[A case of disseminated nontuberculous mycobacteriosis during purpura associated with hypergammaglobulinemia and hepatitis type C].

A 74-year-old woman with hepatitis due to hepatitis C virus followed up using oral predonisolone (3 mg/day) for two years because of hypergammaglobulinemia-associated purpura reported fever and lumbago in February 2005. Upon admission in June, she was found in chest-computed tomography to have atelectasia in the right middle lung lobe and a nodule with a cavity in the right lower lobe. She tested positive for tuberculous glycolipid antibody. Gallium scintigraphy showed an abnormal accumulation in the lower lumbar vertebra. Magnetic resonance imaging showed abnormal enhancement at L4, L5, and their intervertebral disc. Mycobacterium intracellulare (M. intracellulare) was detected in blood culture, bronchoalveolar lavage, and a biopsy specimen from the intervertebral disc, yielding a diagnosis of disseminated nontuberculous mycobacteriosis (NTM) due to M. intracellulare. She was treated with clarithromycin (CAM), ethambutol (EB), and rifampicin (RFP), but EB and RFP were discontinued due to of the fever they induced. She was then treated with a combination of CAM, levofloxacin, and streptomycin and followed up as an out patient. Based on case reports of disseminated NTM infection in Japan, the prognosis is poor and a protocol must be established for its treatment.

Combination therapy of prednisolone and mizoribine improves cryoglobulinemic vasculitis with purpura and skin ulcers.

Conventional treatment is not standardized for hepatitis C virus-negative cryoglobulinemia, but corticosteroids, immunosuppressive agents, and plasma exchange typically improved the symptoms. Mizoribine is an immunosuppressive agent that was developed in Japan and has been found to inhibit the proliferation of lymphocytes, especially B cells. We have encountered an elder patient who had hepatitis C virus-negative, type II cryoglobulinemic vasculitis with leg purpura and skin ulcers. Her symptoms improved and cryoglobulin disappeared by the combination therapy of prednisolone and mizoribine. We speculate the action mechanism of this therapy is due to immunosuppressive effects including up-regulation of the efficacy of prednisolone by mizoribine.

Juvenile-onset hypergammaglobulinemic purpura and fetal congenital heart block.

Waldenström'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.

Benign hypergammaglobulinemic purpura of Waldenström associated with Sjögren's syndrome. Case report and review of immunologic aspects.

Since its first description, fewer than 150 cases of benign hypergammaglobulinemic purpura of Waldeström have been reported. There is a preponderance of females with this disorder, which is characterized by long-standing purpuric vasculitic lesions usually in the lower extremities, increased sedimentation rate, anemia, leukopenia, and hyperglobulinemia with a normal clotting process. Numerous associations have been shown between this and other systemic disorders such as Sjögren's syndrome, systemic lupus erythematosus, a rheumatoid arthritis. A 40-year-old woman is described who had an 18-year history of recurrent purpura and increased IgG levels. Skin biopsy showed acute vasculitis, and immunofluorescent study revealed fibrinogen precipitation and C3 deposition. Serologic evaluation demonstrated the presence of rheumatoid factor and antinuclear antibodies (1:80). Raji assay showed increased circulating immune complexes, and the T cell subsets were normal. The purpura was associated with symptoms and physical findings of Sjögren's syndrome. On the basis of serologic and skin biopsy findings, an autoimmune origin of the disease is postulated.

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.

Waldenström hypergammaglobulinemic purpura and pregnancy.

BACKGROUND: Waldenström 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 Waldenström 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 Waldenström hypergammaglobulinemic purpura. In view of the risk of FGR, close monitoring of fetal growth and well-being is recommended in women with this condition.

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.

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.

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.

Benign hypergammaglobulinemic purpura in a patient with ulcerative colitis and peripheric arthritis.

A 32-year-old patient is described presenting with ulcerative colitis and oligoarthritis who later developed benign hypergammaglobulinemic purpura. This diagnosis was confirmed by clinical investigation, immunological findings as well as by histological and immunofluorescent examination. He was successfully treated with steroids and azathioprin. The relevant literature is reviewed.

Peripheral corneal ulcer associated with benign hypergammaglobulinemic purpura.

We describe a 56-year-old woman with benign hypergammaglobulinemic purpura in whom scleritis, acute stromal keratitis and peripheral corneal furrowing developed. Initial therapy with topical antibiotics and steroids failed to control the scleritis and keratitis. Subsequent treatment with plasmapheresis led to a decrease in circulating immune complex and serum immunoglobulin levels, with resolution of the ocular inflammation. After 8 years of follow-up the serum IgG and IgM levels were again elevated; however, the corneal disease remained quiescent. Plasmapheresis may have a role in the therapy of this and other immune-complex-mediated ocular diseases.

[Distal tubular renal acidosis associated with hyperglobulinemic purpura and Sjõgren's syndrome]. / Acidosis tubular renal distal asociada a púrpura hipergammaglobulinémica y síndrome de Sjögren.

A 35-year-old female with a diagnosis of Waldenström's benign hypergammaglobulinemic purpura developed bilateral renal stones during the course of her disease. Evaluation disclosed type I distal tubular renal acidosis. In addition, the patient developed Sjögren's syndrome seven years later. The etiology and pathogenesis of renal tubular acidosis secondary to autoimmune disease are reviewed, and the exceptional occurrence of the three conditions in a single patient is commented.