Crioglobulinemia: una entidad heterogénea / Cryoglobulinemia: a heterogeneous entity

La crioglobulinemia se define como la presencia de inmunoglobulinas en el suero que se precipitan reversiblemente a bajas temperaturas. Se la clasifica en tipos I, II y III, según las características de las inmunoglobulinas. La primera suele asociarse a enfermedades linfoprolife-rativas y las de tipos II y III, denominadas crioglobulinemias mixtas, a infección por el virus de la hepatitis C, seguida de las enfermedades autoinmunes. Las manifestaciones clínicas se relacionan con obstrucción intravascular en el caso de la crioglobulinemia de tipo I, mientras que las de tipos II y III se manifiestan con vasculitis por depósito de inmunocomplejos. El compromiso cutáneo es el hallazgo principal, seguido del articular, el neurológico y el renal. Se presentan 3 casos de crioglobulinemia que, por los datos de laboratorio y las enfermedades asociadas, difieren de la descripción clásica publicada en los textos.

Cryoglobulinemia is defined as the presence of immunoglobulins in serum that reversibly precipitate at low temperatures. It is classified into types I, II and III on the basis of immunoglobulin characteristics. Type I is associated with lymphoproliferative disorders, type II and III known as mixed cryoglobulinemia, are associated with hepatitis C virus infection and autoimmune diseases. Clinical manifestations are related with occlusion of small and medium blood vessels common in type I cryoglobulinemia while immune-mediated vasculitis is frequent in mixed cryoglobulinemia. Cutaneous damage is the main manifestation, followed by joint, peripheral nerves and renal involvement. We present three cases of cryoglobulinemia that differ from the literature due to their laboratory findings and associated diseases.

Early Diagnosis of One Multiple Myeloma Patient with Cold Agglutinin Syndrome as the Initial Presentation / 中国实验血液学杂志

OBJECTIVE@#To analyze one case of multiple myeloma (MM) initially presenting cold agglutinin syndrome (CAS), so as to improve clinical understanding and screening of this disease.@*METHODS@#The clinical data, laboratory examination, bone marrow result, diagnosis and treatment of the patient were analyzed and summarized to provide ideas and clinical experience for the early diagnosis and treatment of CAS secondary to MM.@*RESULTS@#The clinical manifestations of asthenia, hemolysis, jaundice and scattered livedo reticularis were caused by CAS secondary to MM, which was different from the general Raynaud's phenomenon. IgMκ type MM was definitely diagnosed according to the morphological features of bone marrow cells and immunofixation electrophoresis. After 3 courses of chemotherapy with BAD regimen and enhanced thermal support, anemia was corrected, M protein was decreased and the cold agglutinin phenomenon was significantly reduced. The evaluation of efficacy reached very good partial response.@*CONCLUSION@#There are very few MM patients with CAS as the initial presentation, so it is easy to misdiagnose. Early diagnosis and individual therapy are particularly important, which requires clinicians to observe and gain experience further.

Feasibility of Preheating at 41 ℃ to Correct Red Blood Cell Parameters in the Presence of High-titer Cold Agglutinins / 中国医学科学院学报

Objective To explore the feasibility of preheating in 41 ℃ water bath for 30 minutes to correct the red blood cell parameters in the specimens containing high-titer cold agglutinins(CAs). Methods Two specimens containing high-titer CAs were selected during work,and the parameters of complete blood count at room temperature or after preheating in 37 ℃ or 41 ℃ water bath were compared.The smears were stained,and the distribution of red blood cells was observed with a microscope.Further,74 specimens without CAs were collected for complete blood count,and then the test results at room temperature and after preheating at 41 ℃ were compared. Results At room temperature,the specimens containing high-titer CAs showed significantly reduced red blood cell count(RBC)and hematocrit(HCT),abnormally increased mean corpuscular hemoglobin(MCH)and mean cell hemoglobin concentration(MCHC),abnormal percents of hemoglobin(HGB)and RBC,and aggregation of a large number of red blood cells.After being preheated at 37 ℃ for a certain time,the specimens demonstrated obviously improved parameters while still aggregation of a small number of red blood cells.After being preheated at 41 ℃ for 30 minutes,the specimens showed significantly increased RBC,normal HCT,MCH,and MCHC,and evenly distributed red blood cells.The 74 specimens without CAs showed the comparability was ≥80% between room temperature and preheating at 41 ℃ for 30 minutes or 60 minutes. Conclusion We can preheat the specimens containing high-titer CAs in a water bath at 41 ℃ to obtain accurate red blood cell parameters.

Manifestaciones cutáneas extraglandulares en pacientes con síndrome de Sjögren primario / Extraglandular cutaneous manifestations in patients with primary Sjögren's syndrome

RESUMEN Objetivos: Describir la frecuencia de manifestaciones cutáneas extraglandulares en pacientes con síndrome de Sjögren primario. Determinar el perfil clínico y de laboratorio de los pacientes que presentan estas manifestaciones en comparación con aquellos que no las presentan. Materiales y métodos: Se analizaron los datos de los pacientes incluidos en la base GESSAR (Grupo de Estudio Síndrome de Sjögren de la Sociedad Argentina de Reumatología). Para la comparación entre grupos, los controles se seleccionaron en forma aleatoria con una relación casos: controles de 1:4. A su vez, se compararon los pacientes con púrpura con los controles. Resultados: Sesenta y siete (14,1%) de los 474 pacientes incluidos en la base de datos tuvieron manifestaciones cutáneas extraglandulares. De ellos, el 58% tuvo púrpura. La artritis, la neuropatía, el descenso de C3 y de C4, y la crioglobulinemia fueron estadísticamente más frecuentes en los casos en comparación con los controles; sin embargo, no se encontró asociación independiente con ninguna de estas variables. En lo que respecta a púrpura, la artritis, la neuropatía periférica, la anemia, el descenso de C3 y de C4, anti-La y crioglobulinemia fueron estadísticamente más frecuentes en comparación con los controles. Solo el descenso de C4 y la positividad de crioglobulinas se asociaron en forma independiente a la presencia de púrpura. Conclusión: El 14% de los pacientes presentaron manifestaciones cutáneas extraglandulares. La púrpura fue la manifestación más frecuente. Esta se asoció en forma independiente con el descenso de C4 y la presencia de crioglobulinas.

ABSTRACT Objectives: To describe the frequency of extra-glandular cutaneous manifestations in patients with primary Sjögren's syndrome. To determine the clinical and laboratory profile of patients who present with these manifestations compared to those who do not. Materials and methods: A study was made of patients included in GESSAR database (Sjögren Syndrome Society of Argentina Rheumatology Study Group) were analyzed. For the comparison between groups, the controls were randomly selected, with a case:control ratio of 1:4. Patients with purpura were compared with controls. Results: A total of 67 (14.1%) of the 474 patients included in the database had extra-glandular cutaneous manifestations. Of them, 58% had purpura. Arthritis, neuropathy, a decrease in C3 and C4 levels, and the presence of cryoglobulins, were statistically more frequent in cases compared to controls, although there was no independent association found with any of these variables. As regards purpura, arthritis, peripheral neuropathy, anaemia, decrease in C3 and C4, anti-La, and cryoglobulinemia were statistically more frequent compared to controls. Only the decrease in C4, and the presence of cryoglobulins were independently associated with the presence of purpura. Conclusion: Extra-glandular cutaneous manifestations were observed in 14% of the patients. Purpura was the most frequent cutaneous manifestation. This was independently associated with decreased C4 and the presence of cryoglobulins.

Essential Cryoglobulinemia Presenting as Polyneuropathy and Cerebral Infarction

Cryoglobulinemia is a rare medical condition defined by presence of cryoglobulins in serum and it may cause hyperviscosity syndrome or systemic vasculitis in variable organs including the skin, joints, liver, kidneys, lungs and nervous system. A vasculitic polyneuropathy or mononeuritis multiplex commonly occurs. However, central nervous system complications of cryoglobulinemia including stroke, encephalopathy and seizures are rarely reported. We represent a case of essential cryoglobulinemic vasculitis manifested as a sensorimotor polyneuropathy combined with an acute ischemic stroke attack.

Crioglobulina con propiedad crioaglutinina en un paciente con linfoma esplénico de zona marginal / Cryoglobulin with cold agglutinin properties in a patient with splenic marginal zone lymphoma / Crioglobulina com propriedade crioaglutinina em um paciente com linfoma esplênico de zona marginal

El objetivo del trabajo fue describir un caso de linfoma esplénico de zona marginal (LEZM) con un componente monoclonal que posee propiedades de crioglobulina y crioaglutinina, un hallazgo de muy baja frecuencia. Una paciente con LEZM padeció una anemia hemolítica autoinmune 5 años después del diagnóstico, con hematocrito de 0,15 L/L, hemoglobina 49 g/L, lactato deshidrogenasa 16,82 μkat/L, prueba de Coombs directa positiva con anti- IgG/C3d, bilirrubina total 90,6 μmol/L e indirecta de 58,1 μmol/L. No presentó evidencia clínica ni serológica de infección por VIH, hepatitis B ni C. El proteinograma sérico presentó un pico monoclonal de 14 g/L, con crioglobulinemia positiva a las 24 h, y un criocrito de 30%. La crioglobulina purificada fue de tipo I con un componente monoclonal IgM-lambda, coincidente al observado en suero. El suero, el eluato a 37 °C y la crioglobulina purificada de la paciente presentaron actividad de crioaglutinina con especificidad anti-I, fenómeno producido por la misma inmunoglobulina. El hallazgo de una crioglobulina con propiedades de crioaglutinina en pacientes con LEZM no ha sido descrito previamente en la bibliografía.

The aim of this paper is to describe a case of splenic marginal zone lymphoma (SMZL) with monoclonal component having properties of cryoglobulin and cold agglutinin, a finding of very low frequency. A patient with SMZL suffered autoimmune hemolytic anemia five years after diagnosis, with hematocrit 0.15 L/L, hemoglobin 49 g/L, lactate dehydrogenase 16.82 μkat/L, direct Coombs test with anti-IgG/C3d positive, total bilirubin 90.6 μmol/L and indirect 58.1 μmol/L. She presented no clinical or serological evidence of HIV, hepatitis B or C infection. Serum protein electrophoresis showed a monoclonal peak of 14 g/L, with positive cryoglobulinemia at 24 hours, and 30% cryocrit. Purified cryoglobulin was type I with a monoclonal IgM-lambda component coincident with that observed in serum. The patient serum, eluate at 37 °C and purified cryoglobulin showed cold agglutinin activity with anti-I specificity, phenomenon produced by the same immunoglobulin. The finding of a cryoglobulin with cold agglutinin properties in patients with SMZL has not been previously described in the literature.

O objetivo do trabalho foi descrever um caso de linfoma esplênico de zona marginal (LEZM) com um componente monoclonal com propriedades do crioglobulina e crioaglutinina, um achado de muito baixa frequência. Um doente com LEZM sofreu uma anemia hemolítica autoimune cinco anos após o diagnóstico, com hematócrito de 0,15 L/L, hemoglobina 49 g/L, lactato desidrogenase de 16,82 μkat/L, teste de Coombs direto positivo com anti-IgG/C3d, bilirrubina total 90,6 μmol/L e indireta 58,1 μmol/L. Não apresentou evidência clínica ou sorológica de infecção por HIV, hepatite B ou C. O proteinograma sérico mostrou um pico monoclonal de 14 g/L, com crioglobulinemia positiva 24 horas, e um criocrito de 30%. Crioglobulina purificada foi tipo I com o componente monoclonal IgM-lambda, coincidente com a observada no soro. O soro, o eluato a 37 ° C e a crioglobulina purificada do paciente mostraram atividade de crioaglutinina com especificidade anti-I, fenômeno produzido pela mesma imunoglobulina. O achado de uma crioglobulina com propriedades de crioaglutinina em pacientes com LEZM não foi previamente descrito na literatura.

Crioglobulinemia esencial como causa de hemorragia alveolar - reporte de caso y revisión de la literatura / Essential cryoglobulinemia as a cause of alveolar hemorrhage - case report and literature review / Crioglobulinemia essencial como causa de hemorragia alveolar

Presentamos el caso de una paciente quien ingresa por la presencia de lesiones petequiales, artralgias y una úlcera en miembro inferior. La presencia de azoados en ascenso progresivo se documenta durante su estancia. Se instaura manejo con corticoide IV sin respuesta y se obtienen mejoría con plasmaféresis. Posteriormente se observa la presencia de deterioro del patrón respiratorio, disnea sumado a la presencia de compromiso pulmonar en parches por medio de tomografía de tórax y con evidencia de hemorragia alveolar. Se descartan causas autoinmunes e infecciosas, así como neoplasias hematológicas. Se concluye que la presencia de crioglobulinemia es la única causa de dicha entidad.

We report the case of a patient who is admitted by the presence of petechial lesions, arthralgia and an ulcer in lower limb. The presence of progressively increasing creatinine is documented while staying. Unanswered management with corticosteroid IV was instituted and there was an improvement with plasmapheresis. Later, the presence of impaired breathing pattern was notice, dyspnea combined with the presence of lung involvement in patches through chest tomography and evidence of alveolar hemorrhage. Autoimmune and infectious causes and hematological malignancies were dismissed. The presence of cryoglobulinemia was identified as the unique cause of such entity.

Presentamos o caso duma paciente que ingressa pela presencia de lesiones petequiales, artraigias e uma úlcera no membro inferior. A presencia de azoados em ascensão progressivo documenta-se durante sua estancia. Instaura-se manejo com corticoide IV sim resposta e melhoram com plasmaféresis. Posteriormente, observou-se além a presencia de deterioro do padrão respiratório disnea a presencia de compromisso pulmonar em parches com tomografia de tórax e com evidencia de hemorragia alveolar. Descartem-se causas autoimunes e infecciosas, assim como neoplasias hematológicas. Conclui-se que a presencia de crioglobulinemia é a única causa de essa entidade.

Serum levels of immunoglobulin free light chains in patients with chronic hepatitis C presenting cryoglobulinemia

Hepatitis C virus (HCV) infects B-lymphocytes, provokes cellular dysfunction and causes lymphoproliferative diseases such as cryoglobulinemia and non-Hodgkin's B-cell lymphoma. In the present study, we investigated the serum levels of kappa and lambda free light chains (FLC) of immunoglobulins and the kappa/lambda FLC ratio in Brazilian patients with chronic HCV infection and cryoglobulinemia. We also analyzed the immunochemical composition of the cryoglobulins in these patients. Twenty-eight cryoglobulinemic HCV patients composed the target group, while 37 HCV patients without cryoglobulinemia were included as controls. The median levels of kappa and lambda FLC were higher in patients with cryoglobulinemia compared to controls (p = 0.001 and p = 0.003, respectively), but the kappa/lambda FLC ratio was similar in patients with and without cryoglobulinemia (p > 0.05). The median FLC ratio was higher in HCV patients presenting with advanced fibrosis of the liver compared to HCV patients without fibrosis (p = 0.004). Kappa and lambda FLC levels were strongly correlated with the IgA, IgG and IgM levels in the patients with cryoglobulinemia. In patients without cryoglobulinemia, the kappa FLC level was only correlated with the IgG level, whereas the lambda FLC were weakly correlated with the IgA, IgG and IgM levels. An immunochemical pattern of mixed cryoglobulins (MC), predominantly IgM, IgG, IgA and kappa light chain, was verified in these immune complexes. We concluded that HCV-infected patients presenting cryoglobulinemia have vigorous polyclonal B-lymphocyte activation due to chronic HCV infection and persistent immune stimulation.

Musculoskeletal manifestations and autoantibodies in children and adolescents with leprosy / Manifestações musculoesqueléticas e autoanticorpos em crianças e adolescentes com hanseníase

Objective: To evaluate musculoskeletal involvement and autoantibodies in pediatric leprosy patients. Methods: 50 leprosy patients and 47 healthy children and adolescents were assessed according to musculoskeletal manifestations (arthralgia, arthritis, and myalgia), musculoskeletal pain syndromes (juvenile fibromyalgia, benign joint hypermobility syndrome, myofascial syndrome, and tendinitis), and a panel of autoantibodies and cryoglobulins. Health assessment scores and treatment were performed in leprosy patients. Results: At least one musculoskeletal manifestation was observed in 14% of leprosy patients and in none of the controls. Five leprosy patients had asymmetric polyarthritis of small hands joints. Nerve function impairment was observed in 22% of leprosy patients, type 1 leprosy reaction in 18%, and silent neuropathy in 16%. None of the patients and controls presented musculoskeletal pain syndromes, and the frequencies of all antibodies and cyoglobulins were similar in both groups (p > 0.05). Further analysis of leprosy patients demonstrated that the frequencies of nerve function impairment, type 1 leprosy reaction, and silent neuropathy were significantly observed in patients with versus without musculoskeletal manifestations (p = 0.0036, p = 0.0001, and p = 0.309, respectively), as well as multibacillary subtypes in leprosy (86% vs. 42%, p = 0.045). The median of physicians' visual analog scale (VAS), patients' VAS, pain VAS, and Childhood Health Assessment Questionnaire (CHAQ) were significantly higher in leprosy patients with musculoskeletal manifestations (p = 0.0001, p = 0.002, p = 0002, and p = 0.001, respectively). Conclusions: This was the first study to identify musculoskeletal manifestations associated with nerve dysfunction in pediatric leprosy patients. Hansen's disease should be included in the differential diagnosis of asymmetric arthritis, especially in endemic regions. .

Objetivo: Avaliar o envolvimento musculoesquelético e os autoanticorpos em pacientes pediátricos com hanseníase. Métodos: Foram avaliados 50 pacientes com hanseníase e 47 crianças e adolescentes saudáveis de acordo com manifestações musculoesqueléticas (artralgia, artrite e mialgia), síndromes dolorosas musculoesqueléticas (fibromialgia juvenil, síndrome de hipermobilidade articular benigna, síndrome miofascial e tendinite) e painel de autoanticorpos e crioglobulinas. Escores de avaliação de saúde e tratamento foram realizados nos pacientes com hanseníase. Resultados: Pelo menos uma manifestação musculoesquelética foi observada em 14% dos pacientes com hanseníase e em nenhum controle. Dentre os pacientes com hanseníase, cinco tinham poliartrite assimétrica das pequenas articulações das mãos. Comprometimento da função do nervo foi observado em 22% dos pacientes com hanseníase, reação tipo I hansênica em 18% e neuropatia silenciosa em 16%. Nenhum dos pacientes e controles apresentou síndromes de dor musculoesquelética e as frequências dos anticorpos e crioglobulinas foram semelhantes nos dois grupos (p > 0,05). Comprometimentos da função nervosa, reação hansênica tipo I e neuropatia silenciosa foram observados em pacientes com vs sem manifestações musculoesqueléticas (p = 0,0036, p = 0,0001 e p = 0,309, respectivamente), bem como subtipos de hanseníase multibacilar (86% vs 42%, p = 0,045). A escala visual analógica (EVA) do médico, dos pacientes, e da dor e o Questionário de Avaliação de Saúde Infantil foram maiores em pacientes com manifestações musculoesqueléticas (p = 0,0001, p = 0,002, p = 0002 e p = 0,001, respectivamente). Conclusão: Este foi o primeiro ...

Crioglobulinemia esencial como causa de síndrome pulmón-riñón / Essential cryoglobulinemia as cause of lungkidney synd

Resumen La crioglobulinemia es una entidad poco frecuente y hace parte de los diagnósticos diferenciales delsíndrome pulmón-riñón; a continuación se presenta el caso de una paciente quien ingresa por lesiones petequiales, artralgias y úlcera en miembro inferior derecho; posteriormente cursando con deterioro dela función renal y luego falla respiratoria secundaria a hemorragia alveolar diagnósticada por imágenesy lavado broncoalveolar manejada con plasmaféresis presentando mejoría clínica, descartando etiologíaautoinmune, infecciosa y neoplásica como causa del cuadro actual, documentando únicamente la presencia de crioglobulinemia esencial. (Acta Med Colomb 2014; 39: 72-76).

Abstract Cryoglobulinemia is a rare entity and is part of the differential diagnosis of pulmonary-renal syndrome. This study reports the case of a patient admitted by petechial lesions, arthralgias and ulcer on the right lower limb, subsequently associated with impaired renal function and later respiratory failure secondary to alveolar hemorrhage, diagnosed by images and bronchoalveolar lavage and managed with plasmapheresis, presenting clinical improvement, discarding autoimmune, infectious and neoplastic etiology as the cause of the current clinical picture, documenting only the presence of essential cryoglobulinemia. (Acta Med Colomb 2014; 39: 72-76).

Manifestações músculoesqueléticas e auto-anticorpos em crianças e adolescentes com hanseníase / Musculoskeletal manifestations and autoantibodies in children and adolescents with leprosy

Introdução: A hanseníase é uma doença infecciosa crônica causada pelo Mycobacterium leprae. É considerada um dos maiores problemas de saúde pública nos países em desenvolvimento. Os principais sinais clínicos são manchas de pele com perda de sensibilidade e envolvimento de nervos periféricos. Manifestações musculoesqueléticas são descritas em adultos, mas este envolvimento é raramente descrito na população pediátrica. Objetivo: Avaliar envolvimento musculoesquelético e auto-anticorpos em pacientes pediátricos com hanseníase. Métodos: Foram avaliados 50 pacientes com hanseníase e 47 crianças e adolescentes saudáveis de acordo com manifestações musculoesqueléticas (artralgia, artrite e mialgia), síndromes dolorosas musculoesqueléticas (fibromialgia juvenil, síndrome de hipermobilidade articular benigna, síndrome miofascial e tendinite) e painel de auto-anticorpos e crioglobulinas. Escores de avaliação de saúde e tratamento foram realizados nos pacientes com hanseníase. Resultados: A frequência de manifestações musculoesqueléticas foi maior em pacientes com hanseníase comparada aos controles (14% vs. 0%, p=0,0012). Cinco pacientes com hanseníase tinham poliartrite assimétrica das pequenas articulações das mãos (10% vs. 0%, p=0,057). Comprometimentos da função do nervo, reação tipo I hansênica, e neuropatia silenciosa foram observados nos pacientes com hanseníase (p=0,0006; p=0,003; p=0,0059; respectivamente). Nenhum dos pacientes e controles apresentou síndromes de dor musculoesquelética e as frequências dos anticorpos e crioglobulinas foram semelhantes nos dois grupos (p > 0,05). Comprometimentos da função nervosa, reação hansênica tipo I e neuropatia silenciosa foram observados em pacientes com versus sem manifestações musculoesqueléticas (p=0,0036; p=0,0001; p=0,309; respectivamente), bem como subtipos de hanseníase multibacilar (86% vs. 42%, p=0,045). A escala visual analógica do médico (VAS), dos pacientes (VAS), de dor (VAS) e CHAQ foram maiores em...

Introduction: Leprosy is a chronic infectious disease caused by Mycobacterium leprae. It is considered one of major public health issue in developing countries. The important clinical signs of leprosy are hypopigmented or reddish localized skin lesions with loss of sensation and peripheral nerves involvement. Musculoskeletal manifestations were described in leprosy adult patients and these involvements were rarely described in pediatric leprosy population. Objective: To evaluate musculoskeletal involvement and autoantibodies in pediatric leprosy patients. Methods: 50 leprosy patients and 47 healthy children and adolescents were assessed according to musculoskeletal manifestations (arthralgia, arthritis and myalgia), musculoskeletal pain syndromes (juvenile fibromyalgia, benign joint hypermobility syndrome, myofascial syndrome and tendinitis) and a panel of autoantibodies and cryoglobulins. Health assessment scores and treatment were performed in leprosy patients. Results: The frequency of at least one musculoskeletal manifestation was significantly higher in leprosy patients compared to controls (14% vs. 0%, p=0.0012) and five leprosy patients had asymmetric polyarthritis of small hands joints (10% vs. 0%, p=0.057), Nerve function impairment, type I leprosy reaction and silent neuropathy were significantly observed in leprosy patients (p=0.0006; p=0.003; p=0.0059; respectively). None of the patients and controls presented musculoskeletal pain syndromes and the frequencies of all antibodies and cyoglobulins were similar in both groups (p>0.05). Further analysis of leprosy patients showed that the frequencies of nerve function impairment, type I leprosy reaction and silent neuropathy were significantly observed in patients with versus without musculoskeletal manifestations (p=0.0036; p=0.0001; p=0.309; respectively), as well as multibacillary subtypes in leprosy (86% vs. 42%, p=0.045). The median of physician visual analogue scale (VAS)...

Crioglobulinemia tipo I asociada a mieloma múltiple en un paciente con hapatits C / Type I cryoglobulinemia associated with multiple myeloma in a patient with hepatitis C

La presencia de crioglobulinas asociadas a manifestaciones clínicas sistémicas constituyen el síndrome crioglobulinémico. Se describen tres subtipos de esta entidad con características serológicas, clínicas e histológicas distintivas. En todos los casos, el órgano más afectadoes la piel. La sospecha clínica y el abordaje multidisciplinario son fundamentales para arribar al diagnóstico correcto e iniciar el tratamiento correspondiente.A continuación se presenta un caso de crioglobulinemia tipo I asociada a mieloma múltiple de reciente diagnóstico en un paciente con antecedente de hepatitis C que presentó un extenso compromiso cutáneo...

Differential Diagnosis of Secondary Glomerulonephritis / 대한내과학회지

Early diagnosis of glomerulonephritis is important in initiating appropriate treatment and controlling chronic glomerular injury that may eventually lead to end-stage renal disease. Although many patients with glomerular disease have only renal involvement, systemic presentations are not uncommon, as frequently resulted from an underlying systemic disease or a factor such as infections, drugs, or metabolic diseases. The presence of an underlying secondary reason should be investigated in each glomerular disease independent of its histopathologic type. Laboratory tests may include C3, C4, ANA, dsDNA, antineutrophil cytoplasmic antibody titers, a streptozyme test, HBV and HCV serologies, and in some cases, blood cultures, anti-glomerular basement membrane antibodies, and cryoglobulins. Although serologic tests may be helpful in establishing a diagnosis in some patients, a renal biopsy is usually performed even if serologic testing is positive. In addition, serologic test may assist in interpretation of the renal pathology.

Cryoglobulinemia is an independent factor negatively associated with sustained virological response in chronic hepatitis C patients / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Mixed cryoglobulinemia (MC) is one of the most common and severe symptoms in chronic hepatitis C patients. The aim of this study was to investigate whether mixed cryoglobulinemia is a factor associated with sustained virological response in chronic hepatitis C patients treated with combination therapy of pegylated interferon alpha-2a and ribavirin.</p><p><b>METHODS</b>This is a single-center study including 57 chronic hepatitis C patients who received combination treatments of pegylated interferon alfa-2a and ribavirin. Serum cryoglobulin was detected by cryoprecipitation prior to treatment. Serum hepatitis C virus (HCV) RNA levels were checked before treatment, during the fourth and 12th week of treatment, and during the 24th week after cessation of treatment. The genotype of HCV was determined at baseline. Logistic regression analysis was used to assess the factors associated with sustained virological response.</p><p><b>RESULTS</b>Twenty-five patients were with MC (43.9%). Twenty-four weeks after cessation of antiviral treatment, sustained virological response achievement in MC(+) patients was significantly lower than that in MC(-) patients (32.0% vs. 75.0%, P = 0.001). Univariate Logistic regression analysis and multivariate Logistic regression analysis found that only MC (odds ratio: 6.375; 95% CI: 1.998- 20.343, P = 0.002) was negatively associated with sustained virological response achievement.</p><p><b>CONCLUSION</b>MC is an independent factor negatively associated with sustained virological response in chronic hepatitis C patients treated with pegylated interferon alpha-2a and ribavirin.</p>

Crioglobulinemia de tipo I, reporte de caso / Cryoglubulinemia, case report

Las crioglobulinas son inmunoglobulinas que se precipitan de manera reversible a temperaturas bajas (inferiores a 37 ºC) y se disuelven nuevamente con el calor, y están asociadas a numerosas infecciones, enfermedades autoinmunitarias y trastornos neoplásicos. Producen síntomas por dos mecanismos principales, que son la vasculitis por depósito de inmunocomplejos en las paredes de los vasos y la isquemia de los tejidos como resultado directo de la oclusión del vaso. El tratamiento debe enfocarse hacia la enfermedad de base, aunque en algunos casos se requiere tratamiento con inmunosupresores.

Eritema elevatum diutinumasociado a crioglobulinemiamixta esencial / Erythema elevatum diutinum associated cryoglobulinemia mixed essential

El eritema elevatum diutinum (EED) es una forma infrecuente de vasculitis leucocitoclásica cutánea crónica de etiología desconocida. Desde el punto devista clínico se observan lesiones papulosas, placas y nódulos persistentes, de color eritematovioláceo, pardo o amarillento, localizadas en forma simé-trica en áreas de extensión de extremidades especialmente sobre las articulaciones. A nivel histopatológico se evidencia una vasculitis leucocitoclásica neutrofílica acompañada de fibrosis concéntrica. Se asocia a diversas entidades, entre ellas enfermedades infecciosas, hematológicas, colagenopatías y neoplasias malignas.Presentamos a una mujer de 22 años de edad con EED asociado a crioglobulinas en suero...

Erythema Elevatum Diutinum (EED) is an uncommon chronic cutaneous leukocytoclasticvasculitis of unknown etiology. Clinically, it consist of persistentbilateral, symmetrical and periarticular erithematous and purpuric, brownishor yellowish papules, plaques and nodules usually over the dorsal aspectof the joints. Histologic findings. neutrophilic leukocytoclastic vasculitis andconcentric fibrosis.It has been associated with infectious diseases, hematological disorders, collagendiseases and carcinomas.We report a 22 years old woman with EED and cryoglobulinemia...

Análise epidemiológica e laboratorial de indivíduos coinfectados pelo vírus linfotrópicos para células T humanas do tipo 1 (HTLV-1) e vírus da hepatite C (HCV) / Epidemiological and laboratory analysis of patients coinfected with human T-cell lymphotropic virus type 1 (HTLV-1) and hepatitis C virus (HCV)

A infecção pelo vírus linfotrópico para células T humanas tipo 1 (HTLV-1) pode levar a alterações funcionais da resposta imune do hospedeiro. O HTLV-1 está associado ao desenvolvimento de duas doenças principais: leucemia de células T do adulto e paraparesia espástica tropical/mielopatia associada ao HTLV-1. A infecção pelo vírus da hepatite C (HCV) pode levar ao desenvolvimento de hepatite crônica, cirrose hepática e carcinoma hepatocelular. [...] Devido ao papel da resposta imune celular no desenvolvimento e progressão de doenças associadas ao HCV, espera-se que a interação entre o HTLV-1 e HCV possa modificar o curso natural dessas infecções. Com o objetivo de avaliar aspectos epidemiológicos e laboratoriais foi realizado um estudo transversal envolvendo uma serie de casos. Foram incluídos no estudo 50 pacientes coinfectados com HTLV-1/HCV, 46 portadores de infecção pelo HCV e 50 pacientes HTLV-1 monoinfectados selecionados randomicamente. Pacientes portadores de infecção pelo vírus da hepatite B e/ou vírus da imunodeficiência adquirida foram excluídos do estudo. A análise estatística das variáveis avaliadas foi realizada utilizando métodos descritivos, paramétricos ou não-paramétricos. Não houve diferença significativa em relação às características sócio-demográficas entre os grupos. O relato de uso de álcool foi mais frequente no grupo HCV (p< 0,001). O passado de transfusão de sangue foi considerado como provável via de infecção em 80,6 por cento dos casos do grupo HCV e 39 por cento dos pacientes HTLV-1/HCV coinfectados. Em 26,8 por cento dos coinfectados a provável via de infecção foi a utilização de drogas injetáveis. Por outro lado, a transmissão sexual (55,6 por cento) predominou no grupo HTLV-1 (p<0,001)...

Human T-cell lymphotropic virus type 1 (HTLV-1) infection may lead to functional alterations of host immune response. HTLV-1 is associated with the development of two major diseases: adult T-cell leukemia/lymphoma and tropical spastic paraparesis/HTLV-1-associated myelopathy. [...] Due to the role of cellular immunity in the development and progression of HCV-associated diseases, one could expect that the interaction between HTLV-1 and HCV might modify thenatural course of these infections. with the aim to evaluate the epidemiological and laboratory aspects of these infections, a cross-sectional study with a series of cases was conducted. The study included 50 HTLV-1/HCV coinfected patients, 46 HCV and 50 HTLV-1 infected patients randomly selected. Patients with hepatitis B virus and/or human immunodeficiency virus infection were excluded. Statistical analysis was performed using descriptive, parametric or nonparametricmethods. The socio-demographic characteristics were similar among the three groups of patients. Alcohol drinking was more frequent in HCV group (p <0.001). Past history of blood transfusion was considered as probable route of infection in 80.6 percent of HCV group cases and in 39 percent of HTLV-1/HCV coinfected patients. In 26.8 percent of coinfected patients the probable infection route was history of drug addiction...

A Case of Hereditary Spherocytosis with Hemolytic Anemia due to Mycoplasma pneumonia / 소아감염

Mycoplasma pneumoniae is a common cause of community-acquired pneumonia in children, with a peak incidence at 5-14 years. Extrapulmonary manifestations occur in 20-25% of patients with M. pneumoniae infection. Most auto-antibodies that cause immune hemolytic anemia in humans are cold agglutinins. The formation of cold agglutinins is frequently observed during M. pneumoniae infections, and cold agglutinin disease usually occurs during M. pneumoniae infections. Nevertheless, severe hemolysis is exceptional. If a patient has any underlying disease related to hemolysis, it is possible to accelerate hemolysis. Hereditary spherocytosis is a common cause of hereditary hemolytic anemia resulting from red blood cell membrane defects. Hemolysis of red cells may result from corpuscular abnormalities or extracorpuscular abnormalities, such as immune or non-immune mechanisms. We report a case of hereditary spherocytosis associated with severe hemolytic anemia due to Mycoplasma pneumonia.