Hypergammaglobulinemic purpura of Waldenström.

Hypergammaglobulinemic purpura of Waldenström is a rare syndrome that includes recurrent episodic purpura occurring mainly on the lower extremities and dorsum of the feet. The hallmark of this condition is polyclonal hypergammaglobulinemia primarily composed of IgG. Although the condition generally is benign, it may herald an underlying connective tissue disease or hematologic malignancy. We report a case of a 47-year-old woman with episodic purpura of 3 years' duration associated with Raynaud phenomenon.

Cutaneous macroglobulinosis deposits in a patient with IgM paraproteinemia/incipient Waldenström macroglobulinemia.

A 43-year-old healthy patient developed disseminated flat skin-colored to red-brown papules over a few months. These papules were the result of cutaneous IgM deposits representing the first symptom of a hitherto undiagnosed IgM paraproteinemia. This extremely rare skin manifestation of IgM paraproteinemia e. g. possibly incipient Waldenström macroglobulinemia should be included in the histopathological differential of eosinophilic dermal deposits.

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.

Hypergammaglobulinemic purpura of Waldenström: report of 3 cases with a short review.

Benign hypergammaglobulinemic purpura of Waldenström (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 Sjögren'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'.

Waldenström's hyperglobulinemic purpura: an unusual immune complex disorder.

A case of Waldenström's hypergammaglobulinemic purpura in a 37-year old woman is presented. The symptomatology was very mild and confined only to the skin, despite very high quantities of circulating immune complexes which were found in her serum. The unusual manifestations of Waldenstrom's purpura compared to other immune complex disorders is discussed.

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.

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.

"Benign" hypergammaglobulinemic purpura is not benign in pregnancy.

A woman presented at 25 weeks gestation in her first pregnancy with severe preeclampsia and an intrauterine death. It later emerged that she had Waldenstrom's benign hypergammaglobulinemic purpura. We discuss the implications of this diagnosis in pregnancy and explore possible management options during subsequent pregnancies.

Cryoglobulinemic purpura in visceral leishmaniasis.

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.

Sjögren,'s syndrome and benign hyperglobulinemic purpura of Waldenstrom.

This article describes the relationship between Sjögren's syndrome and benign hyperglobulinemic purpura of Waldenstrom (BHPW). A case is reported, and evidence is shown that they are the same disease differing only in some prominent clinical manifestations.