Gamma-heavy chain disease (γ-HCD) is a rare B-cell tumor producing truncated IgG lacking the light chain. The clinical features of γ-HCD are heterogeneous, similar to lymphoplasmacytic lymphoma, and most patients have generalized and progressive disease. In some γ-HCD patients, autoimmune diseases are associated. Thus, γ-HCD as a restricted or indolent disease is exceptional. A 66-year-old male was referred to our hospital because of subungual hemorrhage at the bilateral halluces. Physical and laboratory examination results were nonspecific, and the hemorrhage was revealed to be traumatic. However, serum electrophoresis demonstrated a small M-peak, which was monoclonal IgG-Fc without the corresponding light chain on immunofixation and immunoelectrophoresis. Bone marrow aspirate demonstrated a small number of lymphoplasmacytic cells that were positive for CD19, CD38, CD138, and cyIgG, but negative for cyκ- and -λ light chains on flow cytometry. A diagnosis of γ-HCD was made. Chest and abdominal CT demonstrated neither hepatosplenomegaly, lymphadenopathy, nor bone lytic lesions. The serum concentrations of IgG and M-peak configuration have remained relatively unchanged for nearly 3 years. Therefore, this γ-HCD may correspond to a rare form of monoclonal gammopathy with undetermined significance.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6798140PMC
http://dx.doi.org/10.3960/jslrt.19008DOI Listing

Publication Analysis

Top Keywords

gamma-heavy chain
8
monoclonal gammopathy
8
gammopathy undetermined
8
undetermined significance
8
disease γ-hcd
8
light chain
8
demonstrated small
8
γ-hcd
6
chain monoclonal
4
significance mgus
4

Similar Publications

CDR identification, epitope mapping and binding affinity determination of novel monoclonal antibodies generated against human apolipoprotein B-100.

Biochim Biophys Acta Proteins Proteom

January 2025

Biochemistry-Electrochemistry Research Unit, School of Chemistry, Institute of Science, Suranaree University of Technology, Nakhon Ratchasima 30000, Thailand. Electronic address:

In-house generated mAbs to apolipoprotein B-100 (apoB-100) clones hLDL-E8, hLDL-2D8 and hLDL-F5 were extensively studied to determine their complementarity-determining regions (CDRs), binding epitopes and affinity. RT-PCR revealed that all mAbs consisted of kappa light chains and gamma heavy chains. DNA sequencing and bioinformatic analysis showed that the variable gene and protein sequences of their CDRs shared over 50 % identity with the existing databases.

View Article and Find Full Text PDF
Article Synopsis
  • Monoclonal immunoglobulin deposition disease (MIDD) affects systemic tissues, primarily the kidneys, and is categorized into three types: light-chain, mixed light and heavy chain, and heavy-chain deposition disease.
  • A 32-year-old man with skin laxity and nephritic syndrome was diagnosed with heavy-chain deposition disease after renal biopsy and underwent successful treatment with bortezomib, dexamethasone, and thalidomide.
  • Long-term follow-up of four years showed significant improvement in kidney function and overall health of the patient, emphasizing a rare but positive outcome in HCDD cases.
View Article and Find Full Text PDF

Gamma heavy chain disease (gHCD) is a rare B-cell lymphoproliferative disorder that mostly occurs after childbearing age. Here we report the first case of gHCD in a pregnant patient that was diagnosed in the second trimester, and another pregnancy in the same patient after initial treatment for gHCD. The former pregnancy ended in intrauterine fetal death, believed to be caused by insufficient maternal blood flow due to multiple placental infarcts.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!