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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.

Camping
  • Prostaglandins act by modulating cyclic nucleotides, the E series increasing the level of cAMP and the F series causing a rise in cGMP. Our findings with prostaglandins could be mimicked by the relevant cyclic nucleotide.[ncbi.nlm.nih.gov]
  • Large population studies also indicate that MM, prostate cancer and malignant melanoma run in families, as do central nervous system neoplasms, although the results of some studies do not confirm this [ Eriksson, Hallberg 1992, Camp, Werner, Cannon-Albright[intechopen.com]
Constitutional Symptom
  • Patients may also report certain constitutional symptoms related to hyperviscosity. An increased incidence of infections may also be found in such patients.[symptoma.com]
Collapse
  • Onset of sudden pain may be a sign of a fracture or collapse of a vertebral body caused by the excessive growth of malignant plasma cells and their destructive nature.[forums.phoenixrising.me]
Sputum
  • […] anaphylaxis passive sensitization patients penicillin peritoneal permeability Physiol plasma present rat reagin reaginic antibody release of histamine respiratory response role saline sera serotonin serum IgE skin tests skin-sensitizing antibody specific sputum[books.google.com]
Osteopenia
  • Skeletal roentgenograms and bone scans of this patient showed osteolytic lesions and osteopenia of the thoracic and lumbar spine and osteolytic destruction of the right half of the sacrum.[ncbi.nlm.nih.gov]
Stroke
  • 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]
Somnolence
  • 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.[symptoma.com]

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]
  • الصفحة 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]
  • Work with your doctor to determine the right treatment plan, including any clinical trials There is no one standard multiple myeloma treatment plan.[themmrf.org]

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]
  • This study is particularly interesting because for the first time ever a group of researchers has found a connection between IgE levels and survival prognosis. The higher those levels, the better off you are.[margaret.healthblogs.org]
  • 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]

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]

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]
  • 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]
  • 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]
  • As myeloma cells multiply, they crowd the bone marrow and prevent it from making normal numbers of red cells (causing anaemia), white cells (increasing your susceptibility to infections) and platelets (increasing your susceptibility to bleeding and bruising[leukaemiaqld.org.au]
  • […] blood and look for infection Tissue antibody: These antibodies are present in tissues or organs all over the body and fights infections in the body Secretory antibody: These antibodies are secreted in body fluids, such as saliva and mucus, and help prevent[dovemed.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