1/9. 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) |
2/9. Hypergammaglobulinemic purpura of Waldenstrom.Hypergammaglobulinemic purpura of Waldenstrom is characterized by hypergammaglobulinemia, recurring purpura, an elevated erythrocyte sedimentation rate, and the presence of rheumatoid factor indicative of circulating immune complexes. There is a significant association with autoimmune diseases, especially sjogren's syndrome and lupus erythematosus. Hypergammaglobulinemic purpura is considered primary when there is no other associated disease or secondary when associated with other diseases, usually autoimmune. Immune derangements are fundamental in its pathogenesis, although its cause is still unknown. Therapy is unrewarding and is probably unnecessary for this usually benign condition. Three cases are presented that are representative of patients with hypergammaglobulinemic purpura.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/9. immunoblastic lymphadenopathy with purpura and cryoglobulinemia.immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/9. Hypergammaglobulinemic purpura in childhood. Report of two cases and review of the literature.Hypergammaglobulinemic purpura is a rare syndrome characterized by long-standing recurrent purpura of the lower limbs, polyclonal hypergammaglobulinemia, increased erythrocyte sedimentation rate and high titer rheumatoid factor positivity. The syndrome can occur isolated or in association with a systemic disease. Few cases have been described in childhood. We report two cases together with a review of the paediatric literature.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/9. Immune complexes in the pathogenesis of hypergammaglobulinemic purpura.Direct immunofluorescence (DIF) was performed and circulating immune complex (CIC) levels evaluated in three patients with hypergammaglobulinemia purpura (HGP) of Waldenstrom. The Raji cell immunoradiometric assay was used to detect complexes of both IgG and IgM type. IgM and C3 were detected in blood vessel walls of all three patients. Elevated levels of IgG complexes were detected in two patients, and elevated levels of IgM complexes were detected in all three patients. Clinical improvement after plasmapheresis was noted in one patient. A cause-and-effect relationship between CIC and the inflammation of the superficial dermal blood vessels is postulated.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/9. Nodular pulmonary amyloidosis associated with benign hypergammaglobulinemic purpura.A 59-year-old woman, who had had benign hypergammaglobulinemic purpura for three years, developed a solitary amyloid nodule of the lung. The benign hypergammaglobulinemia was associated with an IgG band of restricted heterogeneity and IG2-IgG3 subclass containing complexes of intermediate size with rheumatoid factor activity. serum levels of IgA and IgM were also increased. autopsy after death from an unrelated cause revealed no amyloid in the remainder of the lungs or other organs and no evidence of lymphoproliferative disorder.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/9. purpura associated with hypergammaglobulinemia, renal tubular acidosis and osteomalacia.Two patients with hyperglobulinemia associated with purpura were studied. One had features of sjogren's syndrome, while the other appeared to have a primary condition -- "chronic benign purpura". Both patients also had renal tubular acidosis, osteomalacia and renal calculi, with disturbed calcium metabolism and acid-base balance. autoantibodies were detected in the serum of both patients, and mononuclear cell infiltrates were noted in skin and kidney biopsies from both.- - - - - - - - - - ranking = 4keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/9. Waldenstrom hypergammaglobulinemic purpura and pregnancy.BACKGROUND: Waldenstrom hypergammaglobulinemic purpura is characterized by hypergammaglobulinemia, recurring purpura, and an elevated erythrocyte sedimentation rate. It is a rare disease and, to our knowledge, there have been no previous reports of its presence during pregnancy. We report a patient with this disease whose pregnancy was complicated by severe fetal growth restriction (FGR) and acute fetal distress. CASE: A 24-year-old primigravid woman with a history of Waldenstrom hypergammaglobulinemic purpura and renal insufficiency developed FGR at 32 weeks' gestation. Cesarean delivery was performed at 33.5 weeks because of acute fetal distress, and a 1305-g male infant was delivered. Neonatal outcome was successful. No deterioration of the woman's medical condition occurred during or after her pregnancy. CONCLUSION: Successful pregnancy outcome is possible in women with Waldenstrom hypergammaglobulinemic purpura. In view of the risk of FGR, close monitoring of fetal growth and well-being is recommended in women with this condition.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/9. Primary hypergammaglobulinemic purpura associated with IgG2 deficiency. A case report.Hypergammaglobulinemic purpura is a rare disease in children. We report a case of a 12 year-old girl with a history of frequent infections. We found the presence of IgG2 deficiency despite polyclonal hypergammaglobulinemia. An IgG subclass determination should be obtained in every child with polyclonal hypergammaglobulinemia and features of immunodeficiency.- - - - - - - - - - ranking = 2keywords = hypergammaglobulinemia (Clic here for more details about this article) |