ABSTRACT
Multiple Myeloma (MM) is characterized by a clonal expansion of plasma cells in the bone marrow. These cells typically produce a monoclonal immunoglobulin, and its symptoms arise either from plasma cell infiltration in several organs, or secondary to the presence of a monoclonal protein peak. Symptoms can be summarized by the acronym CRAB (hypercalcemia, renal failure, anemia and bone lesions). Sometimes, in the setting of a protein secreting monoclonal gammopathy, formation of cryoglobulins develops. Cryoglobulins are plasma proteins that precipitate at low temperatures, forming a cold - induced precipitate at small vessels, causing a wide range of clinical manifestations. We report a female consulting for ulcers lasting 2 months in the left foot associated with purpuric lesions in both lower limbs. Protein electrophoresis showed a monoclonal peak in the gamma region. Bone marrow aspirate showed 27% of plasma cells with kappa chain restriction by cytometry. The presence of cryoglobulins was confirmed. The patient was treated with dexamethasone and bortezomib, with a progressive healing of lower limb lesions and disappearance of cryoglobulins. She was discharged in good conditions.
Subject(s)
Humans , Female , Vasculitis/complications , Cryoglobulinemia/complications , Cryoglobulinemia/diagnosis , Multiple Myeloma/complications , Multiple Myeloma/diagnosis , Cryogels , Bortezomib/therapeutic useABSTRACT
La vasculitis crioglobulinémica es una vasculitis de vasos pequeños que se da en presencia de crioglobulinas séricas. Las crioglobulinemias mixtas son las más frecuentes y se asocian a infecciones crónicas, típicamente al virus de la hepatitis C, así como a enfermedades linfoproliferativas y autoinmunes, más a menudo al síndrome de Sjögren. Las manifestaciones clínicas incluyen púrpura de los miembros inferiores, neuropatía periférica, artralgias y glomerulonefritis. La presencia de vasculitis crioglobulinémica en el contexto de un síndrome de Sjögren es marcadora de peor pronóstico. Presentamos el caso de una paciente con vasculitis crioglobulinémica con compromiso cutáneo y renal, que condujo al diagnóstico de un síndrome de Sjögren primario. (AU)
Cryoglobulinemic vasculitis is a small-sized vasculitis that occurs in the context of serum cryoglobulins. Mixed cryoglobulinemias are the most frequent and are associated with chronic infections, typically hepatitis C and autoimmune diseases, most commonly Sjögren's syndrome. Clinical manifestations include purpura of lower limbs, peripheral neuropathy, arthralgias and glomerulonephritis. The presence of cryoglobulinemic vasculitis in the context of Sjögren's syndrome is a marker of poor prognosis. We present the case of a patient with cryoglobulinemic vasculitis associated to cutaneous and renal involvement that led us to the diagnosis of primary Sjögren's syndrome. (AU)
Subject(s)
Humans , Female , Aged , Glomerulonephritis, Membranoproliferative/diagnosis , Sjogren's Syndrome/diagnosis , Vasculitis, Leukocytoclastic, Cutaneous/diagnosis , Cryoglobulinemia/diagnosis , Glomerulonephritis, Membranoproliferative/complications , Sjogren's Syndrome/complications , Vasculitis, Leukocytoclastic, Cutaneous/complications , Vasculitis, Leukocytoclastic, Cutaneous/pathology , Cryoglobulinemia/complications , Lower ExtremityABSTRACT
Introduction: le virus de l'hépatite C (VHC) a plusieurs manifestations extra hépatiques parmi lesquelles la cryoglubulinémie. La cryoglobulinémie se définit par la présence anormale dans le sang d'une ou plusieurs protéines (cryoglobuline) pouvant précipiter au froid. Méthodes: nous avons mené une étude transversale et analytique dans le service du laboratoire de biologie et l'unité d'hépatologie de l'Hôpital Général de Douala (HGD) pendant une durée de 6 mois. Etaient inclus dans le travail tous les patients acceptant de participer et porteurs d'un anticorps anti VHC avec ou sans traitement. Les cryoglobulines étaient recherchés par la méthode de Biuret et la classification était réalisée par une immunoélectrophorèse de Brouet. Une analyse multivariée a été réalisée, des facteurs de confusion tels que l'âge, le sexe et la durée après dépistage du VHC ont été ajustés.Résultats: nous avons inclus 116 patients. L'âge moyen était de 58,47 ± 9,95 ans. Le sexe masculin représentait 50,86% des cas. L'arthralgie était présente dans 69,80% des cas. La cryoglubiline était présente chez 63,80% des cas. Apres ajustement, le sexe féminin (ORa =2,18; IC à 95% [0,97-4, 90]; p= 0,059), l'asthénie seule (ORa =2,45; IC à 95% [1,04-5,80]; p= 0,041), l'asthénie couplée à l'arthralgie (ORa =2,84; IC à 95% [1,13-7, 10]; p= 0,026) et la présence de l'ARN du VHC (ORa =2,84; IC à 95% [1,13-7, 10]; p= 0,028) étaient des facteurs indépendamment associés à la présence de cryoglobuline.Conclusion: la prévalence de la cryoglobubine est élevée chez les patients porteurs de l'Ac anti VHC à l'HGD. Elle est recherchée par les méthodes biologiques simples. La recherche de cryoglobuline chez les patients porteurs du VHC est essentielle dans un pays à ressource limité
Subject(s)
Cameroon , Cryoglobulinemia/diagnosis , Hepatitis C/diagnosisABSTRACT
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...
Subject(s)
Humans , Cryoglobulins , Cryoglobulinemia/diagnosis , Multiple Myeloma , Neoplasms, Plasma Cell , ParaproteinemiasABSTRACT
Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well described separately, but cryoglobulinemic glomerulonephropathy is very rarely reported. A 61-yr-old woman complained of generalized edema, cyanosis of the extremities in cold weather, visual disturbance, and pancytopenia. Bone marrow and renal biopsy showed CD5+ expressing B-cells and cryoglobulinemic glomerulonephropathy. With the diagnosis of WM, she received cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy and got complete remission. Here, we report a rare case of WM associated with unusual expression of CD5+ B-lymphocytes and cryoglobulinemic glomerulonephropathy, and emphasize the importance of the clinical features in differentiating CD5+ B-cell lymphoproliferative disorders.
Subject(s)
Female , Humans , Middle Aged , CD5 Antigens/metabolism , Antineoplastic Agents/therapeutic use , B-Lymphocytes/immunology , Bone Marrow/pathology , Cryoglobulinemia/diagnosis , Cyclophosphamide/therapeutic use , Diagnosis, Differential , Doxorubicin/therapeutic use , Drug Therapy, Combination , Glomerulonephritis/diagnosis , Kidney/pathology , Paraproteinemias/diagnosis , Prednisolone/therapeutic use , Vincristine/therapeutic use , Waldenstrom Macroglobulinemia/diagnosisABSTRACT
La brucelosis produce diversas manifestaciones clínicas muchas de las cuales son secundarias a alteraciones del sistema inmune desencadenadas por esta infección. Presentamos el caso de una paciente mujer de 71 años de edad que ingresó por un cuadro clínico de fiebre y púrpura, en quien se diagnosticó síndrome de crioglobulinemia mixta por infección brucellar. Este es el quinto caso que se reporta en el mundo. A pesar que la causa mas frecuente de púrpura por crioglobulinemia mixta es infección por VHC, se resalta que se debe sospechar de brucelosis especialmente cuando se presente con fiebre.(Rev Med Hered 2007;18:34-38).
Subject(s)
Humans , Female , Aged , Brucellosis/epidemiology , Brucellosis/pathology , Cryoglobulinemia/classification , Cryoglobulinemia/diagnosis , Cryoglobulinemia/pathology , Vasculitis/pathologyABSTRACT
Cryoglobulinemia is a systemic vasculitis that principally affects small and medium caliber vessels. It is thought that the inmunocomplex deposit made up of cryoglobulins that precipitate at temperatures under 38°C activate vessel wall complement, which in turn causes damage to the same. The cryoglobulins have been found in a wide spectrum of pathologies, though generally transitory and with no clinical importance. Monoclonal cryoglobulinemia is associated with hematologic diseases, while mixed cryoglobulinemias are found in infectious and systemic diseases. The association between the hepatitis C (VHC) virus and mixed cryoglobulinemias (MC) is more and more evident, and estimates show that 90 percent of cases include this infection. Clinical manifestations are varied and are due to the tissular ischemia caused by vessel occlusion, affecting the skin and visceral organs. Chronic VHC infection induces a persistent stimulation of the immune system, generating a constellation of autoimmune and neoplastic diseases.
Subject(s)
Humans , Cryoglobulinemia/classification , Cryoglobulinemia/diagnosis , Immune System Diseases/diagnosis , Vasculitis , Virus Diseases/diagnosis , Cryoglobulins/classification , Diagnosis, Differential , Patient Selection , Risk FactorsABSTRACT
Hepatitis C virus [HCV] is involved in an increasing number of non hepatic diseases. Mixed cryoglobulinemia [MC] is considered a lymphoproliferative disorder. It is characterized by arthralgia, generalized weakness, purpura and organ involvement such as membranoproliferative glomerulonephritis, peripheral neuropathy, vasculitis and chronic liver diseases. Cryoglobulinemia is quite frequent in chronic HCV infection and especially when appropriate techniques of cryoglobulin detection are applied. Its frequency is usually higher in older male cirrhotic patients and who have a longer duration of HCV infection. The term essential mixed cryoglobulinemia [EMC] has been used to describe the syndrome with no evidence of underlying disease. Chronic HCV infection is the main cause [90%] of EMC type II and III with or without clinical or biochemical evidence of liver damage. In addition, HCV was described as a lymphotropic virus and may play a direct role in the development of low grade non-Hodgkin's lymphoma which is usually associated with EMC. Aiming to estimate cryoglobulins in patients with CHC viral infection to identify the possible correlation between cryoglobulinemia and CHC viral infection, we conducted this study on 40 patients with CHC viral infection. Another group of 10 normal persons was considered a control group. Among our patients, there were 32.5% positive patients for cryoglobulinemia, those patients had increased incidence of cirrhosis and low C4. We suggested that EMC associated with HCV infection should no longer be referred to as essential but rather as hepatitis C associated with MC. In addition, every patient with chronic HCV infection may have MC, so that serum cryoglobulins and complement components must be evaluated repeatedly during the course of chronic HCV infection for avoiding their complications
Subject(s)
Humans , Male , Female , Cryoglobulinemia/diagnosis , Cryoglobulins , Liver Function Tests , Ultrasonography , Liver Cirrhosis , Risk Factors , Schistosomiasis , Blood TransfusionABSTRACT
Se presenta el caso clínico de una mujer de 50 años, con necrosis en sus dedos, crioglobulinemia y Síndrome de Raynaud. Se discuten los diferentes aspectos relacionados con la identificación, clasificación, y patologías asociadas a la crioglobulinemia, así como los mecanismos clínicos y patofisiológicos involucrados en esta enfermedad
Subject(s)
Humans , Female , Middle Aged , Cryoglobulinemia/diagnosis , Cryoglobulinemia/drug therapy , Raynaud Disease/diagnosisABSTRACT
Background: Anti liver kidney microsome antibodies (LKM-1) have been recently incorporated to the study and classification of chronic autoimmune hepatitis (HC-A1). The presence of anti LKM-1 antibodies and essential cryoglobulinemia is frequent in virus C associated chronic hepatitis (HC-VC). Aim: To study the frequency of anti LKM-1 antibodies and cryoglobulin levels in patients with HC-AI, HC-VC and cryptogenic cirrhosis. Patients and methods: Forty two patients were studied. Nineteen adult women with classic HC-AI with positive antinuclear or anti smooth muscle antibodies. Five patients of less than 20 years old with HC-AI and negative antinuclear and anti smooth muscle antibodies. Ten adult women with cryptogenic cirrhosis, 4 women and 4 men with HC-VC. Serum samples were obtained at 37C. Antinuclear, anti smooth muscle and anti LKM-1 antibodies were measured by indirect immunofluorescence using Hep-2 cells and rat tissue slices as substrates. Cryoglobulins were determined by the traditional method and cryocrit. Results: All studied patients were anti LKM-1 negative. All had significant circulating cryoglobulin levels. Conclusions: In this sample of patients with HC-AI or HC-VC, anti LKM-1 antibodies were not detected but all had cryoglobulinemia
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Hepatitis, Autoimmune/immunology , Cryoglobulinemia/diagnosis , Hepatitis C, Chronic/immunology , Biomarkers/analysisABSTRACT
A hepatite C e considerada atualmente a hepatite viral de maior importancia nos aspectos clinico e epidemiologico devido ao carater infeccioso lento e progressivo culminando com maior incidencia de cronicidade e consequentemente de maior suceptibilidade a cirrose e a um risco anual de aproximadamente 3 por cento para hepatocarcinoma...
Subject(s)
Humans , Hepatitis C/diagnosis , Cryoglobulinemia/diagnosis , Interferons/therapeutic use , Hepatitis C/therapy , Cryoglobulinemia/therapy , Diagnosis, DifferentialABSTRACT
Objetivo: Realizar una revisión de la literatura sobre el tema de las crioglobulinemias, su comportamiento biológico, su presentación clínica y sus nuevas perspectivas terapéuticas. En nuestro medio es una patología autoinmune olvidada a pesar de sus implicaiones clínicas y es necesario tenerla en cuenta en el diagnóstico diferencial o complementario de muchas entidades. Fuente de datos. Se realizó una revisión bibliografica de la base de datos Medline (1990 a 1998) y se incluyeron los artículos fundamentales descriptivos entre 1973 y 1983, realizados por el grupo de inmunología del Hospital Saint-Louis de París. Se lección y extracción de datos. Se estudiaron 858 referncias bibliográficas, de las cuales se seleccionaron 50, entre ellas revisiones, actualizaciones y reportes de casos clínicos con información concreta sobre el tema. Síntesis de los datos. El diagnóstico de la crioglobulinemia depende de la calidad de la toma y del procesamiento de las muestras. Las crioglobulinas son inmunoglobulinas séricas que precipitan a temperaturas frías y se disuelven con el calentamiento. Es necesario tener en cuenta ciertas precauciones durante la tima de la muestra de sangre que debe hacerse a 37 grados centigrados durante 8 días y una vez se obtiene el crioprecipitado, se purifica mediante centrifugación repetida y lavando a 4 grados centigrados. Si la toma de la muestra so se realiza a 37 grados centrigrados, se altern los resultados del cuadro hemático, la velocidad de sedimentación globular, la electroforesis de proteínas y algunas fracciones del complemento debido a la precipitación de proteínas. La identificación de las crioglobulinas se realiza por medio de la electroforesis y la inmunoelectroforesis en presencia de antisueros monoespecíficos dirigidos contra las cadenas pesadas µ,y,Ó y ligeras k y ï. La determinación de las cadenas ligeras permite definir la naturaleza monoclonal o policlonal de los constituyentes de la crioglobulinemia. Con frecuencia se asocia con otras patologías y su tratamiento depende de la presentación clínica y de la patología subyacente. Conclusión. El diagnóstico preciso de crioglobulinemia es indispensable ya que el algoritmo terapéutico disponible ofrece posibilidades de tratamiento dicotómicas, de acuerdo con los subtipos de clasificación y las enfermedades asociadas.
Subject(s)
Humans , Cryoglobulinemia/diagnosis , Cryoglobulinemia/drug therapy , Cryoglobulinemia/etiology , Cryoglobulinemia/physiopathology , CryoglobulinsABSTRACT
La hepatitis por virus A es una infección autolimitada que afecta preferiblemente a los niños en una forma anictérica y frecuentemente subclínica. En algunos casos se puede presentar la forma colestática o bifásica con un curso prolongado. La manifestación de desórdenes inmunológicos en hepatitis se describe con relativa frecuencia en la hepatitis por virus C y B. Sin embargo en hepatitis por virus A, estas manifestaciones son excepcionales. Presentamos el caso de una paciente de 33 años con hepatitis aguda a virus A, colestática y bifásica, que durante la fase de recaída desarrolla vasculitis, artritis y criglobulinemia
Subject(s)
Humans , Female , Adult , Allergy and Immunology/classification , Arthritis/diagnosis , Cryoglobulinemia/diagnosis , Hepatitis A/complications , Hepatitis A/diagnosis , Hepatovirus , Vasculitis/diagnosisSubject(s)
Humans , Male , Female , Adult , Arthritis, Rheumatoid/blood , Cryoglobulins/isolation & purification , Cryoglobulinemia/diagnosisABSTRACT
A total of 50,000 patients were surveyed for the presence of monoclonal immunoglobulins during the past two decades. There were 411 cases of monoclonal gammopathies including 243 cases of plasma cell neoplasms and 168 cases of secondary plasma-cell dyscrasia. Among the 227 cases of multiple myeloma and Waldenström's macroglobulinemia, there were 49.3% IgG class, 22.9% IgA class, 9.7% IgM class and 13.2% light chain type. In addition, there were 1.3% of nonexcretory myeloma including an IgM type. A relatively high frequency (4.8%) of IgD M-proteins was detected but heavy chain disease was not encountered in the present series. Purified M-components from patients with possible autoimmune manifestations were subjected to immunofluorescence studies. Autoimmune activity of M-proteins was found in a patient of Waldenström's macroglobulinemia with peripheral neuropathy, and another patient of cryofibrinogenemia with recurrent purpura and gangrene. In conclusion, a high frequency of IgD myeloma is found in Chinese patients of this area. M-components may have autoimmune activity resulting in unusual clinical manifestations.
Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Autoimmune Diseases/diagnosis , Cryoglobulinemia/diagnosis , Electrophoresis, Cellulose Acetate , Female , Fluorescent Antibody Technique , Humans , Immunoglobulins/analysis , Isoelectric Point , Male , Middle Aged , Paraproteinemias/diagnosis , Taiwan/epidemiology , Waldenstrom Macroglobulinemia/diagnosisABSTRACT
Reportamos un caso de Poliarteritis Nodosa Cutánea, Gota y Crioglobulinemia, algo no descrito previamente. Se estudia las características clínicas y de laboratorio, discutiendo los hallazgos con lo señalado en la literatura