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IgE Myeloma

IgE myeloma is a rare variant of multiple myeloma, characterized by the neoplastic proliferation of plasma cells that produce immunoglobulin E (IgE).


Presentation

IgE myeloma was first described in 1967 by Johansson and Bennich and till date, only 40 cases have been documented [1] [2] [3]. IgE myeloma does not present with any specific symptoms or signs when compared to the other forms of multiple myeloma (MM) [4]. It is, however, considered to be a more aggressive entity, with studies showing the median survival time to be reduced (16 months vs 30 months for other types of MM) [5] [6].

The average age of onset of this disease is 62 years, ranging from 38-80 years. It is known to occur slightly more commonly in males. Patients usually present in a similar manner to other forms of MM, with bone pain, anemia, hypercalcemia and renal failure forming the clinical spectrum.

The most common complaint of patients is bone pain, occurring in the lumbar areas. Other areas that may be involved are the long bones, the skull, and pelvis. They may be associated with pathological fractures, mostly involving the axial skeleton. Spinal cord compression may be a result of these compressive, osteolytic bone lesions.

Anemia may present as weakness in a majority of patients. The incidence of hepatosplenomegaly is higher in IgE myeloma, as compared to the commoner variants of MM [7].

Hypercalcemia, seen in almost 30% of patients, may present as nausea, constipation, thirst, somnolence and confusion. Renal insufficiency, a marker of poor prognosis, may be seen in a few patients [8].

Patients may also report certain constitutional symptoms related to hyperviscosity. An increased incidence of infections may also be found in such patients.

Workup

IgE myeloma is to be suspected in middle-aged adults who present with the characteristic symptoms of bone pain, along with the other features, or in those who have incidental abnormalities on routine blood tests, including anemia, raised erythrocyte sedimentation rate (ESR) or hypercalcemia [9].

Routine blood tests to be done include a complete blood count (CBC) that demonstrates a normocytic normochromic anemia, present in roughly three-quarters of patients. WBC and platelet counts are usually unaffected. ESR may be raised to > 100 mm/hr.

Hypercalcemia may be present in 10% of patients at the time of diagnosis. Other renal function tests like serum creatinine, blood urea nitrogen test (BUN) and serum uric acid may reveal features of renal insufficiency.

In contrast to the other variants of multiple myeloma, most patients with IgE myeloma do not demonstrate monoclonal protein bands on serum or urine electrophoresis. The diagnosis is then made by bone marrow aspiration and biopsy, that may reveal large numbers of plasma cells. Immunohistochemical staining will show these plasma cells to be strongly positive for IgE in their cytoplasm. Light chain analysis may have prognostic significance in these patients [10].

Imaging might reveal the characteristic pathological fractures or lytic lesions in the skeleton and hence, plain X-rays of the spine, skull, pelvis and long bones need to be carried out. Magnetic resonance imaging (MRI) may help localize the site of bone pain. Positron emission tomography (PET) scans are rarely required.

Chromosomal studies may be done to detect the characteristic translocation abnormalities seen in IgE myeloma.

Treatment

  • Treatment with prednisone, melphalan, cyclophosphamide and interferon alfa did not produce any improvement and the patient died 5 months after diagnosis.[ncbi.nlm.nih.gov]
  • Seite 295 - Third Report of the National Cholesterol Education Program (NCEP) Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel III). ‎[books.google.com]
  • The patient underwent surgical removal of the sacral tumor and monthly melphalan-prednisone treatment together with intravenous pamidronate infusions.[ncbi.nlm.nih.gov]
  • A Review of the Literature and Analysis of the Results of Treatment of 155 Patients with Various Blood Dyscrasias, Lymphomas and other Malignant Neoplastic Diseases. J. Lab. ‎[books.google.com]
  • Treatments to control the myeloma In many cases, treatment can control the myeloma and put the disease into remission. Remission is not necessarily a cure.[patient.info]

Prognosis

  • Renal insufficiency, a marker of poor prognosis, may be seen in a few patients. Patients may also report certain constitutional symptoms related to hyperviscosity. An increased incidence of infections may also be found in such patients.[symptoma.com]
  • It is very important for you to undergo all of the appropriate tests, as the results will help your doctor better determine treatment options and prognosis .[themmrf.org]
  • Finding these abnormalities can provide information about an individual's prognosis .[labtestsonline.org]
  • Fewer mature plasma cells indicate a poorer prognosis. Bone marrow microvessel density: A high degree of new blood vessel development in the bone marrow indicates active tumor growth and thus a poorer prognosis.[emedicinehealth.com]
  • The prognosis remains poor, especially in the secondary form, where it is a consequence of the progression of the disease and increasing chemoresistance .[intechopen.com]

Etiology

  • IgE levels in adults are less helpful in establishing an allergic etiology for symptoms.[clinlabnavigator.com]
  • Although such an unrelated etiology would be unexpected, one would be obligated to pursue other possible causes. These would include quite a number of illnesses, most of which would not fit the clinical picture you describe.[aaaai.org]
  • The exact etiology of multiple myeloma remains unclear.[aafp.org]
  • Etiology, Epidemiology and Pathophysiology Etiology The cause or causes of myeloma are unknown, but there is some evidence to support a number of theories of its origin, including viral, genetic, and exposure to toxic chemicals, the most notable being[cancer.ucsf.edu]

Epidemiology

  • The term “multiple myeloma” was first used to describe the presence of multiple tumors originating in the bone. 1 Epidemiology Multiple myeloma is the most common primary cancer of the bones in adults.[aafp.org]
  • Research Focus Molecular epidemiology Genetic susceptibility Hematopoietic malignancies Gynecologic tumors Stroke and related cardiovascular endpoints Childhood cancers and conditions Immune biomarkers Infectious and viral causes of cancer There are a[cityofhope.org]
  • Epidemiology It is estimated that in 2017, approximately 30,280 new cases of multiple myeloma will be identified in the U.S., representing 1% of all malignancies and 10% of all hematological malignancies.[cancer.ucsf.edu]
Sex distribution
Age distribution

Pathophysiology

  • Useful For Suggests clinical disorders or settings where the test may be helpful Detecting or monitoring of monoclonal gammopathies and immune deficiencies Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they[mayomedicallaboratories.com]
  • Pathophysiology Malignant plasma cells develop from an immune cell called a B lymphocyte.[cancer.ucsf.edu]
  • Previous reports linking exposure to benzene with the development of multiple myeloma have been refuted. 10 Preliminary research has implicated herpesvirus type 8 in the etiology of multiple myeloma. 11 Pathophysiology A precursor cell arises in patients[aafp.org]

Prevention

  • Collaborative overview of randomised trials of antiplatelet therapy, I: Prevention of death, myocardial infarction, and stroke by prolonged antiplatelet therapy in various categories of patients. ‎[books.google.com]
  • Changes in lifestyle can help prevent many types of cancer. However, no known lifestyle changes can prevent myelomas. Immune System Health A healthy immune system remains your body's best defense.[cancertutor.com]
  • Specific immunotherapy prevents the onset of new sensitizations in children. J Allergy Clin Immunol 1997: 99:450-453. ‎ Página 150 - Barnes NC. Pujet JC.[books.google.es]
  • How can I prevent multiple myeloma? Unfortunately, because the exact cause of multiple myeloma is unknown, there are no specific guidelines for the prevention of multiple myeloma.[oncolink.org]
  • This causes an overproduction of immunoglobulins (antibody proteins) that ultimately crowd out the normal blood-forming cells and prevent them from functioning effectively. The symptoms of MM include bone lesions, anemia and kidney failure.[sebia.com]

References

Article

  1. Jako JM, Gesztesi T, Kaszas I. IgE lambda monoclonal gammopathy and amyloidosis. Int Arch Allergy Immunol. 1997;112:415-21.
  2. Johansson SG, Bennich H. Immunological studies of an atypical (myeloma) immunoglobulin. Immunology. 1967;13:381-94.
  3. Hua J, Hagihara M, Inoue M, Iwaki Y. A case of IgE-multiple myeloma presenting with a high serum Krebs von den Lungen-6 level. Leuk Res. 2012;36:e107-9.
  4. Macro M, André I, Comby E, et al. IgE multiple myeloma. Leuk Lymphoma. 1999;32:597-603.
  5. Hayes MJ, Carey JL, Krauss JC, et al. Low IgE monoclonal gammopathy level in serum highlights 20-yr survival in a case of IgE multiple myeloma. Eur J Haematol. 2007;78:353-7.
  6. Kairemo KJ, Lindberg M, Prytz M. IgE myeloma: a case presentation and a review of the literature. Scand J Clin Lab Invest. 1999;59(6):451–456.
  7. Alexander RLJr, Roodman ST, Petruska PJ, Tsai CC, Janney CG. A new case of IgE myeloma. Clin Chem. 1992;38(11):2328–2332.
  8. Galton DAG, Peto R. Report on the first myelomatosis trial. Part 1. Analysis of presenting features of prognostic importance. Br J Haematol. 1973;24(1):123–139.
  9. Dimopoulos M, Kyle R, Fermand JP, et al. Consensus recommendations for standard investigative workup: report of the International Myeloma Workshop Consensus Panel 3. Blood. 2011;117:4701-5.
  10. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology, Multiple Myeloma Version 3.2016. Available with free registration at www.nccn.org.

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Last updated: 2017-08-09 17:40