RESUMO
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.
Assuntos
Doença das Cadeias Pesadas/diagnóstico , Cadeias gama de Imunoglobulina/sangue , Doença das Cadeias Pesadas/sangue , Humanos , Masculino , Pessoa de Meia-Idade , PrognósticoAssuntos
Xantogranuloma Necrobiótico/diagnóstico , Paraproteinemias/diagnóstico , Administração Intravenosa , Adulto , Biópsia , Sedimentação Sanguínea , Medula Óssea/patologia , Ciclofosfamida/administração & dosagem , Pálpebras , Feminino , Humanos , Cadeias gama de Imunoglobulina/sangue , Imunoglobulinas Intravenosas/administração & dosagem , Xantogranuloma Necrobiótico/sangue , Xantogranuloma Necrobiótico/tratamento farmacológico , Xantogranuloma Necrobiótico/etiologia , Paraproteinemias/sangue , Paraproteinemias/complicações , Paraproteinemias/tratamento farmacológico , Pele/patologiaAssuntos
Anticorpos Monoclonais/imunologia , Fator Estimulador de Colônias de Granulócitos e Macrófagos/antagonistas & inibidores , Fator Estimulador de Colônias de Granulócitos e Macrófagos/imunologia , Paraproteinemias/diagnóstico , Paraproteinemias/imunologia , Proteinose Alveolar Pulmonar/tratamento farmacológico , Anticorpos Monoclonais/sangue , Autoanticorpos/sangue , Autoanticorpos/imunologia , Biópsia , Medula Óssea/patologia , Cromatografia Líquida , Humanos , Imunoglobulina G/sangue , Imunoglobulina G/imunologia , Cadeias gama de Imunoglobulina/sangue , Cadeias gama de Imunoglobulina/imunologia , Masculino , Gamopatia Monoclonal de Significância Indeterminada/sangue , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Paraproteinemias/sangue , Proteinose Alveolar Pulmonar/diagnóstico , Proteinose Alveolar Pulmonar/etiologia , Espectrometria de Massas em Tandem , Tomografia Computadorizada por Raios XAssuntos
Anticorpos Monoclonais/uso terapêutico , Eletroforese das Proteínas Sanguíneas/métodos , Cadeias gama de Imunoglobulina/sangue , Cadeias kappa de Imunoglobulina/sangue , Mieloma Múltiplo/tratamento farmacológico , Paraproteínas/análise , Artefatos , Humanos , Cadeias gama de Imunoglobulina/análise , Estudos RetrospectivosRESUMO
There are limited data on serum total light chain (sTLC) in lymphoma and its relative role on the outcome of diffuse large B cell lymphoma (DLBCL) patients. Blood samples from 46 cases newly diagnosed with DLBCL were collected consecutively during chemotherapy to detect sTLC, IgG, IgA, and IgM levels. Clinical data and survival outcomes were analyzed according to the results of sTLC measurements. In summary, 22 patients (47.8 %) had abnormal k or λ light chain, respectively, and 6 patients (13.0 %) had both abnormal k and λ light chains before chemotherapy. Patients with elevated k light chain more frequently displayed multiple extra-nodal organ involvement (P = 0.01) and had an inferior overall survival (OS) (P = 0.041) and progression-free survival (PFS) (P = 0.044) compared to patients with normal level of k light chain. Furthermore, patients with elevated level of both k and λ also exhibited significant association with shorter OS (P = 0.002) and PFS (P = 0.009). Both elevated k alone and concurrent elevated k and λ had independent adverse effects on PFS (P = 0.031 and P = 0.019, respectively). sTLC level was reduced gradually by treatment in this study and reached the lowest point after the fourth cycle of chemotherapy, which was consistent with the disease behavior during chemotherapy. Considering the small sample size of this study, these results should be confirmed in a larger prospective study.
Assuntos
Cadeias gama de Imunoglobulina/sangue , Cadeias kappa de Imunoglobulina/sangue , Linfoma Difuso de Grandes Células B/sangue , Linfoma Difuso de Grandes Células B/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Monoclonais Murinos/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Biomarcadores/sangue , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Feminino , Seguimentos , Humanos , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prednisona/administração & dosagem , Rituximab , Vincristina/administração & dosagemRESUMO
Gamma heavy chain disease (gHCD) is a rare lymphoproliferative disorder characterized by the production of a truncated immunoglobulin heavy chain. Although some cases of gHCD are concurrent with other lymphoid neoplasms, few have been reported. We herein present the case of a 73-year-old woman with gHCD and T-cell large granular lymphocytic leukemia. A multiparameter flow cytometry analysis revealed neoplastic cells that were positive for CD28, a marker of T-cell activation, the anti-apoptotic antigen of neoplastic plasma cells, CD38 and CD45. The results of this multiparameter flow cytometry analysis may contribute to furthering the understanding of the clinicopathological features of gHCD.
Assuntos
Anemia/imunologia , Fadiga/imunologia , Doença das Cadeias Pesadas/patologia , Cadeias gama de Imunoglobulina/metabolismo , Leucemia Linfocítica Granular Grande/patologia , Linfócitos/metabolismo , Idoso , Anemia/etiologia , Análise Citogenética , Fadiga/etiologia , Feminino , Citometria de Fluxo , Doença das Cadeias Pesadas/complicações , Doença das Cadeias Pesadas/imunologia , Humanos , Cadeias Pesadas de Imunoglobulinas , Cadeias gama de Imunoglobulina/sangue , Leucemia Linfocítica Granular Grande/complicações , Leucemia Linfocítica Granular Grande/imunologiaAssuntos
Crioglobulinemia/imunologia , Síndrome de Guillain-Barré/imunologia , Imunoglobulina G/imunologia , Imunoglobulina M/imunologia , Cadeias gama de Imunoglobulina/imunologia , Cadeias kappa de Imunoglobulina/imunologia , Vasculite Leucocitoclástica Cutânea/imunologia , Vitiligo/imunologia , Biópsia , Crioglobulinemia/complicações , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Glucocorticoides/uso terapêutico , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/tratamento farmacológico , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Cadeias gama de Imunoglobulina/sangue , Cadeias kappa de Imunoglobulina/sangue , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Vasculite Leucocitoclástica Cutânea/diagnóstico , Vasculite Leucocitoclástica Cutânea/tratamento farmacológico , Vitiligo/diagnóstico , Vitiligo/tratamento farmacológicoRESUMO
Gamma heavy-chain disease (gHCD) is defined as a lymphoplasmacytic neoplasm that produces an abnormally truncated immunoglobulin gamma heavy-chain protein that lacks associated light chains. There is scant information in the literature regarding the morphologic findings in this rare disorder, but cases have often been reported to resemble lymphoplasmacytic lymphoma (LPL). To clarify the spectrum of lymphoproliferative disorders that may be associated with gHCD, this study reports the clinical, morphologic, and phenotypic findings in 13 cases of gHCD involving lymph nodes (n=7), spleen (n=2), bone marrow (n=8), or other extranodal tissue biopsies (n=3). Clinically, patients showed a female predominance (85%) with frequent occurrence of autoimmune disease (69%). Histologically, 8 cases (61%) contained a morphologically similar neoplasm of small lymphocytes, plasmacytoid lymphocytes, and plasma cells that was difficult to classify with certainty, whereas the remaining 5 cases (39%) showed the typical features of one of several other well-defined entities in the 2008 WHO classification. This report demonstrates that gHCD is associated with a variety of underlying lymphoproliferative disorders but most often shows features that overlap with cases previously reported as "vaguely nodular, polymorphous" LPL. These findings also provide practical guidance for the routine evaluation of small B-cell neoplasms with plasmacytic differentiation that could represent a heavy-chain disease and give suggestions for an improved approach to the WHO classification of gHCD.
Assuntos
Doenças Autoimunes/diagnóstico , Doença das Cadeias Pesadas/diagnóstico , Cadeias gama de Imunoglobulina/sangue , Tecido Linfoide/patologia , Linfoma de Células B/diagnóstico , Macroglobulinemia de Waldenstrom/diagnóstico , Adulto , Idoso , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/genética , Doenças Autoimunes/metabolismo , Biomarcadores Tumorais/metabolismo , Células Clonais , Comorbidade , Análise Citogenética , Feminino , Doença das Cadeias Pesadas/sangue , Doença das Cadeias Pesadas/epidemiologia , Doença das Cadeias Pesadas/genética , Humanos , Cadeias gama de Imunoglobulina/genética , Imunofenotipagem , Hibridização in Situ Fluorescente , Linfócitos/metabolismo , Linfócitos/patologia , Tecido Linfoide/metabolismo , Linfoma de Células B/epidemiologia , Linfoma de Células B/genética , Linfoma de Células B/metabolismo , Masculino , Pessoa de Meia-Idade , Plasmócitos/metabolismo , Plasmócitos/patologia , Fatores Sexuais , Reino Unido/epidemiologia , Estados Unidos/epidemiologia , Macroglobulinemia de Waldenstrom/epidemiologia , Macroglobulinemia de Waldenstrom/genética , Macroglobulinemia de Waldenstrom/metabolismoAssuntos
beta-Globulinas/análise , Doença das Cadeias Pesadas/diagnóstico , Cadeias gama de Imunoglobulina/sangue , Idoso de 80 Anos ou mais , Eletroforese das Proteínas Sanguíneas , Neoplasias Ósseas/complicações , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/secundário , Diagnóstico Diferencial , Reações Falso-Positivas , Fraturas do Colo Femoral/etiologia , Doença das Cadeias Pesadas/sangue , Doença das Cadeias Pesadas/urina , Humanos , Imunoeletroforese , Imunoglobulina G/sangue , Imunoglobulina G/urina , Cadeias Leves de Imunoglobulina/sangue , Cadeias Leves de Imunoglobulina/urina , Masculino , Neoplasias da Próstata/diagnóstico , Neoplasias da Próstata/patologiaAssuntos
beta-Globulinas/análise , Doença das Cadeias Pesadas/diagnóstico , Cadeias gama de Imunoglobulina/sangue , Albuminúria/diagnóstico , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/secundário , Diagnóstico Diferencial , Reações Falso-Positivas , Doença das Cadeias Pesadas/sangue , Doença das Cadeias Pesadas/urina , Humanos , Imunoglobulina G/sangue , Imunoglobulina G/urina , Cadeias kappa de Imunoglobulina/sangue , Cadeias kappa de Imunoglobulina/urina , Masculino , Mieloma Múltiplo/diagnóstico , Neoplasias da Próstata/diagnóstico , Neoplasias da Próstata/patologiaRESUMO
Heavy chain diseases (HCDs) are rare B-cell lymphoproliferative neoplasias characterized by the production of a monoclonal component consisting of a truncated monoclonal Ig heavy chain without the associated light chain. Among them, patients with gamma-HCD are so rare that no more than 150 cases can be found in the literature. In this paper, we report one additional case: an 83-year-old man with a gamma-HCD, in whom a kappa light chain component was detected in the serum by using the serum free light-chain assessment and in addition monoclonal kappa cytoplasmic expression was detected in bone marrow plasma cells by flow cytometric analysis. In the work-up of the patient, the underlying anatomopathological lymphoproliferative disease corresponded to a lymphoplasmacytic lymphoma, as it is stated in the current World Health Organization classification (2008), with both lymphadenopathic and bone marrow infiltration. As in other cases, several autoimmune manifestations (antiphospholipidic syndrome and immune thrombocytopenia) were present during the course of the disease in this patient. This case report illustrates a new case of gamma-HCD, in which serum free light-chain analysis and flow cytometry represented a valuable tool for diagnosis, a finding that could be very important for the future management of these patients.
Assuntos
Doença das Cadeias Pesadas/sangue , Doença das Cadeias Pesadas/diagnóstico , Cadeias gama de Imunoglobulina/sangue , Idoso de 80 Anos ou mais , Citometria de Fluxo , Humanos , MasculinoRESUMO
We describe the case of a 34-year-old gentleman investigated for persistent neutropaenia following two episodes of pneumonia. Specialist investigations led to the diagnosis of multiple myeloma (MM) producing a truncated monoclonal gamma(3) heavy chain (HC) immunoglobulin molecule unattached to a light chain (LC) with atypical features for both MM and HC disease. Western blot showed gamma(3)HC was truncated with a large deletion (75 kDa). Flow cytometry of the bone marrow aspirate revealed an unusual staining pattern. This plasma cell dyscrasia was also unusual in that a subpopulation (30%) secreted large quantities of free LC (FLC) as well as truncated IgG HC. This is the first description, investigation and treatment of MM with a plasma cell population producing truncated gamma(3)HC and kappaFLC M-proteins and illustrates a number of unique immunological and clinical features.
Assuntos
Cadeias gama de Imunoglobulina/sangue , Cadeias kappa de Imunoglobulina/sangue , Mieloma Múltiplo/imunologia , Adulto , Humanos , Cadeias gama de Imunoglobulina/química , Cadeias kappa de Imunoglobulina/química , Masculino , Mieloma Múltiplo/sangue , Mieloma Múltiplo/terapia , Transplante de Células-Tronco de Sangue Periférico , Resultado do TratamentoRESUMO
AIM: To detect and evaluate the antibodies against Helicobacter pylori (H pylori) neutrophil-activating protein (HP-NAP) in patients with gastric cancer and other gastroduodenal diseases. METHODS: Recombinant HP-NAP was prepared from a prokaryotic expression system in Escherichia coli. Serum positivity and level of HP-NAP-specific antibodies in sera from 43 patients with gastric cancer, 28 with chronic gastritis, 28 with peptic ulcer, and 89 healthy controls were measured by rHP-NAP-based ELISA. rHP-NAP-stimulated production of interleukin-8 (IL-8) and growth-related oncogene (GRO(alpha)) cytokines in the culture supernatant of SGC7901 gastric epithelial cells was also detected. RESULTS: The serum positivity and mean absorbance value of HP-NAP-specific antibodies in the gastric cancer group (97.7% and 1.01 +/- 0.24) were significantly higher than those in the chronic gastritis group (85.7% and 0.89 +/- 0.14, P < 0.005) and healthy control group (27.7% and 0.65 +/- 0.18, P < 0.001). The sensitivity and specificity of ELISA for the detection of HP-NAP-specific antibodies were 95.5% and 91.5%, respectively. HP-NAP could slightly up-regulate IL-8 production in gastric epithelial cell lines but had no effect on GRO(alpha) production. CONCLUSION: Infection with virulent H pylori strains secreting HP-NAP is associated with severe gastroduodenal diseases, and HP-NAP may play a role in the development of gastric carcinoma. rHP-NAP-based ELISA can be used as a new method to detect H pylori infection. The direct effect of HP-NAP on gastric epithelial cells may be limited, but HP-NAP may contribute to inflammatory response or carcinogenesis by activating neutrophils.
Assuntos
Anticorpos Antibacterianos/sangue , Proteínas de Bactérias/metabolismo , Helicobacter pylori/imunologia , Neoplasias Gástricas/imunologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Linhagem Celular , Quimiocina CXCL1/biossíntese , Clonagem Molecular , DNA Bacteriano/genética , Ensaio de Imunoadsorção Enzimática , Células Epiteliais/metabolismo , Escherichia coli/metabolismo , Gastrite/imunologia , Helicobacter pylori/genética , Humanos , Cadeias gama de Imunoglobulina/sangue , Interleucina-8/biossíntese , Pessoa de Meia-Idade , Dados de Sequência Molecular , Úlcera Péptica/imunologia , Reação em Cadeia da Polimerase , Proteínas Recombinantes/biossíntese , Adulto JovemRESUMO
Erythropoietin (Epo) is the main erythropoietic hormone. Recombinant human Epo (rHuEpo) is thus used in clinical practice for the treatment of anemia. Accumulating data reveals that Epo exerts pleiotropic activities. We have previously shown an anti-neoplastic activity of Epo in murine multiple myeloma (MM) models, and in MM patients. Our findings that this anti-neoplastic effect operates via CD8+ T lymphocytes led us to hypothesize that Epo possesses a wider range of immunomodulatory functions. Here we demonstrate the effect of Epo on B lymphocyte responses, focusing on three experimental models: (i) tumor-bearing mice, (5T2 MM mouse); (ii) antigen-injected healthy mice; and (iii) antigen-injected transgenic mice (tg6), overexpressing human Epo. In the MM model, despite bone marrow dysfunction, Epo-treated mice retained higher levels of endogenous polyclonal immunoglobulins, compared to their untreated controls. In both Epo-treated wild type and tg6 mice, Epo effect was manifested in the higher levels of splenocyte proliferative response induced in vitro by lipopolysaccharide. Furthermore, these mice had increased in vivo production of anti-dinitrophenyl (DNP) antibodies following immunization with DNP-keyhole limpet hemocyanin. Epo-treated mice showed an enhanced immune response also to the clinically relevant hepatitis B surface antigen. These findings suggest a potential novel use of rHuEpo as an immunomodulator.
Assuntos
Adjuvantes Imunológicos/farmacologia , Formação de Anticorpos/efeitos dos fármacos , Eritropoetina/farmacologia , Adjuvantes Imunológicos/genética , Animais , Anticorpos Monoclonais/imunologia , Anticorpos Monoclonais/farmacologia , Formação de Anticorpos/imunologia , Linfócitos B/citologia , Linfócitos B/efeitos dos fármacos , Linfócitos B/imunologia , Complexo CD3/imunologia , Proliferação de Células/efeitos dos fármacos , Eritropoetina/genética , Eritropoetina/uso terapêutico , Feminino , Hemocianinas/imunologia , Antígenos de Superfície da Hepatite B/imunologia , Humanos , Imunoglobulina G/sangue , Cadeias gama de Imunoglobulina/sangue , Cadeias kappa de Imunoglobulina/sangue , Lipopolissacarídeos/farmacologia , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Endogâmicos C57BL , Camundongos Transgênicos , Mieloma Múltiplo/sangue , Mieloma Múltiplo/tratamento farmacológico , Proteínas Recombinantes/farmacologia , Proteínas Recombinantes/uso terapêutico , Análise de Sobrevida , Linfócitos T/citologia , Linfócitos T/efeitos dos fármacos , Linfócitos T/imunologia , VacinaçãoRESUMO
We conducted an electrophoretic analysis of monoclonal gamma-globulin found in the serum of a patient with splenic marginal-zone lymphoma. This monoclonal protein showed electrophoretic mobility to the gamma region and reacted with anti-immunoglobulin (IgG) antiserum but not with anti-kappa or anti-lambda light chain antisera. Sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) and Western blotting of the monoclonal protein-rich gamma-globulin fraction extracted from the sliced gel revealed the presence of two types of abnormal IgG molecule, low- and high-molecular-weight IgG, neither of which reacted with anti-kappa or anti-lambda light chain antisera. Additionally, an abnormal high-molecular-weight gamma heavy chain was identified by reducing SDS-PAGE. These findings suggest that this monoclonal protein is composed only of gamma heavy chains of normal and larger size. 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). However, the unique composition of monoclonal gamma-globulin makes our case distinct from typical gammaHCD, which is characterized by an abnormal truncated low-molecular-weight gamma heavy chain. Thus, the unusual monoclonal protein may have been produced by a somatic mutation of IgH gene associated with splenic marginal-zone lymphoma.
Assuntos
Genes de Cadeia Pesada de Imunoglobulina/genética , Cadeias gama de Imunoglobulina/sangue , Linfoma de Células B/imunologia , Neoplasias Esplênicas/imunologia , Feminino , Doença das Cadeias Pesadas , Humanos , Imunoglobulina G/sangue , Linfoma de Células B/genética , Pessoa de Meia-Idade , Mutação , Neoplasias Esplênicas/genéticaRESUMO
Various data support the pathogenetic significance of serum IgM autoantibodies against glycolipid GM1 in patients with multifocal motor neuropathy. Although some patients with this neuropathy have an extraneural lymphoma, IgM anti-GM1 glycolipid autoantibodies have not been investigated in these cases. We found IgM anti-GM1 autoantibody in the serum of a 52-year-old man who developed multifocal motor neuropathy that was associated with an extraneural diffuse large B-cell lymphoma. An autopsy showed severe widespread demyelination without lymphoma cell infiltration in the peripheral nerves. Immunofluorescent flow cytometry and thin-layer chromatographic immunostaining demonstrated that most of the anti-GM1 antibody in the serum was monoclonal IgM of lambda type, which was also demonstrable in secretory form on lymphoma cells. The antibody showed affinity for the Galbeta1-3GalNAc terminal disaccharide of glycolipids GM1 and GD1b, which both are widespread in peripheral nerve myelin. Enzyme-linked immunosorbent assay demonstrated that this antibody was much more abundant in lymphoma cell culture supernatant than in normal lymphocyte culture supernatant. Thus, our patient's B-cell lymphoma cells produced a monoclonal IgM lambda autoantibody against this terminal disaccharide residue. This antibody bound to glycolipids GM1 and GD1b in peripheral motor nerve myelin, presumably initiating formation of destructive immune complexes that caused multifocal motor neuropathy.
Assuntos
Autoanticorpos/sangue , Imunoglobulina M/sangue , Cadeias gama de Imunoglobulina/sangue , Linfoma de Células B/complicações , Oligossacarídeos/imunologia , Polirradiculoneuropatia/imunologia , Complexo Antígeno-Anticorpo , Cromatografia em Camada Fina , Gangliosídeos/imunologia , Humanos , Linfoma de Células B/imunologia , Linfoma de Células B/patologia , Masculino , Pessoa de Meia-Idade , Nervos Periféricos/patologia , Polirradiculoneuropatia/patologiaRESUMO
We report the cases of 23 patients with gamma-heavy chain disease seen at our institution (8 patients previously reported, 15 new patients). There were 15 women and 8 men; the median age at diagnosis was 68 years (range, 42-87 yr). Sixteen patients had an associated lymphoplasma cell proliferative disorder, 3 had a lymphoplasma cell proliferative disorder and an autoimmune disorder, another 3 had an autoimmune disorder only, and 1 had no underlying disease. The lymphoplasma cell proliferative disorder was disseminated in 10 patients and localized in 6. Patients with localized lymphoplasma cell proliferative disorder included 3 with plasmacytoma (1 tongue, 1 submandibular area, and 1 thyroid), 2 with lymphoplasma cell proliferative disorder involving the bone marrow only, and 1 with amyloid of the skin. At the time of diagnosis, lymphadenopathy was present in 8 patients, splenomegaly in 7, and hepatomegaly in 1. A monoclonal spike on serum protein electrophoresis was documented in 19 patients. gamma-Heavy chain was documented by immunofixation in the serum of all patients; 2 had an additional immunoglobulin M-lambda. gamma-Heavy chain was present in the urine in 19 of 22 patients. Sixteen patients were treated for lymphoplasma cell proliferative disorder or autoimmune disorder (14 with chemotherapy, 1 splenectomy, and 1 thyroidectomy followed by radiation therapy). For 5 patients, treatment was not felt to be necessary; 2 patients were thought to be too sick for treatment. Of the 16 patients treated, 6 had a complete clinical response (in 2, gamma-heavy chain disappeared; in 2, gamma-heavy chain persisted; and for 2, no serologic follow-up was available); in 10 patients, clinical disease persisted (in 3, gamma-heavy chain disappeared; in 6, it persisted; and for 1, no serologic follow-up was available). Of 7 patients not treated, 2 died within 5 months; 1 died after 15 months; 2 had no clinical disease at latest follow-up, although gamma-heavy chain persisted; and 2 had no change in clinical and serologic status. The median duration of follow-up was 33 months (range, 1-261 mo). Median survival was 7.4 years.
Assuntos
Doença das Cadeias Pesadas/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/complicações , Doenças Autoimunes/epidemiologia , Linfócitos B/imunologia , Feminino , Doença das Cadeias Pesadas/complicações , Doença das Cadeias Pesadas/mortalidade , Doença das Cadeias Pesadas/terapia , Humanos , Imunoeletroforese , Cadeias gama de Imunoglobulina/sangue , Cadeias gama de Imunoglobulina/imunologia , Cadeias gama de Imunoglobulina/urina , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/epidemiologia , Transtornos Linfoproliferativos/imunologia , Masculino , Pessoa de Meia-IdadeRESUMO
A 41-year-old male with IgG kappa multiple myeloma is described. He developed a free gamma heavy chain without an accompanying light chain following high-dose chemotherapy and autologous peripheral blood stem cell transplantation. The free gamma heavy chain was detected in serum and urine specimens 2 months after transplant, and eventually evolved into an IgG kappa monoclonal protein with electrophoretic properties similar to the original myeloma protein. Although the origin of the free gamma heavy chain remains uncertain, it was most likely related to the underlying plasma cell malignancy and, therefore, was an early sign of disease relapse.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Cadeias gama de Imunoglobulina/sangue , Mieloma Múltiplo/imunologia , Mieloma Múltiplo/terapia , Adulto , Eletroforese em Gel de Ágar , Humanos , Cadeias gama de Imunoglobulina/urina , Cadeias kappa de Imunoglobulina , Masculino , Transplante AutólogoRESUMO
This report describes a new case of gamma-1-heavy chain disease found in a woman with malignant lymphoproliferative disease. The patient's serum and urine containing gamma-1-heavy chains were analyzed using different electrophoretic approaches, especially two-dimensional electrophoresis and immunoblotting analysis. In a serum sample, five sets of gamma-1-heavy chain spots differing in molecular weight with acidic pI values and one set of more basic gamma-1-heavy chain spots were found. The major group of spots exhibited molecular weight in the range from 29 to 39 kDa. Examination of urine sample proved the presence of the more basic set of gamma-1-heavy chain spots and two acidic groups, including 29 to 39 kDa set.
Assuntos
Doença das Cadeias Pesadas/sangue , Doença das Cadeias Pesadas/urina , Idoso , Western Blotting , Eletroforese em Gel Bidimensional , Feminino , Humanos , Cadeias gama de Imunoglobulina/sangue , Cadeias gama de Imunoglobulina/urinaRESUMO
We reported a rare case of gamma-heavy chain disease. A 63-year-old man had been given a diagnosis of diabetes mellitus at the age of 30 and had received hemodialysis since the age of 55. The patient presented with swollen lymph nodes in the neck. Lymph node biopsy findings suggested immunoblastic lymphadenopathy. The patient was admitted to Kitasato University hospital. Serum protein electrophoresis showed an increase of beta-fraction peak, and immunoelectrophoresis revealed an increase of gamma-heavy chain protein. Further studies of the gamma-heavy chain protein showed that it contained three different components and that the molecular weight of the main component was 34,000 Da. The patient died on the 11th day of hospitalization. The diagnosis at autopsy was unclassified malignant lymphoma.