Vasculitis crioglobulinémica por hiperviscosidad y formación de criogel como debut de mieloma múltiple. Caso clínico / Cryoglobulinemic vasculitis due to hyperviscosity and cryogel formation as the onset of multiple myeloma. Report of one case

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.

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.

The application of modified capillary immunotyping technique for cryoglobulin identification / 中华检验医学杂志

Objective To establish and evaluate the application of modified capillary immunotyping for cryoglobulin qualification .Methods Referred to literature and benchwork experience , a modified capillary immunotyping technique was set up for cryoglobulin identification . Seventy-eight cryoglobulin positive specimens were collected by a standard method in Peking Union Medical College Hospital from November 2016 to July 2017.Thirty-nine samples were identified the type of the cryoglobulin simultaneously by modified capillary immunotyping ( CI ) and immunofixation electrophoresis ( IFE ) .Results Using the modified capillary immunotyping method , the types of cryoglobulin in seventy-eight specimens were identified.The number of cases decreased in the order of Ⅲ, Ⅱ and Ⅰ type of cryoglobulin .The clinical characteristics coincidence with previous reports .The modified CI method had a dramatic advantage in the speed, clarity, and accuracy of results compared with IFE .The ratio of reportable cases between these two methods was 1:0.87.Conclusion The modified capillary immunotyping was an accurate and easy method for cryoglobulin qualification , and feasible for clinical application .

Spurious platelet count due to cryoglobulins in a patient with smoldering myeloma.

Use of automated hematology analyzers for routine blood count reporting has increased the reproducibility and accuracy of test results. However, at times, these instruments may generate spurious test results. Such results can result in inappropriate investigations or treatment decisions in patients. Spuriously normal or high platelet counts carry the risk of under diagnosis of the true thrombocytopenia with adverse clinical implications. We present a patient with smoldering myeloma with spurious platelet count due to cryoglobulins.

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).

Autoantibody profile in individuals with chronic hepatitis C

Introduction Autoantibodies are often produced during infection with chronic hepatitis C virus (HCV), but it remains controversial whether they influence the biochemical profile and histological features of this disease. Therefore, this current study sought to describe these autoantibodies and evaluate their impact on the clinical and histological presentation of hepatitis C. Methods This cross-sectional analytical study assessed patients with HCV (RNA+) from October 2011 to July 2012. Results This study included 66 patients, with a mean age of 53.2±10.5 years. Of these patients, 60.6% were male, and 54.3% presented with genotype 1. Non-organ-specific autoantibodies (NOSA) were detected in 24% of the patients; of these, 7.6% were anti-mitochondrial antibodies (AMA+), 26.7% were anti-smooth muscle antibodies (SMA+) and 6.8% were liver kidney microsomal type 1 antibodies (LKM1+). With respect to the thyroid autoantibodies, 7.4% were anti-peroxidase (ATPO+) antibodies, and none were anti-thyroglobulin (ATG+) antibodies. Regarding celiac disease autoantibodies, 5.8% were endomysial antibodies (EMA+), and no transglutaminase (TTG+) antibodies were detected. Cryoglobulins were found in 2.1% of patients. When NOSA+ individuals were compared to patients without the presence of NOSAs, they exhibited higher median alkaline phosphatase (0.7 vs. 0.6 xULN; p=0.041), lower median platelet counts (141,500.0 vs. 180,500.0/mm 3 ; p=0.036), lower mean prothrombin activity (72.6±11.5% vs. 82.2±16.0%; p=0.012) and an increased prevalence of significant fibrosis (E≥2) (45.5% vs. 18.2%; p=0.012). There was also a tendency for a greater proportion of NOSA+ cases to have marked periportal activity (APP≥3) (44.5% vs. 15.6%; p=0.087). Conclusions In addition to the high prevalence of autoantibodies associated with HCV infection, it was observed that NOSA ...

Crioglobulinemias / Cryoglobulinemia

Las crioglobulinemias son una forma especial de vasculitis sistémica secundaria a la presencia de inmunoglobulinas circulantes que se precipitan a temperaturas menores de 37 °C y se solubilizan nuevamente con el aumento de la temperatura. Esta precipitación lleva a una vasculitis sistémica de medianos y pequeños vasos, como consecuencia de la presencia de complejos inmunes circulantes y expansión clonal de linfocitos B. Este grupo de entidades debe sospecharse en pacientes con fenómeno de Raynaud, signos clínicos de isquemia periférica en ausencia de enfermedad vascular, cianosis de orejas, nariz y dedos inducida por el frío, vasculitis cutánea, glomerulonefritis membranoproliferativa y gammopatías monoclonales, principalmente. La presencia de crioglobulinas circulantes, la disminución en los niveles de complemento, usualmente de los componentes tempranos como el C4 y el C1q, y la púrpura ortostática son característicos de la enfermedad. El tratamiento de las crioglobulinemias depende de la severidad de los síntomas, la enfermedad de base, la presencia del virus de la hepatitis C y el tipo de crioglobulinas. Las principales causas de muerte en estos individuos son: los compromisos renal, hepático y gastrointestinal, las infecciones, sexo masculino y criocrito mayor a cinco por ciento.

Cryoglobulinemia are a special form of systemic vasculitis secondary to the presence of circulating immunoglobulins that precipitate at temperatures below 37 °C and are solubilised again with increasing temperature. This precipitation leads to medium and small-vessel systemic vasculitis as a result of the presence of circulating immune complexes and clonal expansion of B lymphocytes. This group of entities should be suspected in patients with Raynaud phenomenon, clinical signs of ischemia in the absence of peripheral vascular disease, cold-induced cyanosis of ears, nose and fingers, cutaneous vasculitis, membranoproliferative glomerulonephritis, and monoclonal gammopathy. The presence of circulating cryoglobulins, decreased complement levels, usually early components, such as C1q and C4, and orthostatic purpura, are characteristic of the disease. Treatment of cryoglobulinemia depends of severity of the symptoms, underlying disease, the presence of hepatitis C virus and the type of cryoglobulins. The main causes of death in these individuals are: renal, hepatic, gastrointestinal involvements, infections, male and cryocrit more than 5%.

Smooth muscle antibodies and cryoglobulinemia are associated with advanced liver fibrosis in Brazilian hepatitis C virus carriers

Cryoglobulinemia and non-organ-specific-autoantibody are biomarkers of autoimmunity of the chronic infection caused by hepatitis C virus (HCV). In this work, we report the association between the presence of smooth muscle antibodies (SMA) and cryoglobulinemia and chronic liver disease in HCV carriers. Sixty-five untreated HCV patients, 38 women and 27 men were included in this study. Cryoglobulinemia was tested by cryoprecipitation, SMA by indirect fluorescent antibody test, and liver fibrosis and hepatocellular inflammation activity was investigated by histology of liver biopsy using the METAVIR score. The prevalence of SMA in the patients was 33.8 percent and cryoglobulinemia was demonstrated in 36.9 percent patients. Cryoglobulinemia and SMA seropositivity was associated with advanced fibrosis (p < 0.05). The presence of SMA and cryoglobulinemia was not associated with hepatocellular inflammation activity, age, carrier gender or HCV genotype. We concluded that liver biopsy should be recommended for HCV carriers that are seropositive for SMA or cryoglobulinemia.