μ‐heavy chain disease: Presentation as a benign monoclonal gammopathy

Wahner‐Roedler, Dietlind L.; Kyle, Robert A.

doi:10.1002/ajh.2830400112

Cited by 41 publications

(13 citation statements)

References 29 publications

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“…As described previously for other patients with m HCD, there was no lymphadenopathy [16]. k-light chains were seen in the urine, as reported before [10].…”

Section: Discussionmentioning

confidence: 51%

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A case of μ heavy-chain disease associated with hyperglobulinemia, anemia, and a positive Coombs test

Witzens

Egerer

Stahl

et al. 1998

Annals of Hematology

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We report here for the first time a patient with mu heavy-chain disease (HCD), hyperimmunoglobulinemia, and a positive direct antiglobulin test (DAT, Coombs test). The heavy-chain diseases involve the proliferation of lymphoplasma cells of B cell origin and are characterized by the production of incomplete heavy chains devoid of light chains. The association of mu heavy-chain disease with either hyperglobulinemia or a positive DAT has not been reported in the literature to date. In this patient, immunofixation of serum proteins with monospecific antisera to alpha-, gamma-, mu,- or delta-chains and to kappa- and lambda-chains revealed a precipitation band with antibody to IgM, but not with kappa and lambda light-chain antibodies, indicating mu heavy-chain disease. Hyperglobulinemia was present, which is very uncommon for HCD. A DAT of the patient's red blood cells (RBC) was found to be strongly positive for anti-IgG but negative for anti-IgM, -IgA, -C3c, and -C3d. However, when the eluate from the patient's red blood cells was investigated with nephelometry, it was found to contain antigens reactive with anti-y as well with anti-mu-antiserum. When a DAT was performed with a randomly chosen test cell incubated with the eluate, the antibody-containing eluate was shown to react with anti-IgG as well as with anti-IgM-antiserum. In summary, the eluate from the patient's RBCs contained IgG and an immunoglobulin structure reactive with anti-IgM in an RBC agglutination assay as well as with anti-mu antiserum in a nephelometric investigation. Whether this IgM on the patient's erythrocytes is penta- or oligomeric, complete IgM, or the heavy chain cannot be concluded from these observations.

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“…As described previously for other patients with m HCD, there was no lymphadenopathy [16]. k-light chains were seen in the urine, as reported before [10].…”

Section: Discussionmentioning

confidence: 51%

“…Since the first report appeared in 1969 [5] only 27 additional cases have been documented in the world literature [16]. Almost all reported patients with m HCD present with lymphadenopathy and constitutional symptoms; in 60% splenomegaly is present.…”

Section: Introductionmentioning

confidence: 98%

A case of μ heavy-chain disease associated with hyperglobulinemia, anemia, and a positive Coombs test

Witzens

Egerer

Stahl

et al. 1998

Annals of Hematology

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“…Its clinical features resemble chronic lymphocytic leukemia. Vacuolated lymphocytes in the bone marrow are characteristic [ 11 ]. Unlike alpha and gamma heavy chain diseases, mu heavy chain disease can be associated with increased free light chain excretion as some tumor cells seem to have a defect in the assembly of both light and heavy chains.…”

Section: Discussionmentioning

confidence: 99%

Two Cases of Heavy Chain MGUS

Keer

Meijers

Delforge

et al. 2016

Case Reports in Oncological Medicine

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Heavy chain diseases are rare variants of B-cell lymphomas that produce one of three classes of immunoglobulin heavy chains, without corresponding light chains. We describe two patients with asymptomatic heavy chain monoclonal gammopathy. The first patient is a 51-year-old woman with alpha paraprotein on serum immunofixation. The second case is a 46-year-old woman with gamma paraprotein on urine immunofixation. Neither patient had corresponding monoclonal light chains. Workup for multiple myeloma and lymphoma was negative in both patients. These two cases illustrate that heavy chain monoclonal gammopathy can exist in the absence of clinically apparent malignancy. Only a few reports of “heavy chain MGUS” have been described before. In the absence of specialized guidelines, we suggest a similar follow-up as for MGUS, while taking into account the higher probability of progression to lymphoma than to myeloma.

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“…Th e μ heavy chain disease is a rare lymphoproliferative disorder with clonal IgM molecules with defective variable region [101][102][103] . Clinically, it resembles chronic lymphocytic leukemia and is often associated with hepatosplenomegaly [1] .…”

Section: Plasma Cell Myeloma and Related Disordersmentioning

confidence: 99%