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mixed cryoglobulinemia splenomegaly

Monika Prochorec-Sobieszek, Teresa Wagner
INTRODUCTION: Sjögren's syndrome [SS] is an autoimmune disease that mainly affects the exocrine glands. B-cell lymphoproliferation is a characteristic feature of this syndrome and the lesion may range from benign to malignant. MATERIAL AND METHODS: After a systematic search of Pubmed we reviewed literature regarding the histopathology, pathophysiology and clinics of lymphoproliferation in SS. RESULTS: Patients with Sjögren's syndrome [SS] have over 40-fold increased risk of the development B-cell non-Hodgkin's lymphoma...
2005: Otolaryngologia Polska
Morie A Gertz, Athanasios Anagnostopoulos, Kenneth Anderson, Andrew R Branagan, Morton Coleman, Stanley R Frankel, Sergio Giralt, Todd Levine, Nikhil Munshi, Alan Pestronk, Vincent Rajkumar, Steven P Treon
This presentation represents consensus recommendations for the treatment of patients with Waldenstrom's macroglobulinemia (WM), which were prepared in conjunction with the second International Workshop held in Athens, Greece during September 2002. The faculty adopted the following statements for the management of patients with Waldenstrom's macroglobulinemia: (1) Alkylating agents, nucleoside analogues, and rituximab are reasonable choices for first line therapy of WM. (2) Both cladribine and fludarabine are reasonable choices for the therapy of WM...
April 2003: Seminars in Oncology
S Guastafierro, F Sessa, A Tirelli
The presence of biclonal gammopathy [immunoglobulin (Ig)Mk + IgAlambda] and platelet antibodies in a patient with chronic hepatitis C virus (HCV) infection is reported. A type-II cryoglobulinemia (IgMk and polyclonal IgG) has also been detected. The IgAlambda monoclonal component still persists after removal of the cryoprecipitate. Two populations of atypical lymphoid cells with plasmocytoid features in bone marrow have been detected. They express IgM with k restriction and IgA with lambda restriction, respectively...
August 2000: Annals of Hematology
Y Ubara, S Hara, H Katori, T Tagami, A Kitamura, M Yokota, Y Matsushita, F Takemoto, A Yamada, K Nagahama, M Hara, K Chayama
Many patients with type II mixed cryoglobulinemia have been shown to be infected with hapatitis C virus (HCV). Therefore, interferon-alfa has become the first choice of treatment for patients with HCV-associated cryoglobulinemia. However, the disease often relapses after the discontinuation of interferon therapy. The long-term effect of interferon therapy is controversial. Therefore, a more effective therapy needs to be developed. A 62-year-old Japanese woman was admitted to our hospital for the examination of abnormal liver function tests, severe edema, and purpura in her lower extremities...
June 2000: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
B Grosbois, P Jego, R Leblay
INTRODUCTION: This review is aimed at defining the frequency, anatomical and clinical presentation, pathogenesis, predictive factors and treatment of malignant lymphoproliferative diseases occurring in the course of Sjögren's syndrome. CURRENT KNOWLEDGE AND KEY POINTS: The frequency of non-Hodgkin's lymphoma (NHL) is estimated to be about 7%. Other malignant lymphoproliferative diseases (Waldenstrom's macroglobulinemia, multiple myeloma, Hodgkin's disease) are rarely observed...
May 1998: La Revue de Médecine Interne
A G Tzioufas
Sjögren's syndrome (SS) is characterized by lymphocytic infiltration of the exocrine glands, along with a polyclonal B-cell activation which is illustrated by the presence of multiple autoantibodies against organ and non-organ specific autoantigens. Lymphoproliferative disorders present with a higher frequency in patients with Sjögren's syndrome. The spectrum of lymphoproliferation extends from an increased frequency of circulating monoclonal immunoglobulins, free light chains and mixed monoclonal cryoglobulins (type II cryoglobulinemia) to an increased frequency of non-Hodgkin's lymphoma...
January 1996: Clinical and Experimental Rheumatology
M Schlieper, M Kurrer, U Strebel, C Moll
Over a period of 25 years, a 67-year-old man again and again developed purpura over the lower legs. When hospitalized because of splenomegaly with thrombocytopenia the diagnosis of idiopathic thrombocytopenic purpura was made. Four years later, after an infection of the upper respiratory tract, purpura again developed, together with a symmetrical sensory-motor polyneuropathy with flaccid paraparesis. Biopsy of the N. suralis revealed a florid leukocytoclastic vasculitis. Morphologically there was severe nerve damage with Wallerian degeneration and subtotal nerve fibre loss...
January 29, 1993: Deutsche Medizinische Wochenschrift
A Tarantino, M Campise, G Banfi, R Confalonieri, A Bucci, A Montoli, G Colasanti, I Damilano, G D'Amico, L Minetti
We report the clinical outcome of 105 essential mixed cryoglobulinemia (EMC) patients with renal involvement collected throughout 25 years in three renal Units of Milan. The median follow-up was 72 months since renal biopsy and 131 months since the clinical onset of EMC. Patient survival was 49% at 10 years after renal biopsy. Forty-two patients died primarily from cardiovascular and liver disease or infection, whereas 15 patients developed chronic renal failure. Two patients had a complete remission of the disease while 15 had a remission only of renal signs...
February 1995: Kidney International
A Monteverde, G Bordin, P Zigrossi, A I Monteverde, M Campanini, G Cadario
A review of the less frequent organ damage seen in the so-called essential mixed cryoglobulinemia, and distinguishing between cryodependent and cryofavouring organ involvement, is presented in this paper. In the first group, along with the well-known renal, cutaneous and articular lesions, the peripheral neuropathy and respiratory involvement, to date too little considered, are worthy of more accurate study. Gastroenteric and cardiac participations are rare. Among the cryofavouring manifestations of the disease, we have pointed out those affecting the liver...
April 1986: La Ricerca in Clinica e in Laboratorio
H Torley, H Capell, M Timbury, C McCartney
A 47 year old woman presented with a six month history of vasculitic rash, splenomegaly, and cardiac murmurs. Investigations showed the presence of mixed cryoglobulinaemia and raised titres to Coxiella burnetii consistent with chronic Q fever infection. The patient was treated with tetracycline (1 g four times a day).
March 1989: Annals of the Rheumatic Diseases
Y Levo, P D Gorevic, H J Kassab, H Tobias, E C Franklin
A study of liver abnormalities in 36 patients with mixed cryoglobulinemia in the absence of underlying infectious, connective tissue, or lymphoproliferative disorders revealed clinical or biochemical evidence of liver dysfunction in 84%. Hepatomegaly was detected in 77%, splenomegaly in 54%, and abnormalities in bilirubin, alkaline phosphatase, or serum glutamic oxalacetic transaminase in 77%. Only four of the patients had overt liver disease. Of 15 biopsies from 12 patients, there was normal tissue structure in two, minimal nonspecific changes in one, portal fibrosis in three, chronic persistent hepatitis in one, chronic active hepatitis in two, chronic active hepatitis with cirrhosis in four, and postnecrotic cirrhosis in two...
September 1977: Annals of Internal Medicine
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