IgE Multiple Myeloma Research Articles

Defects and countermeasures in laboratory diagnosis of rare IgE multiple myeloma

BackgroundIgE multiple myeloma (MM) is a rare subtype of MM that is easily misdiagnosed. We report a rare case of IgE-MM and investigate the application of the SLiM-CRAB criteria to screen for high-risk smoldering MM (SMM) patients, so as to summarize the causes and methods used to prevent missed diagnosis or misdiagnosis in IgE-MM. MethodsThe serum monoclonal protein (M-protein) classification and IgE quantification was performed and sent to several individual institutions. The results were collected and the causes of IgE detection defects were analyzed. ResultsUpon admission to our hospital, the patient’s serum free kappa light chain was 1069.9 mg/L, free lambda light chain was 9.2 mg/L, and free kappa/lambda ratio was 115.9, which met the SLiM criteria, but without CRAB features. Immunofixation electrophoresis (IF) showed “M-like protein aggregation bands” in all lanes. After pretreatment with 1% β-mercaptoethanol to depolymerize the aggregation of monoclonal protein, the “M-like protein aggregation bands disappeared. The other five institutions did not provide the correct typing results. The quantification of serum IgE was as high as 2.06 × 107 IU/mL, whereas 7 other testing institutions reported IgE levels ranging from 1.0 to 1100 IU/mL. ConclusionHigh-risk biomarkers in SLiM criteria can achieve good therapeutic effects in rare IgE-MM patients. Serum immunofixation performed without antisera against IgE, insufficient identification of the lytic bands produced by high macromolecule aggregation in IF, and the absence of a prozone effect avoidance procedure during IgE quantitative detection are the primary causes of missed diagnosis or misdiagnosis in patients with IgE-MM.

Unusual case of IgE multiple myeloma: case report

Unusual case of IgE multiple myeloma: case report

ID: 1075 Concentration and κ/λ ratio of serum free light chain in multiple myeloma patients at Cho Ray Hospital

Introduction: multiple myeloma (MM) is a B cell malignancy which characterized by accumulation of plasmocyte in bone marrow, immunoglobulins and free light chain in serum (sFLC). According to IMMWG (International multiple myeloma working group), sFLC is a criterion in diagnosis of MM. In this study, we investigate the concentration and κ/λ ratio of sFLC in newly diagnosed MM patients at Cho Ray hospital. Methods: cross-sectional study. Serum samples of 36 newly diagnosed MM patients were analyzed by Binding Site Freelite method to measure the amount of κ and λ sFLC.
 
 Results: the median age of 36 MM patients was 61 yrs (41 – 88 yrs), with the male/female ratio was 1.2/1. MM stage III, stage II and stage I accounting for 63.9% (23/36), 27.8% (10/36), and 8.3% (3/36), respectively. Clonal IgG, IgA and IgE MM accounting for 61.1% (22/36), 30.6% (11/36), and 8.3% (3/36), respectively. We reported the median of plasmocyte in 36 cases was 35.4% (95%CI: 27.6 – 43.3). The median of concentration of κ sFLC, λ sFLC, and involved/uninvolved sFLC ratio were 1601.8 mg/L (95%CI: 104.9 – 3098.7), 900.1 mg/L (95%CI: 10.7 – 1789.5), and 191.0 (95%CI: 87.6 – 294.4), respectively. The abnormally high in sFLC concentration was reported in 97.2% MM cases (35/36), in which 55.6% cases (20/36) had increased κ sFLC, 19.4% cases (7/36) had increased λ sFLC, and 22.2% cases (8/36) had increased both κ+λ sFLC concentration. Increased involved/uninvolved sFLC ratio was found in 94.4% MM cases (34/36), in which 36.1% cases (13/36) had sFLC ratio > 100, with all involved sFLC concentration > 100 mg/L. In 3 cases of MM with kidney failure, 2 cases had increased both κ+λ sFLC, 1 case had increased κ sFLC concentration; and all of 3 cases had involved sFLC level > 5300 mg/L with sFLC ratio > 100. No significant difference of sFLC concentration and sFLC ratio among gender, stage of disease, Ig clone, or plasmocyte percent in bone marrow of MM patients. Conclusions: due to the abnormally high of sFLC concentration and sFLC ratio in MM, it is necessary to monitor frequently these parameters during treatment to prevent the risk of kidney failure for patients.

Clinical features and efficacy of IgE multiple myeloma

目的 探讨IgE型多发性骨髓瘤（MM）的临床特征及治疗效果。 方法 收集蚌埠医学院附属连云港医院收治的1例以及文献报道的6例IgE型MM患者的临床资料，分析其临床表现、实验室特点及治疗效果。 结果 7例MM患者中，男性5例，女性2例，年龄40~59岁，平均年龄（52.4±6.0）岁；临床表现：贫血5例，肾损害3例，感染2例，高钙血症2例，骨骼疼痛5例，均呈溶骨性破坏，4例伴有病理性骨折；Durie-Salmon分期：ⅡA期4例，ⅢB期3例；血清免疫固定电泳分型：IgE-κ型3例，IgE-λ型4例；治疗效果：6例接受联合化疗和（或）自体外周血造血干细胞移植，完全缓解3例，部分缓解2例，疾病稳定1例，其中3例接受硼替佐米或造血干细胞移植治疗均获完全缓解。 结论 IgE型MM非常罕见，准确分型依赖血清免疫固定电泳，基于硼替佐米以及造血干细胞移植的治疗效果良好。

Bortezomib plus melphalan and prednisone (VMP regimen) as the initial treatment for IgE multiple myeloma: a case study

Patients with IgE multiple myeloma have poor prognosis. Due to the rarity of this condition, no standard treatment has been developed. As the VMP regimen (bortezomib plus melphalan and prednisone) has been reported to be highly effective in the treatment of other types of myeloma, we treated a patient with newly diagnosed IgE myeloma using the VMP regimen. Her myeloma responded well to VMP regimen. The results of this case report thus suggest that physicians may consider VMP regimen for use as the initial treatment for IgE myeloma.

An unusual case of IgE-multiple myeloma presenting with systemic amyloidosis 2 years after cervical plasmacytoma resection

A 69-year-old man suffered from a fractured cervical vertebra, and magnetic resonance imaging revealed a solitary mass occupying the injured lesion. Surgical resection of the mass was conducted, and the infiltration of plasma cells expressing IgE-lambda monoclonal protein was shown on pathological investigation. Concurrently, a monoclonal band of IgE-lambda was shown in the sera on immunoelectrophoresis, and the proliferation of plasma cells (more than 10%) was also detected in bone marrow aspirates. A rare IgE-multiple myeloma (MM) was diagnosed, and careful monthly follow-up was started. During this observation period, the level of IgE gradually increased and, at 2 years after the initial diagnosis, bilateral leg edema and ascites with marked hepatosplenomegaly appeared. Combination chemotherapy of melphalan and prednisolone (MP) was started, which resulted in the partial, transient resolution of symptoms. Additional treatment with bortezomib and dexamethasone could neither resolve these clinical symptoms nor decrease the serum IgE concentration. At 5 months after the start of chemotherapy, amyloid deposition was suggested based on typical echocardiographic findings, and the diagnosis of amyloidosis was confirmed based on the histopathology of a liver biopsy. In spite of MP plus thalidomide treatment, cardiac failure due to amyloidosis aggravated, and the patient died of multiple organ failure. This is the second reported case in which IgE-MM was complicated by systemic amyloidosis.

IgE Myeloma: the First Report of Chinese Case and a Review of the Literature

Objective: To report a case of IgE myeloma and to compare its clinical features with those reported in the literature. Methods: M-component in serum and urine was determined using cellulose- acetate membrane electrophoresis, immunofixation electrophoresis and quantification of immunoglobulins. Immunohistochemistry was performed to detect the expression of IgE and its light chain. Results: A monoclonal peak was detected by cellulose-acetate membrane electrophoresis. The monoclonal band showed on immunofixation electrophoresis the following reactive patterns: positive for λ antisera and negative for κ, IgG, IgA, IgM and IgD antisera. The serum immunoglobulin concentrations were: IgG 21.6 g/L, IgA 1.2 g/L, IgM 2.64 g/L, κ 7.49 g/L, λ 16.0 g/L, κ/λ 0.47. The results of immunohistochemistry showed cytoplasmic expression of IgE immunoglobulin and λ light chain in the tumor tissue.Conclusion: This is the first case of IgE multiple myeloma reported in China.

A case of IgE multiple myeloma

A case of IgE multiple myeloma

A case of IgE multiple myeloma

A case of IgE multiple myeloma

A case of IgE multiple myeloma

A case of IgE multiple myeloma

IgE Multiple Myeloma

IgE multiple myeloma is a rare disease characterized by a high frequency of Bence-Jones proteinuria and plasma cell leukaemia when compared to other isotypes of monoclonal proteins. Only 35 cases have been reported. We describe a 70-year-old woman with a stage III IgE kappa multiple myeloma presenting with a sacral plasmacytoma. Immunological and biochemical studies showed IgE kappa producing tumoral plasma cells. Serum total IgE was high without clinical symptoms suggesting an hyperlgE syndrome or mast cell activation. The patient underwent surgical removal of the sacral tumor and monthly melphalan-prednisone treatment together with intravenous pamidronate infusions. Magnetic Resonance Imaging (MRI) of the dorsolumbar spine revealed an epidural process leading to T6-T9 radiotherapy. Bone densitometry showed a decreased bone mineral content supporting the management of myeloma-related osteoporosis with bisphosphonate infusions.A good partial response with plateau-phase and increase of bone mineral content was achieved after 1 year of treatment and still persists after a 28 months follow-up.

IgE Multiple Myeloma: A Report of the Third Case

Abstract The third known case of IgE multiple myeloma is described. The patient was a 65-yr-old woman who died 11 mo after a diagnosis of multiple myeloma was made by a surgical biopsy of the left iliac crest. Her serum contained approximately 2.7 g/100 ml of a paraprotein of mid-γ mobility. The isolated anomalous protein gave a reaction of identity with the IgE myeloma protein of the second reported case when reacted with a specific IgE antiserum. The light-chain type of the myeloma protein was κ in contrast to λ of the other two IgE myeloma proteins. Her clinical manifestations differed from the two previously reported cases in several respects. She had neither plasma cell leukemia nor overt Bence Jones proteinuria and developed rapidly progressive widespread osteosclerotic lesions that were associated with bone pain.

IgE Myeloma: Total Body Tumor Cell Number and Synthesis of IgE and DNA

Abstract Studies of myeloma cell DNA and immunoglobulin synthesis and total body tumor cell number were performed on a patient with IgE multiple myeloma and plasma cell leukemia; results were contrasted to similar observations in patients with IgG myeloma. Autoradiographic studies demonstrated significantly greater DNA labeling in the IgE patient’s bone marrow plasma cells than in cells from IgG myeloma patients who did not have leukemic manifestations; an additional patient with plasma cell leukemia also had a high labeling index of the myeloma cells in the blood. The IgE myeloma patient’s molecular synthesis rate for immunoglobulin averaged 26,000 molecules of IgE per minute per myeloma cell, a synthetic rate similar to that found in IgG myeloma. These observations have relevance to the normal immune response, as they can be used to approximate the number of normal IgE- and IgG-producing cells in the body. In the patient with IgE myeloma, the total body myeloma cell number was 2.7 x 1012 cells at the time of study and was of the same order of magnitude as in IgG myeloma; however, the distribution of tumor cells was strikingly different. Serial estimates of tumor cell number were made; such estimates may prove useful in evaluation and treatment of patients with multiple myeloma.