutic consideration for patients with SIgM deficiency who have demonstrated findings of defective antigen-specific IgG responses, particularly if they lack IgG against encapsulated bacteria and have chronic or recurrent sinopulmonary infection.Commonly prescribed antibiotics include amoxicillin (Trimox® or Biomox®), cefuroxime (Ceftin®, Kefurox®, or Zinacef®), amoxicillin (Amoxil®, Polymox®, or Trimox®), trimethoprim/sulfamethoxazole, also called TMP/SMX (Bactrim®, Cotrim®, or Septra), azithromycin (Zithromax®), clarithromycin (Biaxin®), erythromycin (Erythrocin® or Ery-Tab®), and amoxicillin and clavulanate (Augmentin®).NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

8 Comments

  1. Craig O'Brien

    I have <10 Igm and my Doctor feels I'm ok with that level.

    Also my IgG is 531 was 55 points high two months ago and Doctor feels it's ok to go down to 400 level

    Problem I very concerned, any help would be much appreciated .

    Reply
    • maisteri

      The thing is that the doctors do not treat the results of the lab analyses but also take into consideration the patient’s symptoms and complaints. The slightly decreased immunoglobulin levels are considered mild hypogammaglobulinemia and may be caused by numerous factors. However, if there are no other symptoms except the changed lab results may consider these changes not relevant. If you are concerned and face any disturbing symptoms such as frequent bacterial infections consult with an immunologist.

      Reply
  2. Devayani Lathi

    I have Bcell follicular lymphoma, treated with Rituxamab 4times, along with CVP and Bendamustin on two seperate occsions.,followed by Rituximab mentainance.
    As a result, my IGM has dropped to below detectable levels. I am havinf prolonged respirotory infections.
    Is there a way to treat IGM deficiency or lack of IGM with replacement by extrnal IGM ?

    Reply
    • maisteri

      There is no way to to provide external IgM. However, intravenous IgG replacement therapy may be an option for you. These immunoglobulins as well as IgM are involved in the humoral immunity and administering them may help you to prevent recurrent infections. Nevertheless, you should consult with your doctor regarding the best treatmet plan for you.

      Reply
    • Smile Meadow

      Hello Devayan,

      My husband has the exact same history you have except he only had the Rituximab one round with a continuation of bimonthly prophylactic treatments of Rituximab for about 18months. As long as he was getting the CHOP chemo and Nurlasta he was fine but the follicular lymphoma came back so then they gave him Bendamustine which put the lymphoma into remission. I think the Nurlasta shots are what drove the lymphoma to return, after all the point of that shot is to drive WBC proliferation. There may be some point in doing this because the malignancy would probably respond most and thus more residual malignancy could be eliminated.

      Since he was getting the Rituximab alone his lungs were progressively declining and he began losing weight also. Stopping the drugs was the choice since now the lymphoma is in remission. He is however continuously infected with these respiratory infections, and has the low IgM with the rest of immunoglobulins normal.

      We are looking for an Immunology consult but since his care is through the VA and since we have this administration and since the immunologists primarily take care of AIDS patients they have been cut out of the VA system. Such a shame, treatments that have been refined by a disease’s treatments become unavailable for other uses because of an outdated puritanical prejudice against the sufferers of that disease. Also the rest of our citizens are at peril because of the proliferation allowed by weakness in the herd that could be treated and are not.

      Any ideas on raising the IgM would be very appreciated.

      Reply
      • maisteri

        As it was stated before, there is no way to increase IgM levels. But intravenous IgG may be administered if so would be recommended by your doctor. Of course, IgM antibodies are essential for the body’s defence against infections, but IgG antibodies play the same role, although they act slower and are considered slightly less efficient.
        Except the immunotherapy in your case preventive antibiotic treatment, flu shot and some other vaccinations may be recommended.

        Reply
  3. Inquiring Minds

    Can pancreatic diabetes (DMT3C) or Chronic Pancreatitis cause IgM deficiency?

    Reply
    • maisteri

      IgM deficiency cannot be caused by the pancreatic diabetes. On the contrary, there are evidences that IgM levels may appear decreased in autoimmune pancreatitis and, furthermore, low IgM concentration may play role in the development of this disorder. Autoimmune pancreatitis is a chronic inflammation of the pancreas mediated by the hyperactive immune system. So in your case autoimmune pancreatits should be considered as a possible diagnosis.

      Reply

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