1/39. purpura hyperglobulinemica (Waldenstrom). A case of hyperglobulinemic purpura of Waldenstrom with involvement of trunk and arms of 10 years duration has been presented. The patient had mild anemia, hepatosplenomegaly, positive rheumatoid factor and no evidence of immonological abnormalities associated for with systemic lupus erythematosus. A brief review of clinical manifestation and pathogenesis is cited. ( info) |
2/39. Hypergammaglobulinaemic purpura presenting as reticulate purpura. Hypergammaglobulinaemic purpura, first described by Waldenstrom, is a rare skin disease. The essential features are the presence of purpura with polyclonal hypergammaglobulinaemia. We describe a case of hypergammaglobulinaemic purpura occurring in a Chinese man with reticulate purpura and haemorrhagic blisters. ( info) |
3/39. 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'. ( info) |
4/39. Hypergammaglobulinaemic purpura associated with IgG subclass imbalance and recurrent infection. We describe a child who initially presented with recurrent infections. He subsequently developed hypergammaglobulinemic purpura but responded well to corticosteroids and hydroxychloroquine. ( info) |
5/39. Systemic lupus erythematosus in twin sisters following ten years of hyperglobulinemic purpura (Waldenstrom). Uniovular twin sisters have been diagnosed 10 years ago as having hyperglobulinemic purpura (Waldenstrom) at age 12. The diagnosis was documented by purpura of the lower extremities, increased gamma-globulin after serum electrophoresis, and increased 7S component upon ultracentrifugation. In addition, there was an elevated ESR, a positive rheumatoid arthritis latex test, and their LE prep. was negative at that time. At a later date, however, both of them developed polyarthritis. After 9 years for the one and 10 years for the other, their LE prep. became strongly and constantly positive, while their gamma-globulin remained within abnormal limits. ( info) |
6/39. Hypergammaglobulinemic purpura in two sisters with sjogren's syndrome responding to colchicine treatment. purpura is the hallmark of hypergammaglobulinemic purpura (HP). It appears mainly after strenuous activity in the dependent areas of the body. Treatment is mostly symptomatic. Here we report two sisters with sjogren's syndrome (SS) and HP who had a remarkable response to colchicine treatment. ( info) |
| This is the first description of a patient with both polymyositis and Waldenstrom hyperglobulinemic purpura. There was evidence of circulating immune complexes, and immune deposits were found in dermal and muscular vessels. Similar electron-dense deposits were seen ultrastructurally in the basement membrane of both normal and abnormal microvasculature. The findings suggest that the muscle and skin lesions may be associated with deposition of circulating immune complexes in and around blood vessels, followed by complement activation and subsequent inflammation. ( info) |
| Visceral leishmaniasis may present with cytopenias along with the formation of many autoantibodies and, rarely, with the presence of mixed cryoglobulinemia, type II, resembling an autoimmune disease. The syndrome of mixed cryoglobulinemia is characterized by the triad of purpura, arthralgias, and asthenia, in conjunction with cryoglobulins in the serum. In this article mixed cryoglobulinemia, type II, was diagnosed in a negative for hepatitis b or C patient suffering from visceral leishmaniasis. Antimicrobial therapy against leishmania eliminated the cryoglobulin titer, as well as the clinical manifestations of cryoglobulinemia. The role of the immune system and the type of immune response for the formation of cryoglobulins are discussed. ( info) |
9/39. Juvenile-onset hypergammaglobulinemic purpura and fetal congenital heart block. Waldenstrom's hypergammaglobulinemic purpura (HGP) is a rare chronic disorder characterized by recurrent purpura on the legs, a polyclonal increase in serum gamma-globulin, an elevated erythrocyte sedimentation rate and a positive rheumatoid factor. A 30-year-old primigravid woman with 14 years of HGP was found to have fetal bradycardia at 25 weeks' gestation. Laboratory investigations demonstrated positive anti-Ro/SSA and anti-La/SSB antibodies in the maternal serum. Cesarean delivery was performed at 39 weeks, and a 2750-g female infant was born with complete atrioventricular block. Fortunately, the neonatal period has been uneventful without need for pace-making. Maternal HGP exacerbated just after delivery, but resolved within 1 week without treatment. physicians should be aware of the possible presence of neonatal lupus-related anti-Ro/SSA and anti-La/SSB autoantibodies in patients with HGP. Screening for these autoantibodies is important and could be used as a marker to identify and manage high-risk pregnancies. ( info) |
10/39. Peripheral neuropathy in monoclonal gammapathy with cryoglobulinemia and arteritis. We report a patient with IgM gammapathy, cryoglobulinemia, Raynaud's phenomena, purpura hyperglobulinemica of the legs and polyneuropathy. Endoneural vasculitis with infiltrations of eosinophilic and neutrophilic granulocytes and an extensive loss or wallerian degeneration of myelinated nerve fibers were seen on histopathologic examination of a sural nerve biopsy specimen. The microscopic picture differed somewhat from that observed previously in cryoglobulinemic vasculitis. Although vasculitis is most often believed to represent an immunologically mediated lesion, we propose an alternate explanation, namely, that the disease manifestations in the present case were secondary to cold-induced effects of the cryoglobulin on the microcirculation. ( info) |