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Archives of Internal Medicine Jan 1975The clinical and laboratory features of the seven known patients with heavy chain disease showed that all but one had chronic lymphocytic leukemia. Two common and...
The clinical and laboratory features of the seven known patients with heavy chain disease showed that all but one had chronic lymphocytic leukemia. Two common and striking features are the presence of kappa-type Bence Jones proteins and vacuolated plasma cells in the marrow.
Topics: Adult; Aged; Bence Jones Protein; Bone Marrow Examination; Female; Heavy Chain Disease; Humans; Immunoglobulin Fragments; Immunoglobulin M; Leukemia, Lymphoid; Male; Middle Aged; Plasma Cells
PubMed: 1111471
DOI: No ID Found -
Annals of Internal Medicine Sep 1971
Topics: Adult; Agammaglobulinemia; Anemia; Bence Jones Protein; Chlorambucil; Female; Fractures, Bone; Heavy Chain Disease; Hepatomegaly; Humans; Immunoelectrophoresis; Immunoglobulin A; Immunoglobulin G; Immunoglobulin M; Leukemia, Lymphoid; Lymph Nodes; Prednisone; Ribs; Splenomegaly
PubMed: 5568152
DOI: 10.7326/0003-4819-75-3-407 -
Klinicka Onkologie : Casopis Ceske a... 2020Gamma-heavy chain disease is a rare disease, described so far in approximately 150 cases. The aim of this work was laboratory dia-gnostics of immunoglobulin heavy chain...
BACKGROUND
Gamma-heavy chain disease is a rare disease, described so far in approximately 150 cases. The aim of this work was laboratory dia-gnostics of immunoglobulin heavy chain disease.
MATERIALS AND METHODS
A 60-year-old patient was referred to the University Hospital in Ostrava for suspected marginal zone lymphoma from gastric bio-psy. Staging examinations including bone marrow trepanobio-psy and PET/CT were added; special examinations required serum protein electrophoresis, immunofixation electrophoresis, determination of polyclonal immunoglobulins, free light chains, and immunoglobulin heavy/light chain pairs. Isoelectric focusing in agarose gel followed by affinity immunoblotting and SDS electrophoresis was added due to unclear findings.
RESULTS
0.1 % of plasma cells were found in the bone marrow, of which 87 % were clonal (pathological) plasma cells, followed by the cyt cytotype LAMBDA + CD38 + CD138 + CD45 + CD19 + CD56- CD27 + CD81- CD117-. Monoclonal heavy chains were found in the patients serum. No monoclonal immunoglobulin heavy or light chains were detected in urine. The PET/CT examination showed generalized lymphadenopathy, splenomegaly and inhomogeneous accumulation of fluorodeoxyglucose in axillary and appendicular skeleton, but without the presence of typical osteolytic lesions.
CONCLUSION
Monoclonal heavy chains of immunoglobulins are a rare disease. In contrast to the detection of a complete paraprotein molecule, additional methods must be used to confirm them. The finding of monoclonal heavy chain gamma in the serum of the study patient is related to the presence of marginal zone lymphoma, which was proven from a gastric bio-psy. The study was supported by the project of MH CZ - DRO - FNOs /2017 (Biobank in Teaching Hospital Ostrava) The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.
Topics: Heavy Chain Disease; Humans; Immunoglobulin gamma-Chains; Male; Middle Aged; Prognosis
PubMed: 32894957
DOI: 10.14735/amko2020280 -
American Journal of Hematology May 1992mu-Heavy chain disease (HCD) is a rare monoclonal lymphoid disorder characterized by the failure to assemble a complete IgM immunoglobulin. The mu-heavy chains analyzed... (Review)
Review
mu-Heavy chain disease (HCD) is a rare monoclonal lymphoid disorder characterized by the failure to assemble a complete IgM immunoglobulin. The mu-heavy chains analyzed to date revealed absence of the variable region and a shortened constant domain. We report the first case of mu-HCD presenting as a benign monoclonal gammopathy. The literature on the 27 reported mu-HCD cases is reviewed, and important clinical and laboratory findings are discussed. The ages of the patients ranged from 15 to 80 years (median, 57.5 years). Twenty-two of 27 patients had an associated lymphoplasma cell proliferative disorder. A monoclonal spike on routine serum protein electrophoresis was found in only 8 of 19 patients. Fourteen of 22 patients had Bence Jones proteinuria, but mu-HCD protein was reported in the urine of only two patients. The survival ranged from less than 1 month to 11 years (median, 24 months).
Topics: Heavy Chain Disease; Humans; Immunoglobulin mu-Chains; Lymphoproliferative Disorders; Male; Middle Aged
PubMed: 1566748
DOI: 10.1002/ajh.2830400112 -
International Journal of Hematology Feb 2004Heavy chain diseases (HCD) are monoclonal lymphoproliferative disorders of B-cells characterized by the synthesis of truncated heavy chains without associated light... (Review)
Review
Heavy chain diseases (HCD) are monoclonal lymphoproliferative disorders of B-cells characterized by the synthesis of truncated heavy chains without associated light chains. In patients with mu-HCD, which is a very rare form of HCD, neoplastic cells produce immunoglobulin M heavy chain. The prognosis for patients with mu-HCD is poor, and there is no specific treatment for mu-HCD. In this report, we present a patient with mu-HCD accompanied by splenomegaly and thrombocytopenia. We treated this patient with the fludarabine monophosphate therapy we use for patients with B-cell chronic lymphocytic leukemia. After 5 courses of fludarabine monophosphate treatment, the splenomegaly and thrombocytopenia improved. Fludarabine monophosphate therapy may be a new strategy to improve the prognosis of patients with mu-HCD.
Topics: Antimetabolites, Antineoplastic; Female; Heavy Chain Disease; Humans; Middle Aged; Radiography; Splenomegaly; Thrombocytopenia; Vidarabine Phosphate
PubMed: 15005347
DOI: 10.1532/ijh97.03053 -
Current Treatment Options in Oncology Jun 2002The heavy chain diseases (HCDs) are rare B-cell malignancies that are distinguished by the production of a monoclonal immunoglobulin heavy chain (HC) without an... (Review)
Review
The heavy chain diseases (HCDs) are rare B-cell malignancies that are distinguished by the production of a monoclonal immunoglobulin heavy chain (HC) without an associated light chain by the malignant B-cells. There are three types of HCD defined by the class of immunoglobulin (Ig) HC produced: IgA (alpha-HCD), IgG (gamma-HCD), and IgM (mu-HCD). Alpha-HCD is the most common and occurs most commonly as intestinal malabsorption in a young adult from a country bordering the Mediterranean Sea. Treatment consists of antibiotics and improved nutrition and hygiene. Surgery is occasionally required for patients with bulky masses at risk for bowel perforation. If there is no response to antibiotics or if aggressive non-Hodgkin's lymphoma (NHL) is diagnosed, the patient should be treated with chemotherapy. Gamma- and mu-HCD are rare and essentially are found in patients with a B-cell NHL that produces an abnormal Ig heavy chain. These patients occasionally may be diagnosed with a monoclonal gammopathy of undetermined significance (MGUS). Patients with MGUS with NHL should be administered chemotherapy. Screening the serum and urine of patients with lymphoplasmacytoid NHL would likely identify more patients with gamma- or mu-HCD.
Topics: Adult; Aged; Gene Rearrangement, B-Lymphocyte, Heavy Chain; Heavy Chain Disease; Humans; Immunoglobulin Heavy Chains; Incidence; Middle Aged; Radiotherapy
PubMed: 12057070
DOI: 10.1007/s11864-002-0014-3 -
Blood Jul 2017
Topics: Heavy Chain Disease; Humans; Immunoglobulin mu-Chains; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Neoplasm Proteins; Plasma Cells; Vacuoles
PubMed: 28751360
DOI: 10.1182/blood-2017-04-781344 -
Lancet (London, England) Apr 1965
Topics: Academies and Institutes; Heavy Chain Disease; Humans
PubMed: 14255250
DOI: No ID Found -
The New England Journal of Medicine May 1968
Topics: Heavy Chain Disease; Humans; gamma-Globulins
PubMed: 4171785
DOI: 10.1056/NEJM196805302782212 -
Annals of the New York Academy of... Dec 1971
Topics: Africa, Northern; Amino Acid Sequence; Europe; Asia, Eastern; Fucose; Heavy Chain Disease; Hexosamines; Hexoses; Humans; Immunodiffusion; Immunoelectrophoresis; Immunoglobulin A; Iran; Israel; Lymphatic Diseases; Lymphoma; Malabsorption Syndromes; Molecular Weight; Neuraminic Acids; Peptides; South America; Syria
PubMed: 5003015
DOI: 10.1111/j.1749-6632.1971.tb13558.x