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2.
Medicine (Baltimore) ; 98(28): e16382, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31305439

RESUMO

RATIONALE: Type I monoclonal cryoglobulinemia is usually associated with lymphoproliferative disorders, such as monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukemia (CLL) and lymphoplasmocytic lymphoma (LPL). Clinical symptoms related to Type I cryoglobulin (CG) isotype often include skin, neurological and renal manifestations. PATIENT CONCERNS: A 42-year-old woman who initially presented urticaria, palpable purpura in both her upper extremities and legs, eosinophilia and Raynaud phenomenon. Skin biopsy revealed eosinophil infiltration. Monoclonal immunoglobulin (Ig) G-κprotein was detected and CG was also positive. DIAGNOSES: The patient was finally diagnosed as MGUS related Type I CG. INTERVENTIONS: Cyclophosphamide-dexamethasone-thalidomide (CDT) therapy was initiated. OUTCOMES: The treatment relieved the skin symptoms efficiently. LESSONS: To our knowledge, this is a rare case of Type I cryoglobulinemic vasulitis with eosinophilia complicated by MGUS, and the effective treatment of cyclophosphamide combined with thalidomide and prednisone may provide a new therapeutic option for cryoglobulinemic vasulitis.


Assuntos
Crioglobulinemia/complicações , Eosinofilia/complicações , Gamopatia Monoclonal de Significância Indeterminada/complicações , Vasculite/complicações , Adulto , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Diagnóstico Diferencial , Eosinofilia/diagnóstico , Eosinofilia/tratamento farmacológico , Feminino , Humanos , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/tratamento farmacológico , Vasculite/diagnóstico , Vasculite/tratamento farmacológico
3.
Saudi J Kidney Dis Transpl ; 30(3): 663-669, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31249231

RESUMO

Cryoglobulinemia is a rare cause of kidney disease that occurs in patients with various diseases. Renal involvement often occurs after appearance of various clinical manifestations dominated by purpura and neuropathy. The aim of this study is to describe clinical, biological, and pathological characteristics of cryoglobulinemic glomerulonephritis (GN), as well as treatment and outcome. This is a retrospective study including all patients with positive cryoglobulin test and biopsy-proven GN secondary to cryoglobulinemia. Fourteen patients with cryoglobulinemic GN were collected. Their mean age was 46.92 ± 15.82 years with male predominance (64.28%). Weight loss, fever (71.42%), and purpuric rash (57.14%) were the main extrarenal manifestations. Eight patients presented with nephrotic syndrome (NS), associated with renal impairment in three patients. Four patients had rapidly progressive GN and two patients had acute kidney injury. Renal biopsy, performed in all patients, revealed membranoproliferative GN with glomerular thrombi in all patients. Crescents and necrotizing vasculitis were present in four patients. Hepatitis C virus (HCV) infection was the most common etiology. Antivirals and steroids or other immunosuppressive agents were used in most of the patients. During follow-up, complete response was observed in three patients and partial response was observed in four patients. Five patients had no response with renal injury requiring hemodialysis. NS with hematuria and renal insufficiency were the main clinical manifestations of cryoglobulinemic GN. In our study, HCV infection dominated the etiologies, although not well described earlier. A half of our patients had poor outcome even after antiviral and immunosuppressive therapy.


Assuntos
Lesão Renal Aguda/etiologia , Crioglobulinemia/etiologia , Glomerulonefrite Membranoproliferativa/etiologia , Hepatite C/complicações , Departamentos Hospitalares , Nefrologia , Síndrome Nefrótica/etiologia , Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/imunologia , Lesão Renal Aguda/terapia , Adulto , Antivirais/uso terapêutico , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Crioglobulinemia/imunologia , Feminino , Glomerulonefrite Membranoproliferativa/diagnóstico , Glomerulonefrite Membranoproliferativa/imunologia , Glomerulonefrite Membranoproliferativa/terapia , Hepatite C/diagnóstico , Hepatite C/tratamento farmacológico , Hepatite C/imunologia , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Marrocos , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/imunologia , Síndrome Nefrótica/terapia , Diálise Renal , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
4.
BMJ Case Rep ; 12(5)2019 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-31138600

RESUMO

Cryofibrinogenaemia is a rare haematological disorder characterised by cold temperature-induced precipitation of plasma proteins causing small-vessel occlusive vascular disorder with a hallmark of skin ulceration. It remains an underdiagnosed entity because of a lack of diagnostic criteria. Cryoglobulinaemia vasculitis is a small-vessel vasculitis involving the skin, the joints, the peripheral nerve system and the kidneys. Its association with cryofibrinogenaemia causes more severe phenotype with poor prognosis. We describe the case of a 59-year-old woman presenting with cold-induced extensive bilateral foot gangrene due to coexisting cryofibrinogenaemia and cryoglobulinaemic vasculitis that required bilateral amputation and rituximab perfusions as maintenance therapy.


Assuntos
Crioglobulinemia/diagnóstico , Pé/irrigação sanguínea , Gangrena/etiologia , Amputação/métodos , Antirreumáticos/uso terapêutico , Crioglobulinemia/complicações , Crioglobulinemia/tratamento farmacológico , Diagnóstico Diferencial , Feminino , Pé/patologia , Gangrena/patologia , Gangrena/cirurgia , Humanos , Pessoa de Meia-Idade , Doenças Raras , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Resultado do Tratamento , Vasculite/complicações
5.
Acta Dermatovenerol Croat ; 27(1): 28-32, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31032788

RESUMO

Cutaneous necrotizing eosinophilic vasculitis (CNEV) is a rare type of vasculitis. Eosinophilic vasculitis is a necrotizing vasculitis with eosinophilic vascular infiltration, in which eosinophils mediate vascular damage in the disease process. We present a case of an 18-year-old girl who developed palpable purpura and hemorrhagic bullae over the lower extremities associated with itching, 7 days after the commencement of penicillin therapy. Plasma cryofibrinogen was positive. Histopathology showed an infiltration of eosinophils within and around the vessel walls and a complete absence of nuclear dust and neutrophils. Oral prednisone at 1 mg/kg induced remission in 2 weeks; the prednisone dose was tapered and discontinued after 2.5 months. There was no evidence of recurrence after 37 months of follow-up. Our patient represents a rare case of drug/penicillin-induced CNEV associated with cryofibrinogenemia, without systemic organ involvement.


Assuntos
Antibacterianos/efeitos adversos , Crioglobulinemia/complicações , Eosinofilia/induzido quimicamente , Penicilinas/efeitos adversos , Vasculite/induzido quimicamente , Adolescente , Crioglobulinemia/diagnóstico , Crioglobulinemia/terapia , Eosinofilia/diagnóstico , Eosinofilia/terapia , Feminino , Humanos , Vasculite/diagnóstico , Vasculite/terapia
8.
BMJ Case Rep ; 12(1)2019 Jan 04.
Artigo em Inglês | MEDLINE | ID: mdl-30612104

RESUMO

We report an interesting case of hepatitis C virus-negative type II cryoglobulinaemic vasculitis (CV) in a patient with a background history of systemic lupus erythematosus. The type II CV became less responsive to traditional treatments over time and culminated in an intensive care unit admission with critical multiorgan failure. A detailed flow cytometric evaluation of the bone marrow proved to be helpful in treatment. It demonstrated that bortezomib was a viable alternative treatment option for the type II CV. The patient received bortezomib and has made a full and durable recovery.


Assuntos
Bortezomib/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Lúpus Eritematoso Sistêmico/complicações , Vasculite/tratamento farmacológico , Administração Intravenosa , Assistência ao Convalescente , Antineoplásicos/administração & dosagem , Antineoplásicos/uso terapêutico , Medula Óssea/imunologia , Bortezomib/administração & dosagem , Crioglobulinemia/diagnóstico , Feminino , Citometria de Fluxo/métodos , Hepatite C/complicações , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/complicações , Resultado do Tratamento , Vasculite/diagnóstico , Vasculite/imunologia
10.
Antivir Ther ; 23(Suppl 2): 1-9, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30451151

RESUMO

Mixed cryoglobulinaemia vasculitis (CryoVas) is a small-vessel systemic vasculitis caused by deposition of mixed cryoglobulins and is characterized by a wide range of clinical symptoms. HCV is the primary cause of CryoVas, which is associated with significant morbidity and mortality. The mortality rate among patients with HCV-associated CryoVas is 3× that of the general population, with a 63% 10-year survival rate. First-line treatment for CryoVas is anti-HCV therapy because viral clearance is associated with clinical improvement. The introduction of highly effective, interferon-free, direct-acting antiviral regimens provides additional treatment options for these patients. Here, we review recent studies investigating the effect of antiviral therapy on HCV-associated CryoVas and provide expert opinion for health-care professionals managing these patients.


Assuntos
Antivirais/uso terapêutico , Crioglobulinemia/terapia , Hepatite C Crônica/terapia , Rituximab/uso terapêutico , Vasculite/terapia , Corticosteroides/uso terapêutico , Crioglobulinemia/complicações , Crioglobulinemia/diagnóstico , Crioglobulinemia/mortalidade , Gerenciamento Clínico , Prova Pericial , Hepacivirus/efeitos dos fármacos , Hepacivirus/imunologia , Hepacivirus/patogenicidade , Hepatite C Crônica/complicações , Hepatite C Crônica/diagnóstico , Hepatite C Crônica/mortalidade , Humanos , Plasmaferese/métodos , Medicina de Precisão/métodos , Índice de Gravidade de Doença , Análise de Sobrevida , Vasculite/complicações , Vasculite/diagnóstico , Vasculite/mortalidade
11.
Medicine (Baltimore) ; 97(39): e12355, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30278513

RESUMO

RATIONALE: Type I cryoglobulinemia is a rare and life-threatening condition. It occurs mainly in B-cell lymphoproliferative disorder. In almost half of the patients, type I cryoglobulinemia is characterized by severe cutaneous involvement. PATIENT CONCERNS: A 71-year-old man comes to our observation because of the onset of persistence of paresthesia and painful acrocyanosis in the fingers and toes. IgG-k multiple myeloma treated with chemotherapy and radiation therapy on the left iliac wing characterized his clinical history. At the evaluation of response after the first-line therapy, the patient achieved a very good partial response. At the time of progression, the same myelomatous disease has taken a typical behavior of cryoglobulinemia. DIAGNOSES: Type I multiple myeloma-associated cryoglobulinemia was diagnosed. INTERVENTIONS: The patient underwent to an immediate composite therapeutic approach based on prostanoid infusion, plasmapheresis along with second-line chemotherapy. OUTCOMES: Despite the rapid biochemical response, the ischemia of the feet worsened. Moreover, a bacterial infection overlapped. The surgical amputation of both feet was necessary. Allowing MM cytoreduction continuation the patient's clinical condition became stabilized. LESSONS: Though rare, type I cryoglobulinemia can be associated with plasma cell dyscrasias. Any delay in diagnosis and the start of therapy can cause worsening of organ damage and endanger the patient's life. Therapeutic strategies in these cases should be directed to the underlying diseases.


Assuntos
Crioglobulinemia/complicações , Mieloma Múltiplo/complicações , Úlcera Cutânea/etiologia , Idoso , Amputação , Crioglobulinemia/diagnóstico , Crioglobulinemia/terapia , Pé/patologia , Doenças do Pé/etiologia , Humanos , Masculino , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/terapia
12.
Nat Rev Dis Primers ; 4(1): 11, 2018 08 02.
Artigo em Inglês | MEDLINE | ID: mdl-30072738

RESUMO

Cryoglobulinaemia refers to the serum presence of cryoglobulins, which are defined as immunoglobulins that precipitate at temperatures <37 °C. Type I cryoglobulinaemia consists of only one isotype or subclass of monoclonal immunoglobulin, whereas type II and type III are classified as mixed cryoglobulinaemia because they include immunoglobulin G (IgG) and IgM. Many lymphoproliferative, infectious and autoimmune disorders have been associated with mixed cryoglobulinaemia; however, hepatitis C virus (HCV) is the aetiologic agent in most patients. The underlying mechanism of the disorder is B cell lymphoproliferation and autoantibody production. Mixed cryoglobulinaemia can cause systemic vasculitis, with manifestations ranging from purpura, arthralgia and weakness to more serious lesions with skin ulcers, neurological and renal involvement. This Primer focuses on mixed cryoglobulinaemia, which has a variable course and a prognosis that is primarily influenced by vasculitis-associated multiorgan damage. In addition, the underlying associated disease in itself may cause considerable mortality and morbidity. Treatment of cryoglobulinaemic vasculitis should be modulated according to the underlying associated disease and the severity of organ involvement and relies on antiviral treatment (for HCV infection), immunosuppression and immunotherapy, particularly anti-CD20 B cell depletion therapies.


Assuntos
Crioglobulinemia/diagnóstico , Crioglobulinemia/fisiopatologia , Antivirais/uso terapêutico , Crioglobulinemia/epidemiologia , Hepacivirus/patogenicidade , Hepatite C/complicações , Humanos , Imunoglobulinas/uso terapêutico , Interleucina-4/efeitos adversos , Interleucina-4/uso terapêutico , Rituximab/uso terapêutico
14.
S D Med ; 71(2): 81-84, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29990417

RESUMO

Dysproteinemia is excessive production of immunoglobulins from clonal proliferation of plasma or B cells. Cryoglobulins are a special group of immunoglobulins that precipitate below 37 degrees C. Type 1 cryoglobulinemia consists mostly of IgM and IgG that infrequently leads to glomerulonephritis. In these situations, an underlying malignancy is even rare with only 21 cases described so far with only four having chronic lymphocytic leukemia (CLL). We present a case of a 68-year-old male admitted with hypertension and kidney dysfunction who upon work up was found to have CLL. Kidney biopsy was performed that showed capillary loops thickening with massive intraluminal cryoglobulin deposition in the glomeruli on periodic acid-Schiff (PAS) stain and "tram tracking" on silver stains. The patient was started on chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisone. After his fourth and final cycle of treatment, the patient's serum creatinine and Glomerular filtration rate improved to 1.2 mg/dL and 85 mL/min from 3 mg/dL and 29 mL/min respectively. One month later, his renal function remained mostly unchanged. The rare association of type 1 cryoglobulinemia with underlying CLL makes it a unique and intriguing case.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Crioglobulinemia/diagnóstico , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Idoso , Crioglobulinemia/complicações , Ciclofosfamida/administração & dosagem , Glomerulonefrite/etiologia , Humanos , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/diagnóstico , Masculino , Prednisona/administração & dosagem , Rituximab/administração & dosagem , Vincristina/administração & dosagem
15.
BMC Nephrol ; 19(1): 170, 2018 07 09.
Artigo em Inglês | MEDLINE | ID: mdl-29986657

RESUMO

BACKGROUND: Recurrence of glomerulonephritis is an important risk factor for renal graft dysfunction. Cryoglobulinemia is known as a relatively rare cause of renal failure, and doctors are usually hesitant to perform transplantation on a recipient with cryoglobulinemia because of the risk for graft loss. We present a case of renal transplantation on a patient with organ manifestations of type II cryoglobulinemia. CASE PRESENTATION: At the age of 44 years, the patient developed acute kidney injury and purpura on the lower extremities with type II cryoglobulinemia after interferon therapy for hepatitis C virus. Cryoglobulinemic glomerulonephritis was suspected; however, despite immunosuppressive therapy combined with plasmapheresis, she eventually needed hemodialysis treatment. She was referred to us at the age of 49 years for renal transplantation. Cryocrit was 14% and the organ manifestations persisted, including the lower extremity purpura and neurologic symptoms. After monitoring and confirming sufficient suppression of cryoglobulin concentration by immunosuppressive treatment with prednisolone, cyclophosphamide, and rituximab combined with plasmapheresis, the operation was performed. After transplantation, the cryoglobulin concentration was continuously monitored, and plasmapheresis and rituximab infusion were performed as appropriate. Her graft function has remained stable for 2 years and 6 months. CONCLUSION: Our case suggested that a patient with cryoglobulinemia and persistent organ manifestations can receive a renal graft if the cryoglobulin concentration is sufficiently controlled by pretransplant treatment.


Assuntos
Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/cirurgia , Crioglobulinemia/diagnóstico , Crioglobulinemia/cirurgia , Transplante de Rim/tendências , Doadores Vivos , Lesão Renal Aguda/tratamento farmacológico , Idoso , Crioglobulinemia/tratamento farmacológico , Feminino , Humanos , Imunossupressores/uso terapêutico , Transplante de Rim/métodos , Pessoa de Meia-Idade , Resultado do Tratamento
17.
Am J Kidney Dis ; 72(4): 606-611, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29784613

RESUMO

We present a unique case of cryoglobulinemic glomerulonephritis associated with nodal and renal infiltration by T-cell lymphoma of T-follicular helper phenotype. The patient presented with transient neurologic symptoms, severe nephritic syndrome with nephrotic-range proteinuria, and acute kidney injury. He had elevated double-stranded DNA levels, low complement levels, detectable cryoglobulin, and detectable immunoglobulin M (IgM) paraprotein. The kidney biopsy showed cryoglobulinemic glomerulonephritis with a membranoproliferative pattern and diffuse interstitial infiltrates on light microscopy; IgM, C3 but weak IgG, C1q, and negative C4d staining on immunofluorescence; and deposits with organized substructures on electron microscopy. Positron emission tomography showed diffuse uptake in bilaterally enlarged kidneys and a localized group of lymph nodes. Subsequent lymph node biopsy revealed Epstein-Barr virus-negative nodal T-cell lymphoma, which was also proven in renal tissue. The association between T-cell lymphoma, autoantibodies, and cryoglobulinemia may represent a paraneoplastic phenomenon. His renal prognosis has been excellent, but overall prognosis and survival is dictated by the clinical course of T-cell lymphoma.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Crioglobulinemia/complicações , Glomerulonefrite Membranoproliferativa/patologia , Neoplasias Renais/patologia , Linfoma de Células T/patologia , Linfócitos T Auxiliares-Indutores/patologia , Adulto , Biópsia por Agulha , Medula Óssea/patologia , Crioglobulinemia/diagnóstico , Ciclofosfamida/uso terapêutico , Progressão da Doença , Doxorrubicina/uso terapêutico , Seguimentos , Glomerulonefrite Membranoproliferativa/complicações , Glomerulonefrite Membranoproliferativa/diagnóstico por imagem , Humanos , Imuno-Histoquímica , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/tratamento farmacológico , Linfoma de Células T/complicações , Linfoma de Células T/diagnóstico por imagem , Linfoma de Células T/tratamento farmacológico , Masculino , Fenótipo , Tomografia por Emissão de Pósitrons/métodos , Prednisona/uso terapêutico , Medição de Risco , Fatores de Tempo , Resultado do Tratamento , Vincristina/uso terapêutico
19.
Pediatr Rheumatol Online J ; 16(1): 31, 2018 Apr 24.
Artigo em Inglês | MEDLINE | ID: mdl-29690915

RESUMO

BACKGROUND: This study aimed to evaluate the demographic, clinical features, laboratory data, pathology and other survey in pediatric patients with cryofibrinogenemia. METHODS: A 12-year retrospective chart review identified eight pediatric patients at Mackay Memorial Hospital, Taipei, Taiwan. RESULTS: The female-to-male ratio was 3:1. The mean age at symptom onset and of diagnosis was 10.3 ± 4.6 years and 12.3 ± 4 years, respectively. One child (12.5%) had primary cryofibrinogenemia. The common symptoms were purpura, arthralgia, and muscle weakness (100%). On laboratory examination, cryofibrinogen was positive in all patients. All patients had increased anti-thrombin III while 87.5% and 62.5% had abnormal protein S and protein C, respectively. All eight also complained of neurologic symptoms. One had vertebral artery narrowing, two showed increased T2-weighted signal intensity on the thalamus or white matter, and one had acute hemorrhagic encephalomyelitis on brain magnetic resonance imaging. CONCLUSIONS: This study reports on the presentations of cryofibrinogenemia, which is rare in children. Most cases are associated with autoimmune disease and have severe and complex presentations. Central nervous system involvement is common.


Assuntos
Crioglobulinemia/diagnóstico , Adolescente , Criança , Pré-Escolar , Crioglobulinemia/complicações , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Estudos Retrospectivos , Taiwan
20.
Am J Emerg Med ; 36(8): 1523.e1-1523.e3, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29691104

RESUMO

The shoulder is one of the most commonly dislocated joints in the human body. Complications usually represent local damage. However, it is unclear whether joint dislocations can have systemic sequellae as wSell. Here we present the case of an 86-year-old female who developed necrotizing cryoglobulinaemic vasculitis in immediate response to a shoulder dislocation. We hypothesize there might be a link between trauma and systemic disease.


Assuntos
Crioglobulinemia/diagnóstico , Crioglobulinemia/etiologia , Luxação do Ombro/complicações , Vasculite/etiologia , Idoso de 80 Anos ou mais , Crioglobulinemia/terapia , Feminino , Humanos , Metilprednisolona/uso terapêutico , Troca Plasmática , Luxação do Ombro/fisiopatologia , Vasculite/terapia
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