Heavy chain disease—A new disorder of serum γ-globulins

Franklin, Edward C.; Lowenstein, Jerome; Bigelow, Bradley; Meltzer, M.

doi:10.1016/0002-9343(64)90191-3

Cited by 330 publications

(73 citation statements)

References 38 publications

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“…To evaluate the role of sialic acid moieties on its electrophoretic heterogeneity, mildy reduced and alkylated T. L. protein was treated with neuraminidase (Behring)3 incubating 1 (27).…”

Section: Methodsmentioning

confidence: 99%

“…When Franklin reported the first case of av-heavy-chain disease, an uncommon form of malignant lymphoma characterized by the presence in serum and urine of a naturally occurring immunoglobulin representing mainly the Fc-fragment of the '-ychain, he anticipated that similar conditions involving a-and A-chains would ultimately be identified (1,2). In fact, a new type of immunoglobulin abnormality, characterized by the presence in serum and urine of a protein devoid of light chains and closely related to the heavy polypeptide chains of -yAl-globulin, has recently been described in this laboratory (3).…”

Section: Introductionmentioning

confidence: 99%

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Immunochemical studies in four cases of alpha chain disease

Séligmann¹,

Mihaesco²,

Hurez³

et al. 1969

J. Clin. Invest.

114

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A B S T R A C T Studies of a number of properties of the pathological -vA-proteins in the first four cases of the recently recognized alpha-chain disease demonstrate that, as in -v-heavy-chain disease, the abnormal protein is devoid of light chains and represents a portion of the a-heavy chain related to the Fc-fragment. In two patients, serum electrophoresis showed a broad abnormal band, whereas in the two others the pathological protein was not noticeable on the electrophoretic pattern. The diagnosis of a-chain disease can be established without purification of the protein by immunoelectrophoresis and gel diffusion experiments using selected antisera to 'vA and a reference a-chain disease protein. All four proteins belonged to the al-subclass, displayed electrophoretic heterogeneity. and showed a strong tendency to polymerize. The polymers occurred in vivo and were held together both by disulfide bonds and by strong noncovalent forces. Two of the three purified proteins had a very high carbohydrate content. The abnormal protein was always found in concentrated urines in variable but generally low amounts. It was not detected in parotid saliva but was present in significant amounts in jejunal fluid of all four patients. The a-chain disease protein was shown to be associated with the secretory piece in external secretions of two patients.The clinicopathological features were strikingly similar in the four patients. All patients were affected with a neoplastic and mostly plasmacytic proliferation involving primarily the whole length of the small intestine and the mesenteric nodes and all exhibited a severe malabsorption syndrome. While Israeli authors have emphasized the frequency of this type of abdominal lymphoma in young Arabs and non-Ashkenazi Jews, two of our patients were Kabyles, one a Syrian Arab, and one an Eurasian. Cellular studies showed that the pathological Dr. Rambaud's present address is

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“…To evaluate the role of sialic acid moieties on its electrophoretic heterogeneity, mildy reduced and alkylated T. L. protein was treated with neuraminidase (Behring)3 incubating 1 (27).…”

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Immunochemical studies in four cases of alpha chain disease

Séligmann¹,

Mihaesco²,

Hurez³

et al. 1969

J. Clin. Invest.

114

View full text Add to dashboard Cite

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“…By contrast, in G3-myeloma and in heavy chain disease (J. M.), most of the plasma cells in the (18). These data indicate that teins (1)(2)(3)(4)(5)19). The occurrence of about half of a there is a disturbance in the control of normal immunoy-heavy chain in HCD, which is related to the Fc-frag-globulin synthesis to the extent that structural genes for ment of IgG, raises important questions about the forma-light chains are not expressed in the involved cells.…”

Section: (B) Cellular Studiesmentioning

confidence: 99%

Biosynthetic And structural studies of a heavy chain disease protein

Ein¹,

Buell²,

Fahey³

1969

J. Clin. Invest.

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“…1C), a finding consistent with the diagnosis of ␥-heavy chain disease (␥-HCD). HCDs are rare proliferative B-cell disorders characterized by the production of monoclonal proteins with incomplete heavy chain components and without associated light chains (1 ). HCDs involving the 3 main immunoglobulin classes (IgA, IgM, IgG) have been described.…”

Section: Discussionmentioning

confidence: 99%

“…The serum protein electrophoresis had been performed by capillary electrophoresis, a technique in which radiocontrast dyes and antibiotics can create spikes that mimic an M protein (1)(2)(3)(4)(5). Such spikes are typically quite discrete, however.…”

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confidence: 99%

Unusual Increased β-Globulins in an Elderly Patient

et al. 2011

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A 90-year-old man who had a history of transurethral prostate resection for benign prostatic hyperplasia was admitted for left hip pain. A hip radiography examination revealed a large osteolytic lesion in the left greater trochanter, and a bone scintigraphy evaluation showed increased activity in the same area. Because of the patient's history, metastasis from prostate carcinoma was suspected, and bone biopsies were performed the same week. Ten days later, the patient was hospitalized in the emergency department after a fall that fractured the femoral neck. At admission, plasma sodium, chloride, and potassium concentrations were all within their respective reference intervals. The plasma concentration of total protein was 5. 4 showed the presence of an unusual spike in the ␤ region (Fig. 1A). We measured an increased urine concentration of free light chain [1.4 mg/dL (14 mg/L); reference interval, 0 -0.2 mg/dL (0 -2 mg/L)], along with a normal concentration of urine ␤ 2 -microglobulin.The patient underwent surgical repair of his left femur. In the meantime, our laboratory asked for serum to complete the investigation of the abnormal UPEP pattern. Investigations of the serum sample revealed a decreased albumin concentration [1.45 g/dL (14.5 g/L); reference interval, 3.0 -4.5 g/dL (30 -45 g/L)]. Immunoglobulin quantification revealed an increased IgG value [2950 mg/dL (29.5 g/L); reference interval, 700 -1000 mg/dL (7-10 g/L)]. IgA and IgM were within their respective reference intervals. Serum protein electrophoresis (SPEP) showed an increased ␣1 region, along with decreased albumin and ␥ regions. Moreover, we found an increased ␤ region (19.1 g/L, quantified from the electrophoresis trace and the total protein concentration), along with a loss of separation between the ␤1 and ␤2 regions because of a narrow spike (Fig. 1B). With a normal concentration of ␤-globulins of approximately 300 mg/dL (3 g/L), we estimated the monoclonal protein concentration at approximately 1600 mg/dL (16 g/L). Serum immunofixation was performed with antibodies specific for heavy chains (G, A, M) and light chains (, ). A band was present in the IgG lane with a ␤ electrophoretic mobility, with no corresponding band for the light chain (Fig. 1C). Serum free light chains included a decreased free chain [0.06 mg/dL (0.6 mg/L); reference interval, 0.33-1.94 mg/dL (3.3-19.4 mg/L)], a decreased free

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Heavy chain disease—A new disorder of serum γ-globulins

Cited by 330 publications

References 38 publications

Immunochemical studies in four cases of alpha chain disease

Immunochemical studies in four cases of alpha chain disease

Biosynthetic And structural studies of a heavy chain disease protein

Unusual Increased β-Globulins in an Elderly Patient

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