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.

• Inguinal Hernia

  His past medical history was confined to high blood pressure, gastroesophageal reflux, inguinal hernia surgery, and an uninvestigated mild renal impairment. He was under antihypertension treatment by nebivolol and indapamide. [biochemia-medica.com]

• Abdominal Mass

  There have also been reports of spinal and nerve root compressions resulting in neurological deficits.[17] Patients with IgD MM presenting as a testicular tumor who subsequently developed abdominal masses and ascites have been described.[22] Chromosomal [cancernetwork.com]

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.

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.