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agammaglobulinemia/lafyèv

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Agammaglobulinemia, neutropenia, fever, and abdominal pain.

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Campylobacter fetus bacteremia with purulent pleurisy in a young adult with primary hypogammaglobulinemia.

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A 24-year-old man presented with fever and pleural effusion predominantly containing lymphocytes. Cultures of the pleural effusion and blood revealed Campylobacter fetus, and laboratory studies showed a low serum level of immunoglobulin. The patient was diagnosed with C. fetus pleuritis, bacteremia

[Recurrence of thymoma accompanied with hypogammaglobulinemia 20 years after surgery: a case report].

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We reported a case of recurrence of localized thymoma accompanied with hypogammaglobulinemia (Good's syndrome) 20 years after surgery. A 74-year-old man was admitted to this hospital because of mediastinal tumor and chronic pulmonary infection. He had been thymectomised at the age of 55 because of

Histoplasma meningitis with common variable hypogammaglobulinemia.

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Histoplasma meningitis (HM) has been reported to occur primarily in association with disseminated histoplasmosis (DH). We report a case of histoplasma meningitis occurring in a patient with common variable hypogammaglobulinemia (CVH) in which no manifestations of DH were observed. L. L., a

Rituximab Use and Hypogammaglobulinemia.

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BACKGROUND Rituximab is a genetically engineered chimeric (murine-human) monoclonal antibody (mAb) directed against CD20 antigen on the surface of B cells. Commonly reported adverse effects are chills and fevers, which are usually associated with the first infusion. Recent studies have shown an

[Shifting cellulitis in a patient with X-linked hypogammaglobulinemia].

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BACKGROUND In cases of immunodeficiency, a systemic infection may be revealed by atypical symptoms, particularly those involving the skin. METHODS The present case describes a 19-year-old male with X-linked hypogammaglobulinemia, or Bruton agammaglobulinemia, treated with intravenous immunoglobulin

B cell cytopenia in two brothers with hyper-IgD and periodic fever syndrome.

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We report on two brothers with hyperimmunoglobulinemia D (patient 1: serum immunoglobulin D [IgD] concentration initially 61 IU/ml, later on 340 IU/ml; patient 2: serum IgD concentration 144 IU/ml; normal <100 IU/ml, 97th centile) and periodic fever syndrome (HIDS). Both are compound heterozygous
A 30-year-old woman with a past medical history of autoimmune hemolytic anemia presented with fever. Blood cultures grew Campylobacter. Her medical history was significant for four prior episodes of Campylobacter gastroenteritis and bacteremia. She received ciprofloxacin for the index

Anhidrotic ectodermal dysplasia with transient hypogammaglobulinemia.

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An 8-month-old white boy with anhidrotic ectodermal dysplasia (AED) who was referred to the North Carolina Baptist Hospital because of recurrent respiratory infections and hypogammaglobulinemia is presented. His mother had partial expression of AED suggesting x-linked recessive inheritance in this
A splenectomized patient with hypogammaglobulinemia who was hospitalized because of a high-grade fever subsequently developed osteomyelitis. Although pus cultures were repeatedly sterile, polymerase chain reaction (PCR) analysis with use of 16S rRNA gene primers with a broad specificity detected

[Diagnostic value of lymphadenopathy associated with fever and inflammation of unknown origin: a study of 69 patients].

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OBJECTIVE The diagnosis of fever or inflammation of unknown origin (FUO/IUO) is guided by the search of clinical clues. Lymphadenopathy is thought to be helpful but its actual contribution has never been tested, and little is known about the main causes of FUO/IUO with lymphadenopathy. The aim of

Helicobacter cinaedi bacteremia presenting as macules in an afebrile patient with X-linked agammaglobulinemia.

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We describe a 54-year-old man with X-linked agammaglobulinemia (XLA) and Helicobacter cinaedi bacteremia, who presented with tender, hyper-pigmented skin macules without increased local warmth or fever. We propose that this presentation may be a characteristic early sign of bacteremia caused by H.

Recurrent purulent triaditis in a patient with congenital x-linked agammaglobulinemia.

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A patient with congenital x-linked agammaglobulinemia, who had two separate episodes of an apparent bacterial purulent hepatic triaditis in the absence of any known local predisposing factors, is presented. These episodes may reflect the increased susceptibility of an immunodeficient patient to

Agammaglobulinemia in a horse.

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Immunologic deficiency was suspected in an 18-month-old Standardbred horse with persistent fever, multifocal bacterial infection, and neutropenia with a large number of immature neutrophils. Serum protein electrophoresis revealed marked depression of the gamma-globulin fraction (0.2 g/100 ml).
X-linked agammaglobulinemia (XLA), caused by a mutation in the Bruton's tyrosine kinase (BTK) gene, is rarely reported in patients with recurrent hemophagocytic lymphohistiocytosis (HLH). This mutation leads to significantly reduced numbers of circulatory B cells and serum immunoglobulins in
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