Evaluación de la zona gamma del proteinograma por electroforesis: correspondencia clínico-patológica / Evaluation of protein electrophoresis in the gamma zone: clinicopathological correspondence / Avaliação da área gamma do proteinograma por eletroforese: correspondência clínico-patológica

El proteinograma por electroforesis (PxE) sérico es solicitado para detectar modificaciones del perfil proteico. El objetivo del trabajo fue evaluar las alteraciones de la zona gammaglobulina y su correspondencia con distintos estados clínico-patológicos. Se incluyeron 7.259 pacientes (1-89 años) a los que en 2013 se les solicitó PxE. Según el trazado densitométrico, en la zona gammaglobulina se reconocieron diferentes grupos: hipogammaglobulinemia (<0,60 g/dL), hipergammaglobulinemia policlonal (≥1,80 g/dL), banda monoclonal (BM) y bandas oligoclonales. Prevaleció la hipergammaglobulinemia policlonal (4,2%), seguida por BM (1,4%) e hipogammaglobulinemia (0,8%). Hipergammaglobulinemia policlonal (>3 g/dL) se observó en: hepatitis autoinmune, cirrosis, síndrome de Sjögren, enfermedad mixta del tejido conectivo, HIV, hepatitis C y enfermedad de Castleman. El hallazgo de BM correspondió a 47% de pacientes con gammapatía monoclonal de significado incierto y 40% con mieloma múltiple; el 0,5% fueron casos nuevos. Con hipogammaglobulinemias, en adultos prevaleció la inmunosupresión terapéutica (55%), seguida por diabetes/síndrome metabólico/hipotiroidismo (23%); en niños, 22% por inmunosupresión y 78% con hipogammaglobulinemia no clasificada como inmunodeficiencia primaria. Se concluye que en 6,4% de los PxE se observó alteración de la zona gammaglobulina; prevaleció la hipergammaglobulinemia policlonal. En 1 de cada 200 PxE se pesquisó un paciente con BM. El hallazgo de hipergammaglobulinemia policlonal o BM se correspondió con distintos estados clínico-patológicos.

Serum protein electrophoresis (PEP) is requested to screen changes in the protein profile. The aim of this study was to evaluate alterations in the gamma globulin zone and correspondence with various clinical and pathological states. 7259 patients were included (1-89 years of age) who had been requested a PEP in 2013. According to the densitometric tracing, in the gamma globulin zone different groups were recognized: hypogammaglobulinemia (<0.60 g/dL), polyclonal hypergammaglobulinemia (≥1,80 g/dL), monoclonal band (MB) and oligoclonal band. The polyclonal hypergammaglobulinemia prevailed (4.2%), followed by MB (1.4%) and hypogammaglobulinemia (0.8%). Polyclonal hypergammaglobulinemia (>3 g/dL) was observed in autoimmune hepatitis, alcoholic cirrhosis, Sjögren's syndrome, mixed connective tissue disease, HIV, hepatitis C and Castleman's disease. The MB finding corresponded to a 47% of patients with monoclonal gammopathy of undetermined significance and 40% with multiple myeloma; 0.5% were new cases. In adults, hipogammaglobulinemias prevailed in therapeutic immunosuppression cases (55%), followed by patients with diabetes/ metabolic syndrome/ hypothyroidism (23%); in children, 22% with immunosuppression and 78% corresponded to hipogammaglobulinemias not classified as primary immunodeficiency. To conclude, an alteration in the gamma globulin zone was observed in 6.4% of PEP. In 1 out of 200 PEP MB was found. The finding of polyclonal hypergammaglobulinemia or MB corresponded to different clinicopathological states.

O proteinograma por eletroforese (PXE) sérico é solicitado para detectar modificações no perfil proteíco. O objetivo do trabalho foi avaliar as alterações da área gammaglobulina e sua correspondência com diversos estados clínico-patológicos. Incluíram-se 7259 pacientes (1-89 anos) aos quais, em 2013, foi solicitado um PxE. De acordo com o traçado densitométrico, na área gammaglobulina, diferente grupos foram reconhecidos: hipogammaglobulinemia (<0,60 g/dL), hipergammaglobulinemia policlonal (≥1,80 g/dL), banda monoclonal (BM) e bandas oligoclonais. Prevaleceu a hipergammaglobulinemia policlonal (4,2%), seguida por BM (1,4%) e hipogammaglobulinemia (0,8%). Hipergammaglobulinemia policlonal (>3 g/dL) foi observada em: Hepatite autoimune, cirrose, síndrome de Sjögren, doença mista do tecido conjuntivo, HIV, hepatite C e doença de Castleman. O achado de BM correspondeu a 47% de pacientes com gammapatia monoclonal de significado indeterminado e 40% com mieloma múltiplo; 0,5% eram casos novos. Com hipogammaglobulinemias em adultos prevaleceu a imunossupressão terapêutica (55%), seguida por diabete/síndrome metabólica/hipotireoidismo (23%); em crianças, 22% por imunossupressão e 78% com hipogammaglobulinemia não classificados como imunodeficiência primária. Conclui-se que em 6,4% dos PxE foi observada alteração da área gammaglobulina; prevaleceu a hipergammaglobulinemia policlonal. Em 1 de cada 200 PxE foi encontrado um paciente com BM. O achado de hipergammaglobulinemia policlonal ou BM se correspondeu com diferentes estados clínico-patológicos.

Solitary ulcerated plaque on the face - an unusual presentation of cutaneous plasmacytosis?

Abstract Cutaneous and systemic plasmacytosis is a rare disorder characterized by cutaneous polyclonal plasma cell infiltration frequently associated with polyclonal hypergammaglobulinemia and lymphadenopathy. We report a case of a 67-year-old woman with an inflammatory ulcerated plaque in the left masseter region. A skin biopsy showed dense perivascular infiltrate of mature plasma cells in the dermis without atypia and immunoglobulin light chain restriction. After physical examination and further investigation, we ruled out systemic disease. Our patient was successfully treated only with hydrocortisone cream application. Few cases of isolated benign primary cutaneous plasmacytosis have been described, particularly in children. After excluding the diagnosis of a reactive process to an infection, which is unlikely in this case, we suspected of a rare manifestation of primary cutaneous plasmacytosis in adults with distinct presentation and clinical course.

Doença sistêmica relacionada à IgG4 com linfopenia: relato de caso e breve revisão de literatura / IgG4-related systemic disease associated with lymphopenia: case report and brief literature review

A doença sistêmica relacionada à IgG4 é uma doença emergente, recentemente descrita, caracterizada clinicamente por aumento parcial ou total de um órgão, e, por isso, com amplo espectro de manifestações clínicas. Esta é uma doença sistêmica fibroinflamatória, patologicamente provocada pela infiltração de plasmoblastos IgG4 positivos que levam à inflamação eosinofílica do tecido e, consequentemente, fibrose estoriforme. Quando o diagnóstico é precoce, a melhora clínica e resposta sustentada com corticosteroides sistêmicos é impressionante. O diagnóstico é baseado em critérios patológicos, mas, recentemente, alguns trabalhos têm descrito que plasmoblastos no soro podem servir como um fator independente para auxiliar no diagnóstico da doença. Este artigo descreve uma apresentação atípica da doença relacionada à IgG4, em um paciente linfopênico com medição inconclusiva de plasmoblastos no soro.

IgG4-related systemic disease is a recently described, emerging condition, clinically characterized by partial or total enlargement of an organ, with a broad spectrum of clinical manifestations. It is a systemic fibroinflammatory condition caused by the infiltration of IgG4-positive plasmablasts that lead to eosinophilic inflammation of tissues and consequently storiform fibrosis. When diagnosis is early, clinical improvement and maintained response achieved with systemic corticosteroids is impressive. Diagnosis is based on pathological criteria, but recent papers have described that serum plasmablasts may serve as an independent factor to aid in diagnosis. This paper describes an atypical presentation of IgG4-related disease in a lymphopenic patient with inconclusive serum plasmablast measurement.

Associations of HIV testing and late diagnosis at a Japanese university hospital

OBJECTIVES: This study was conducted to clarify the rate of late diagnosis of HIV infection and to identify relationships between the reasons for HIV testing and a late diagnosis. METHODS: This retrospective cohort study was conducted among HIV-positive patients at the Jikei University Hospital between 2001 and 2014. Patient characteristics from medical records, including age, sex, sexuality, the reason for HIV testing and the number of CD4-positive lymphocytes at HIV diagnosis, were assessed. RESULTS: A total of 459 patients (men, n=437; 95.2%) were included in this study and the median age at HIV diagnosis was 36 years (range, 18-71 years). Late (CD4 cell count <350/mm3) and very late (CD4 cell count <200/mm3) diagnoses were observed in 61.4% (282/459) and 36.6% (168/459) of patients, respectively. The most common reason for HIV diagnosis was voluntary testing (38.6%, 177/459 patients), followed by AIDS-defining illness (18.3%, 84/459 patients). Multivariate analysis revealed a significant association of voluntary HIV testing with non-late and non-very-late diagnoses and there was a high proportion of AIDS-defining illness in the late and very late diagnosis groups compared with other groups. Men who have sex with men was a relative factor for non-late diagnosis, whereas nonspecific abnormal blood test results, such as hypergammaglobulinemia and thrombocytopenia, were risk factors for very late diagnosis. CONCLUSIONS: Voluntary HIV testing should be encouraged and physicians should screen all patients who have symptoms or signs and particularly hypergammaglobulinemia and thrombocytopenia, that may nonspecifically indicate HIV infection.

A case of cutaneous and systemi c plasmacytosis / 中华皮肤科杂志

A 46-year-old male patient developed scatterred reddish-brown plaques and nodules on the back 6 years prior to the presentation. Then, the lesions gradually spread to the axillary fossa and protothorax, and became indurated with slight itching in winter. Laboratory examination revealed hypergammaglobulinemia. Computed tomography(CT)scan showed multiple nodular or patchy shadows in both lungs, lymphadenectasis in axillary, mediastinal and inguinal regions, and spleen enlargement. Histopathological examination of skin lesions showed granulomatous infiltrates with plenty of lymphocytes, histiocytes and mature plasma cells in the middle and lower dermis with the presence of lymphoid follicle-like structures, but no cell atypia was observed. Immunohistochemical study showed positive staining for CD38, CD138, CD79a, κ and λ light chains. According to clinical manifestations and laboratory examination results, the patient was diagnosed with cutaneous and systemic plasmacytosis.

Clinical features analysis of monoclonal immunoglobulinemia of undetermined significance transforming into multiple myeloma in the elderly / 中华老年医学杂志

Objective To study the time of monoclonal immunoglobulinemia of undetermined significance (MIUS) transforming into multiple myeloma (MM),and the changes of immunoglobulin level and the clinical features.Methods A retrospective analysis was conducted in 22 patients with MM.According to the follow-up of immunoglobulin levels,patients were divided into formal and informal groups.The changes in immunoglobulin,serum calcium,hemoglobin and creatinine concentrations,and the time of MIUS transforming into MM were detected,and the impact of treatment on the prognosis was analyzed.Results The median age at diagnosis as MIUS was 69 years in formal group and 79 years in informal group.The median time for MIUS transforming into MM was 9.8 years in formal group and 1.3 years in informal group (P 0.006).The initial average concentration of immunoglobulin in patients with MIUS was 12.5 g/L (2.0-31.6 g/L) in informal group and 11.5 g/L (2.8-29.0 g/ L) in formal group (P＞0.05).There were no significant differences in levels changes of immunoglobulin,serum calcium,hemoglobin,creatinine index before and after the outcome.The transfer time for MIUS transforming into multiple myeloma was 9.8 years and 9.7 years,and the survival time was 13.6 years and 13.5 years in patients with versus without treatment (P＞ 0.05).Conclusions Regular formal follow up can accurately assess the transfer time for MIUS to MM,and the transfer time is longer in formal groups than in informal group.The changes in concentrations of immune globulin,serum calcium,hemoglobin,and creatinine cannot serve as indicators for outcome evaluation.Drug treatment neither delays the progress time of MIUS to MM nor reduces the complications of MM and nor prolongs the survival time.

Waldenström's macroglobulinemia - a review / Macroglobulinemia de Waldenström - uma revisão

Waldenström's macroglobulinemia (WM) is a lymphoproliferative disease of B lymphocytes, characterized by a lymphoplasmocytic lymphoma in the bone marrow and by IgM monoclonal hypergammaglobulinemia. It was first described in 1944 by Jan Gösta Waldenström, reporting two patients with oronasal bleeding, lymphadenopathy, anemia, thrombocytopenia, high erythrocyte sedimentation rate and serum viscosity, normal radiography and bone marrow infiltrated by lymphoid cells. The WM is a rare disease with a typically indolent clinical course, affecting mainly individuals aged between 63 and 68 years. Most patients have clinical signs and symptoms related to hyperviscosity resulting from IgM monoclonal gammopathy, and/or cytopenias resulting from bone marrow infiltration by lymphoma. The differential diagnosis with other lymphomas is essential for the assessment of prognosis and therapeutic approach. Treatment of patients with asymptomatic WM does not improve the quality of life of patients, or increase their survival, being recommended, therefore, their follow-up. For the treatment of symptomatic patients, alkylating agents, purine analogs and anti-CD20 monoclonal antibodies are used. However, the disease is incurable and the response to therapy is not always favorable. Recent studies have shown promising results with bortezomib, an inhibitor of proteasomes, and some patients respond to thalidomide. In patients with relapse or refractory to therapy, autologous transplantation may be indicated. The aim of this paper is to describe in detail the current knowledge on the pathophysiology of WM, main clinical manifestations, diagnosis, prognosis and treatment.

A macroglobulinemia de Waldenström (MW) é uma doença linfoproliferativa dos linfócitos B, caracterizada por um linfoma linfoplasmocítico na medula óssea e por hipergamaglobulinemia monoclonal de tipo IgM. Foi descrita pela primeira vez em 1944, por Jan Gösta Waldenström, que descreveu dois doentes com hemorragia oronasal, adenopatias, anemia, trombocitopenia, velocidade de sedimentação eritrocitária e viscosidade sérica elevadas, radiografia óssea normal e medula óssea infiltrada por células linfoides. A MW é uma doença rara com um percurso clínico normalmente indolente, atingindo principalmente os indivíduos com idades entre 63 e 68 anos. A maioria dos doentes apresenta sintomas e manifestações clínicas relacionadas com a hiperviscosidade, resultante da gamopatia monoclonal IgM e/ou com as citopenias, resultantes da infiltração medular pelo linfoma. O diagnóstico diferencial com outros linfomas é essencial para a avaliação do prognóstico e a abordagem terapêutica. O tratamento dos doentes com MW assintomática não melhora a qualidade de vida do doente nem aumenta a sua sobrevivência, recomendando-se o acompanhamento clínico. Para o tratamento dos doentes sintomáticos, são usados agentes alquilantes, análogos das purinas e anticorpos monoclonais anti-CD20. No entanto, a doença é incurável e a resposta à terapêutica nem sempre é favorável. Estudos relativamente recentes mostram resultados promissores com o bortezomibe, um inibidor dos proteossomas, e alguns doentes respondem à talidomida. Nos doentes com recidivas ou refratários à terapêutica, pode-se indicar o transplante autólogo. O objetivo deste trabalho é descrever, de forma detalhada, o conhecimento atual sobre a fisiopatologia da MW, as principais manifestações clínicas, o diagnóstico, o prognóstico e o tratamento.

Plasmocitosis cutánea y sistémica: presentación de un caso / Cutaneous and systemic plasmocytosis: a case report

La plasmocitosis cutánea y sistémica, es un raro trastorno que se caracteriza por infiltración de células plasmáticas policlonales en piel y otros órganos. A continuación, presentamos el caso de un paciente de sexo masculino, de 31 años de edad con antecedentes de infección por HIV, con compromiso óseo frontal y cutáneo. El estudio histopatológico confirmó el diagnóstico, mostrando un infiltrado dérmico perivascular a predominio de células plasmáticas kappa y lambda positivas. En sangre periférica se evidenció una hipergammaglobulinemia policlonal. El paciente presentó remisión espontánea de su enfermedad.

Cutaneous and systemic plasmocytosis is a rare disorder characterized by a polyclonal plasma cell infiltration in skin and other organs. We report a case of a 31 year old man with a medical history of HIV infection. He had frontal bone and skin involvement. Histopathological examination revealed a perivascular infiltrate composed predominantly of plasma cells expressing kappa and lamba light chains. Laboratory test demonstrated polyclonal hypergammaglobulinemia. The patient had spontaneus remission of his disease.

HIV Infection Itself may be a Cause of Hypokalemic Distal Renal Tubular Acidosis without Hypergammaglobulinemia.

Distal renal tubular acidosis (dRTA) is seen in the human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) population in the setting of hypergammaglobulinemia and antiretroviral agents, whereas isolated HIV infection is rarely reported to be associated with dRTA. We report a case of a young woman with a history of untreated HIV/AIDS who presented with profound generalized weakness and refractory hypokalemia along with non-anion gap metabolic acidosis and inappropriately high urine pH. Her serum gamma-globulin level was not significantly elevated and she was not on highly active antiretroviral therapy (HAART). No other cause of dRTA was evident. Subsequently, a diagnosis of dRTA secondary to isolated HIV/AIDS was made. Distal RTA can be acquired or inherited and is caused by defects in proton pumps or pH pressure gradients. In dRTA, the potassium level can be low, normal, or even high depending upon the pathophysiologic abnormality. Early recognition and prompt treatment is imperative to avoid the serious consequences of severe electrolyte and metabolic disturbances. Our case report is a reminder to clinicians to be mindful of this rare condition when evaluating unexplained dRTA and to include HIV/AIDS as part of the differential diagnosis of dRTA even in the absence of significant hypergammaglobulinemic (IgG level was slightly elevated) state or antiretroviral agents. We believe this is the second such case to be documented.

Hypergammaglobulinemia: a sentinel detecting autoimmune pancreatitis / 中华普通外科杂志

Objective To determine the clinical value of hypergammaglobulinemia as a sentinel for autoimmune pancreatitis and avoid unnecessary pancreas resection.Methods All 14 patients with autoimmune pancreatitis or related pancreatic diseases underwent routine examinations,including liver function,CA199 and imaging.Measurement of serum IgG or IgG4 was performed for patients with clinically suspected or pathologically proved autoimmune pancreatitis.Clinical features were retrospectively compared between the AIP and non-AIP patients using x2 statistics with Yates correction or Fisher exact test.Results Ten cases were finally confirmed as autoimmune pancreatitis.All patients with autoimmune pancreatitis had elevated levels of serum γ-globulins,while only one case without autoimmune pancreatitis had elevated levels of serum γ-globulins.It was proved by subsequent antibody tests that serum IgG/IgG4 and γ-globulins were simultaneously increased.Conclusions Hypergammaglobulinemia can be used as a preoperative sentinel indicator for differentiating autoimmune pancreatitis from pancreatic malignancies and avoiding unnecessary pancreas operation.

Hepatitis autoinmune asociada al uso de adalimumab en un paciente con artritis reumatoide / Autoimmune hepatitis associated to adalimumab in a patient with rheumatoid arthritis

La hepatitis autoinmune es una entidad progresiva de causa desconocida. en su patogénesis, se han propuesto disparadores ambientales, como virus y drogas, que al actuar sobre un paciente predispuesto genéticamente, desencadenan eventos mediados por células t, llevando a injuria hepatocelular. Los antagonistas del factor de necrosis tumoral son medicamentos utilizados para el manejo de la artritis reumatoide. se ha demostrado, sin embargo, que pueden generar daño hepático por diferentes mecanismos, uno de ellos la inducción de hepatitis autoinmune; la mayoría de los casos publicados se han asociado al uso de infliximab o etanercept. Se presenta un caso de hepatitis autoinmune inducido por adalimumab en una paciente con artritis reumatoide. (Acta Med Colomb 2012; 37: 147-151).

Autoimmune hepatitis is a progressive autoimmune disease of unknown cause. in regard to its pathogenesis, environmental triggers such as viruses and drugs have been proposed . these triggers when acting on a genetically predisposed patient, trigger t cell-mediated events, leading to hepatocellular injury. tumor necrosis factor antagonists are drugs used for the management of rheumatoid arthritis. it has been shown, however, that they can generate liver damage by different mechanisms, one of which is induction of autoimmune hepatitis. Most of the reported cases have been associated with use of infliximab or etanercept. We report a case of autoimmune hepatitis induced by adalimumab in a patient with rheumatoid arthritis. (Acta Med Colomb 2012; 37: 147-151).

Macroglobulinemia de Waldenström: remissão completa após tratamento com rituximabe / Successful outcome in Waldenström's macroglobulinemia treated with rituximab

A macroglobulinemia de Waldenstrõm (MW) é uma patologia rara dos linfócitos B caracterizada pela produção monoclonal de IgM, e que pode manifestar-se clinicamente com fadiga, astenia, perda de peso, sangramento de mucosas e do trato gastrintestinal, lifonodonomegalias, hepatoesplenomegalia e alterações neurológicas. A doença é mais comum em pacientes idosos, e seus sintomas são decorrentes da hiperviscosidade sangüínea. Na MW observa-se hipergamaglobulinemia com pico monoclonal na eletroforese de proteínas séricas, níveis elevados de IgM e demais imunoglobulinas normais ou diminuídas, imunofenotipagem com linfócitos B CD19+, CD20+ e CD24+, aspirado de medula óssea hipercelular, e biópsia de medula óssea hipercelular com infiltração difusa de linfócitos, linfócitos plasmocitóides e plasmócitos. Atualmente, anticorpos monoclonais estão sendo usados na terapêutica da MW com grande sucesso. O rituximabe, anticorpo monoclonal anti -CD20, tem mostrado excelentes resultados no tratamento da MW, inclusive naqueles indivíduos que não obtiveram resposta adequada ao tratamento convencional. Nós reportamos o caso de uma mulher de 78 anos de idade com história de fadiga, astenia, anorexia, sonolência, inquietação, urticária, dificuldade para deambular e perda excessiva de peso, aproximadamente 22 kg em um período de cinco meses, cujo tratamento foi realizado com rituximabe. O objetivo deste relato é apresentar uma paciente com diagnóstico de MW e revisar aspectos clínicos e terapêutico atual da doença.

Waldenstrõm's macroglobulinemia is a rare pathology of B lymphocytes characterized by the production of monoclonal IgM, causing clinical manifestations which may include fatigue, asthenia, weight loss, bleeding of the mucosa and intestinal tract, lymphadenomegaly, hepatosplenomegaly and neurological alterations. The disease is more frequent among elderly patients and its symptoms are a result of the hyperviscosity of blood. Waldenstrõm's macroglobulinemia presents hypergammaglobulinemia with a monoclonal peak of serum proteins seen by electrophoresis, high IgM levels and other normal or diminished immunoglobulin levels, immunophenotyping with CD19+, CD20+ and CD24+ B lymphocytes aspirated from hypercellular bone marrow and hypercellular bone marrow biopsy with diffuse infiltration of lymphocytes, plasmocytoid lymphocytes and plasmocytes. Currently, monoclonal antibodies are successfully being used in the treatment of Waldenstrõm's macroglobulinemia. Rituximab, an anti-CD20 monoclonal antibody, has shown excellent results in the treatment of Waldenstrõm's macroglobulinemia even for individuals who did not obtain satisfactory responses to conventional treatment. This work reports the case of a 78-year-old woman with a history of fatigue, asthenia, anorexia, somnolence, restlessness, urticaria, difficulties in walking, and excessive weight loss (approximately 22 Kg within a period of 5 months) who was successfully treated using rituximab. The objective of this report is to present the case of this patient and to review current clinical and therapeutic aspects of the disease.

Correlation analysis between hypergammaglobulinemia and renal involvement in patients with primary Sj(o)gren's syndrome / 中华风湿病学杂志

Objective To identify the correlation between hypergnmmaglobulinemia (hyper-IgG) and renal involvement in patients with primary Sjogren's syndrome (Pss). Methods The data of all patients admitted to hospital with the diagnosis of Pss were retrospectively analyzed. One way ANOVA and Speannan's correlation analysis were used to compare the clinical characteristics, renal injuries, immunology tests and renal pathological changes between patients with or without hyper-lgG. Results One hundred and thirty Pss cases were enrolled including 8 males and 122 females. Their age ranged from 16 to 68 years with an average of (44±12) years. Forty-one patients with Pss underwent renal biopsy. The preys lenee of Drta and tubular protein was significantly higher in patients with hyper-IgG than those without(P<0.05). Spearman's correlation analysis showed a negative correlation between serum IgG levels and seral potassium level(r=-0.269,P<0.01).Protein electrophoresis results revealed predominantly tubular protein in the hyper-lgG group, on the other hand glomerular protein was found in the normal-IgG group (P<0.05). The occurrence of decreased C4 com-plement concentration was significantly higher in normal-lgG group (P<0.05); Spearman's correlation analysis for biopsied materials showed that there was no significant difference in the Tubular Index (TI) and Glomeru-lar Index(GI) between these two groups. Conclusion Tubular lesions, especially Drta, may be predominant and correlate with hypergammaglobulinemia. There is a correlation between hypergammaglo-bulinemia and the level of renal lesions. Renal acidification capacity in patients with hypergammaglobulinemia should be evaluated.

Application of flow cytometry in the diagnosis of X-linked hyper-IgM syndrome / 中华检验医学杂志

Objective To develop a flow cytometrie assay to for diagnosis of X-Linked Hyper.1gM Syndrome(XHIM).Methods Heparinized peripheral blood obtained from patient,mother and a healthy control was diluted with RPMI- 1640(unstimulated control)or with RPMI-1640 containing 15μl PMA(1 rig/μl)and 6 trl ionomycin(50 ng/μl)(stimulated cell).Using directly labeled antibodies,we have examined CD40 ligand levels on CD3+ CD8- lymphocyte surface,and CD69 levels on CD;lymphocyte Surface to determine whether the cells were activated.Results CD69 levels on CD3+ lymphocyte surface from stimulated group and from unstimulated group were above 96% and below 3%,respectively.CD40L levels Oil CD3 CDs-T lymphocyte surface from stimulated group were 0.8% (patient),60.04%(mother) and 62.87%(healthy contr01).CD40L levels on CD3+ CD8-T lymphocyte surface from unstimulated group were 0.88% (patient),4.15%(mother)and 5.51%(healthy contr01).Conclusion This flow cytometric assayis accurate and convenient,which Can be used in neonatal screening.

Criterios diagnósticos y tratamiento de hepatitis autoinmune / Diagnostic criteria and treatment of autoimmune hepatitis

Se desconoce la incidencia real de la hepatitis auto inmune y su etiología exacta; es menos frecuente que las de origen viral; las niñas se afectan 3 veces más que los niños La enfermedad es muy activa; La mayoría de los casos se presentan con una sintomatología similar a la de una hepatitis viral aguda; el resto de los pacientes tiene un comienzo insidioso con astenia, anorexia o pérdida de peso, pudiendo existir también fiebre y dolor abdominal3. Para la aparición de una hepatitis autoinmune debe coexistir una predisposición genética multifactorial y un factor desencadenante, que podría ser una infección viral o la acción de un tóxico actuando como hapteno, producirá un complejo antigénico previamente inexistente que estimularía al sistema inmune. Esto supone una respuesta inmunológica tanto de índole humoral como celular, en cuyo origen podría haber un defecto, bien en los linfocitos T inductores de la supresión (CD4), o bien de los linfocitos T supresores propiamente dichos (CD8). El objetivo del tratamiento consiste en suprimir o eliminar la inflamación hepática con el menor número de efectos secundarios

The real incidence and etiology of autoimmune hepatitis is unknown. It is less frequent than viral hepatitis and three times more common in girls than boys. Most of the cases have the same symptoms as an acute viral hepatitis but there are some cases were the patient has symptoms such as asthenia, anorexia or weight loss, fever and abdominal pain. A patient who has autoimmune hepatitis can have a genetic predisposition that coexists with an environmental factor such as a viral infection that will stimulate the immune system. Immunologic response can be humoral or cellular and the origins of the defect can be at the level of helper T cells or suppressor T cells. The goal of the treatment consists to relieve symptoms.

Study of effect of total peony glucosides on hypergammaglobulinemia in undifferentiated connective tissue disease / 中华风湿病学杂志

Objective To observe the effect of total glucosides of peony on hypergammaglobulinemia of undifferentiated connective tissue disease(UCTD). Methods Fifty patients with UCTD were randomly divided into the treat-ment group (total glucosides of peony and hydroxychloroquine and thymosin) (n=30) and the control group (hydroxychloroquine and thymosin) (n=20). The changes between the two groups on hypergammaglobulinemia after treated for 3 months were compared. Results The two groups all had the IgG and IgA decreased, but the treatment group decreased more significantly. In addition, the treatment group had decreased RF. Conclusion Total glucosides of peony can improve the hypergammaglobulinemia of UCTD.

Cutaneous Plasmacytosis with Multiple Nodular Eruptions and Polyclonal Hypergammaglobulinemia

We report two patients with multiple peculiar skin eruptions and polyclonal hypergammaglobulinemia. Both patients visited our hospital for the evaluation of asymptomatic multiple nodular eruptions on almost their entire body except for the lower extremities. Histologic examinations disclosed prominent infiltration of plasma cells and lymphoid follicular hyperplasia in the dermis but these plasma cells showed neither a mitotic figure nor atypicalities. Laboratory examinations showed polyclonal hypergammaglobulinemias and increased erythrocyte sedimentation rates. In spite of various investigations, the cause of the hypergammaglobulinemia remained obscure.