1/16. Hypergammaglobulinemic purpura of Waldenstrom: report of 3 cases with a short review.Benign hypergammaglobulinemic purpura of Waldenstrom (HGPW) is an uncommon cause of non-thrombocytopaenic purpura that may create diagnostic difficulties. The presence of constitutional symptoms associated with prominent immunological abnormalities may raise alarm, leading to extensive and often unnecessary investigations. This report describes 3 young women with HGPW. Clinical features were characterised by recurrent episodes of bilateral asymmetrical palpable purpuric lesions on the lower extremities that were precipitated by a prolonged increase in hydrostatic pressure (e.g. prolonged standing, tight stockings etc.) associated with constitutional features. In one patient the condition was secondary to sjogren's syndrome with type IV renal tubular acidosis. Laboratory abnormalities included a persistently elevated erythrocyte sedimentation rate, marked polyclonal hypergammaglobulinemia, and high titers of rheumatoid factor and anti-nuclear antibody of the anti-SSA (anti-Ro)/anti-SSB(anti-La) subsets. This topic is reviewed briefly with the emphasis that in its 'primary' form this condition could be considered a 'benign' systemic immunoinflammatory disease that requires neither extensive investigations nor any aggressive form of therapy. Greater awareness of HGPW may increase the frequency of its diagnosis, especially in the patient group with non-thrombocytopenic purpura or the so-called cutaneous vasculitic syndromes with 'palpable purpura'.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/16. agranulocytosis in sjogren's syndrome: two case reports and analysis of 11 additional reported cases.OBJECTIVES: To report 2 patients who presented with agranulocytosis that was found to be immune-mediated and associated with occult primary sjogren's syndrome (primary SS) and to identify and study similar cases reported in the literature. methods: Two patients encountered in 2 large medical centers over a period of 5 years were studied in detail. All reported cases of agranulocytosis in primary SS identified through a medline search were reviewed. RESULTS: Two patients presented with marked systemic symptoms alone or associated with recurrent infections. agranulocytosis with either a pattern of maturation arrest or a hypercellular reactive bone marrow was found and was associated with "acute phase" markers, hypergammaglobulinemia, a small paraprotein peak, and high rheumatoid factor titers. A diagnosis of immune-mediated agranulocytosis associated with an occult primary SS was established and was successfully treated with intravenous immunoglobulins or prednisone. Both patients subsequently developed skin vasculitis. This rare association of agranulocytosis and sjogren's syndrome was identified in 11 other cases and was the presenting manifestation of primary SS in 10 of 13 (77%) patients. CONCLUSIONS: agranulocytosis should be recognized as a rare but well-established association of primary SS. bone marrow neutrophil production may be affected, or neutrophils may be destroyed in the circulation, by both humoral and cellular immune-mediated mechanisms. agranulocytosis or neutropenia should be added to the varied hematologic manifestations of primary SS and may be its presenting feature and an important clue to diagnosis.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/16. A case of reactive plasmacytosis mimicking multiple myeloma in a patient with primary sjogren's syndrome.Primary sjogren's syndrome (pSS) is a chronic autoimmune disease with well-documented association of lymphoid malignancies during the progress of the disease. Although several types of malignancy and pseudomalignancy have been reported in pSS, low-grade non-Hodgkin's lymphomas are the most frequently observed. Reactive plasmacytosis mimicking myeloma is a very rare condition in association with pSS. We describe a 72-yr-old woman with pSS who presented with hypergammaglobulinemia, and extensive bone marrow and lymph node plasmacytosis, which mimicked multiple myeloma. In this patient, there was an abnormal differentiation of memory B cells to plasma cells in the peripheral blood suggesting underlying pathogenetic mechanism for this condition.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/16. Primary sjogren's syndrome after infectious mononucleosis.A healthy young woman developed primary sjogren's syndrome after protracted infectious mononucleosis. The diagnosis of primary sjogren's syndrome was supported by histologic evidence of sialadenitis in labial salivary glands, rheumatoid factor, hypergammaglobulinemia, the HLA-B8 phenotype, and a high titer antibody to the anti-La (SS-B) nucleoprotein that co-precipitated the small ribonucleic acids encoded by Epstein-Barr virus, EBER 1 and EBER 2, as well as "host" rna. There was strong humoral immunity to the Epstein-Barr nuclear and capsid antigens, but weak T-lymphocyte-mediated cytotoxicity to Epstein-Barr-transformed lymphoblasts, anergy to antigens used to elicit delayed-type hypersensitivity, and a low T-helper/T-suppressor cell ratio. The series of events initiated by infectious mononucleosis was attributed to a genetic defect in the immune response. association of viral rna with the La nucleoprotein resulted in a break in immunologic tolerance via a T-cell bypass effect with induction of anti-La (SS-B) by polyclonally activated B lymphocytes leading to autoimmune sialadenitis.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/16. A case of sjogren's syndrome with valvular diseases.The case of a 61 year old woman with sjogren's syndrome with aortic and mitral stenosis is reported. She suffered from rheumatic fever at a young age. Physical and echocardiographic examinations showed findings of mitral and aortic valve stenosis. In addition, she had experienced xerostomia, a gritty sensation in the eyes and Raynaud's phenomenon. blood examination showed hypergammaglobulinemia, positive rheumatoid factor, antinuclear and anti-Ro (SS-A) antibodies. The diagnosis of sjogren's syndrome was confirmed by sialography and biopsy of the labial salivary gland. The combination of valvular disease and sjogren's syndrome is rare and the etiological correlation is discussed.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/16. Granulocytopenia with marked lymphocytosis manifesting Sjogren syndrome.Sjogren syndrome is a multi-system disease leading to diverse organ involvement during its course [1, 2]. Hematologic abnormalities described in Sjogren syndrome include anemia, mild leukopenia [3, 4], eosinophilia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia, mixed cryoglobulinemia, and a variety of autoantibodies [5]. Marked lymphocytosis with granulocytopenia is distinctly unusual and has not been previously reported. We report a case of Sjogren syndrome who presented with constellation of the latter problems without prominent sicca manifestations.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/16. Chronic neutropenia: possible association with sjogren's syndrome.Three white males, ages 42-59, with chronic neutropenia are described. All 3 patients had splenomegaly, positive tests for rheumatoid factor, elevated erythrocyte sedimentation rates, hypergammaglobulinemia and bone marrow examinations which showed normal to increased cellularity. Two of the 3 patients had recurring arthralgias and positive tests for antinuclear antibodies. All 3 patients had evidence for decreased tearing suggestive of sjogren's syndrome, but in only 1 patient was a positive lip biopsy obtained. One patient had repeated cutaneous infections which ceased after splenectomy resulted in an increased neutrophil count. serum IgG neutrophil-binding and in vivo neutrophil kinetic studies on 1 patient were consistent with a humoral immune mechanism for his neutropenia.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/16. Eosinophilic fasciitis: report of a case with features of connective tissue diseases.A patient suffering from eosinophilic facsiitis is described and the literature reviewed. The original description of eosinophilic fasciitis was that of a benign scleroderma-like disease with eosinophilia, hypergammaglobulinemia and diffuse thickening and inflammation of the fascia and subcutaneous tissue. Our patient like a few others, presented additional findings, namely, Raynaud's phenomenon and mild myhositis. She is the first patient described with sjogren's syndrome. It is important that eosinophilic faciitis be recognized clinically because it seems to be a benign and treatable entity.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/16. Clinical analysis of recurrent hypergammaglobulinemic purpura associated with Sjogren syndrome.Recurrent purpuric lesions are occasionally seen in patients with Sjogren syndrome. hypergammaglobulinemia is one of the underlying precipitating factors of this condition. Clinical and histopathological analyses were performed on 5 cases of hypergammaglobulinemic purpura associated with sjogren's syndrome, and the effects of immunomodulatory therapy were evaluated with regards to these conditions. Three out of 5 cases were successfully treated with oral gold compound (auranofin) and one case with a low dose of cyclophosphamide. Episodic purpura subsided two months after initiation of therapy with improved serum IgG levels. Salivary flow and serum amylase levels also improved in some cases. Immunomodulatory therapy may be useful in managing recurrent purpura based on hypergammaglobulinemia associated with Sjogren syndrome.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
10/16. lambert-eaton myasthenic syndrome associated with sjogren's syndrome and discoid lupus erythematosus.A 65-year-old woman with facial erythema and hypergammaglobulinemia developed excessive fatigability. A diagnosis of lambert-eaton myasthenic syndrome (LEMS) was made from electrophysiological studies. She had symptoms and laboratory data compatible with probable sjogren's syndrome. skin biopsy revealed the histological findings of discoid lupus erythematosus. Treatment with 3,4-diaminopyridine resulted in the improvement of fatigability. LEMS should be recognized as a treatable complication of systemic autoimmune diseases.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
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