Heavy Chain Disease

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130 relevant articles (3 outcomes, 12 trials/studies) found for this Disease

Description: A disorder of immunoglobulin synthesis in which large quantities of abnormal heavy chains are excreted in the urine. The amino acid sequences of the N-(amino-) terminal regions of these chains are normal, but they have a deletion extending from part of the variable domain through the first domain of the constant region, so that they cannot form cross-links to the light chains. The defect arises through faulty coupling of the variable (V) and constant (C) region genes.

Also Known As:

Franklin's Disease; Franklins Disease; Heavy Chain Diseases; gamma Chain Disease; gamma-Chain Diseases; mu Chain Disease; mu-Chain Diseases; Franklin Disease; gamma-Chain Disease; mu-Chain Disease

Relationship Network

Disease Context: Research Results

Immune System Diseases: 1319

Immunoproliferative Disorders: 26

Paraproteinemias: 837

Heavy Chain Disease: 130

Immunoproliferative Small Intestinal Disease: 75

human BOT heavy chain disease protein

human CHA heavy chain disease protein

human FOR heavy chain disease protein

human heavy chain disease proteins

human RIV immunoglobulin variant protein

human Wis heavy-chain disease protein

Related Diseases

1.	Lymphoma (Lymphomas)
2.	Waldenstrom Macroglobulinemia (Macroglobulinemia)
3.	Immunoproliferative Small Intestinal Disease
4.	Precursor Cell Lymphoblastic Leukemia-Lymphoma (Acute Lymphoblastic Leukemia)
5.	Cryoglobulinemia

Experts

1.	Sugimoto, Naoshi: 1 article (02/2004)
2.	Maeda, Akinori: 1 article (02/2004)
3.	Yanai, Machi: 1 article (02/2004)
4.	Oida, Tatsuo: 1 article (02/2004)
5.	Takahashi, Takayuki: 1 article (02/2004)
6.	Watanabe, Naoko: 1 article (02/2004)
7.	Nagai, Kenichi: 1 article (02/2004)
8.	Matsushita, Akiko: 1 article (02/2004)
9.	Takaoka, Naomi: 1 article (09/2002)
10.	Murakawa, Koichi: 1 article (09/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Heavy Chain Disease:

1.	Chlorambucil (Leukeran)FDA Link 11/19/1977 - "[Franklin's disease: remission after 3 months using chlorambucil]" 09/01/1980 - "After therapy with chlorambucil, the level of the heavy chain disease protein in the serum decreased substantially." Order ALL the reference details at left...
2.	Prednisone (Sone)FDA LinkGeneric 07/01/1975 - "Gamma heavy chain disease: rapid, sustained response to cyclophosphamide and prednisone." 07/01/1975 - "A patient, CAL, with gamma heavy chain disease is presented who has had a complete remission lasting over 2 yr with combination chemotherapy consisting of pulsatile cyclophosphamide and prednisone. " Order ALL the reference details at left...
3.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 07/01/1975 - "Gamma heavy chain disease: rapid, sustained response to cyclophosphamide and prednisone." 07/01/1975 - "A patient, CAL, with gamma heavy chain disease is presented who has had a complete remission lasting over 2 yr with combination chemotherapy consisting of pulsatile cyclophosphamide and prednisone. " Order ALL the reference details at left...
4.	Adrenal Cortex Hormones (Corticosteroids)IBA 02/15/1984 - "A patient with evidence at presentation of alpha-heavy chain disease and an immunoblastic lymphoma is presented, who had remained in clinical, histologic, and immunologic remission for more than 12 years following initial radiotherapy and corticosteroids. " Order ALL the reference details at left...
5.	Immunoglobulins (Immunoglobulin)IBA 07/01/1990 - "Evidence that membrane immunoglobulin alterations contribute to oncogenesis in vivo comes from studies of heavy chain diseases. " 11/01/1989 - "Immunocytologic studies showed that a gamma heavy chain disease protein may occur in the context of a double monoclonal lymphoproliferative process or in various B or T cell malignancies that are not directly involved in the production of the abnormal immunoglobulin. " 03/01/2006 - "The presence of abnormal serum immunoglobulin composed of only gamma heavy chain has been known as a fundamental feature of gamma heavy chain disease (HCD). " 03/01/1993 - "An insertion-deletion event in murine immunoglobulin kappa gene resembles mutations at heavy-chain disease loci." 09/01/1992 - "Patients with gamma heavy chain disease (gamma-HCD) generally produce incomplete immunoglobulin (Ig) gamma-heavy chains (gamma-HCD protein) which cannot associate with light chains (IgL). " Order ALL the reference details at left...
6.	Proteins (Proteins, Gene)IBA 12/31/1971 - "Structural studies of gamma heavy chain disease proteins." 03/03/1998 - "These deletions/insertions seem to be the main cause for the generation of heavy chain disease proteins. " 10/01/1995 - "The self-aggregation of mu chain disease proteins might account for this constitutive activation. " 06/01/1991 - "[Gamma-1 heavy chain disease with the demonstration of Bence-Jones proteins]" 01/01/1984 - "Heavy chain disease proteins (FOR) were isolated from human plasma. " Order ALL the reference details at left...
7.	Immunoglobulin G (IgG)IBA 03/01/1997 - "Analysis of serum protein resulted in the diagnosis of gamma-chain disease (total protein 6.2 g/dl, M-protein positive, IgG 4150 mg/dl, IgG-Fc fragment positive). " 01/01/1994 - "Western blotting revealed that MAb 200D1, bound to intact IgG3 heavy chain disease protein and not its pFc' fragment; a result consistent with the CH2 domain location of the G3m(g) allotope. " 01/01/1989 - "The immunochemical and structural analysis of an IgG1 heavy chain disease is reported. " 01/01/1989 - "Immunochemical and structural characterization of an IgG1 heavy chain disease." 09/01/1980 - "Chronic lymphocytic leukaemia and IgG heavy chain disease." Order ALL the reference details at left...
8.	Immunoglobulin A (IgA)IBA 10/01/1994 - "Fragments of the heavy alpha-chain of IgA (IgA Fc alpha) were prepared and purified from the serum of patients with heavy chain disease. " 12/01/1988 - "Elevated serum IgA associated with immunoproliferative enteropathy of Basenji dogs: lack of evidence for alpha heavy-chain disease or enhanced intestinal IgA secretion." 03/01/1975 - "IgA heavy chain disease. " 03/01/1975 - "Immunoelectrophoresis showed the pattern of IgA heavy chain disease. " 01/01/1972 - "[Problems of differential diagnosis of IgA myeloma and heavy chain disease]" Order ALL the reference details at left...
9.	Immunoglobulin M (IgM)IBA 07/12/1980 - "[Low molecular weight (7S) IgM in the sera of patients with the alpha heavy chain disease (HCD) (author's transl)]" 08/21/1970 - "Immunoglobulin M heavy chain disease: intracellular origin of the mu chain fragment." 06/01/1978 - "Monoclonal IgM cryoglobulinemia associated with gamma-3 heavy chain disease: immunochemical and biochemical studies." 05/01/1992 - "mu-Heavy chain disease (HCD) is a rare monoclonal lymphoid disorder characterized by the failure to assemble a complete IgM immunoglobulin. " 01/01/1984 - "[Alpha heavy chain disease in the presence of a monomeric IgM without excretion of abnormal immunoglobulins]" Order ALL the reference details at left...
10.	Messenger RNA (mRNA)IBA 11/01/1992 - "In this study we established the nucleotide sequence of alpha mRNA for six cases of alpha heavy chain disease. " 06/19/1993 - "The NH2-terminal structure of serum abnormal protein, as well as the sequence of the corresponding mRNA, were determined in a new case of alpha heavy chain disease. " 11/01/1992 - "Alpha heavy chain disease alpha mRNA contain nucleotide sequences of unknown origins." 10/01/1978 - "Gamma heavy chain disease in man: translation and partial purification of mRNA coding for the deleted protein." 01/01/1991 - "A series of mouse myeloma cell lines producing mutant gamma 2b immunoglobin heavy chains, which resemble heavy chain disease proteins, were analyzed for messenger RNA abundance as a function of mRNA alterations. " Order ALL the reference details at left...

Therapies and Procedures

1.	Combination Drug Therapy (Combination Chemotherapy) 04/01/1986 - "This case supports the notion of Kyle et al that heavy-chain disease may present a diverse picture, and documents the first use of aggressive combination chemotherapy in the management of associated moderately severe hypercalcemia and hypercalcemic renal failure." 07/01/1975 - "A patient, CAL, with gamma heavy chain disease is presented who has had a complete remission lasting over 2 yr with combination chemotherapy consisting of pulsatile cyclophosphamide and prednisone. " Order ALL the reference details at left...
2.	Nutritional Support (Artificial Feeding) 09/01/1988 - "Severe malnutrition associated with alpha-heavy chain disease: response to tetracycline and intensive nutritional support." Order ALL the reference details at left...
3.	Laparotomy 09/01/1988 - "A diagnosis of alpha-heavy chain disease (alpha-HCD) was made on endoscopic duodenal biopsy and serology--lymphoma being excluded by scanning and laparotomy. " Order ALL the reference details at left...
4.	Drug Therapy (Chemotherapy) 12/01/2008 - "Combination of rituximab and chemotherapy showing anti-tumor effect in gamma heavy chain disease expressing CD20." Order ALL the reference details at left...

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