Primary Splenic Neutropenia—Revisited

Hess, Charles E.; Jain, Rushil; Ferguson, Wayne W.

doi:10.1097/00007611-198102000-00009

Cited by 2 publications

(2 citation statements)

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“…There is a long history of efforts to find an effective therapy for severe chronic neutropenia. Splenectomy for neutropenia was introduced in the 1930s, but is generally ineffective, and rarely performed, except for patients with Felty's syndrome [21]. Glucocorticosteroids may transiently elevate neutrophils in patients with idiopathic neutropenia, but they are generally ineffective or unsuitable for long-term treatment because of their side effects [22,23].…”

Section: Discussionmentioning

confidence: 99%

Severe chronic neutropenia: Treatment and follow‐up of patients in the Severe Chronic Neutropenia International Registry

et al. 2003

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Section: Discussionmentioning

confidence: 99%

Severe chronic neutropenia: Treatment and follow‐up of patients in the Severe Chronic Neutropenia International Registry

et al. 2003

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“…An effective therapy for SCN is still a challenge for clinicians; splenectomy is generally. ineffective and rarely performed, corticosteroids and G-CSF are transiently effective, but their use is limited because of the serious side effects (32)(33)(34). Recently, several groups have used IVIG to restore neutrophil count.…”

Section: Discussionmentioning

confidence: 99%

Hyper-IgM, Neutropenia, Mild Infections and Low Response to Polyclonal Stimulation: Hyper-IgM Syndrome or Common Variable Immunodeficiency?

Rosado

Diamanti

Cascioli

et al. 2011

Int J Immunopathol Pharmacol

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A young woman presenting respiratory infections, polyarthritis, severe neutropenia, and increased serum IgM was treated with Intravenous Immunoglobulin (IVIG) with good clinical and laboratory outcome followed by a loss of efficacy. The increased serum IgM associated to recurrent infections and autoimmune manifestations suggested the diagnosis of a hyper–IgM syndrome (HIGMs). The frequency of peripheral T cells, the expression of CD40 on the patients' B cells and CD40L on T cells and the Activation-Induced cytidine Deaminase (AID) and Uracyl-DNA glycosylase (UNG) at mRNA level was comparable to controls. In contrast, the frequency of B cells was one half of the healthy control and all cells showed an atypical phenotype. Although AID and UNG were normal, class-switch recombination was not very efficient because circulating switched memory were reduced and, once stimulated with CpG, generated less antibody-secreting cells than controls. An increase in serum B Lymphocytes stimulator (BLyS) was also found. The patient presented a peculiar clinical and immunological phenotype fitting for many aspects of both HIGM4 and Common Variable Immunodeficiency (CVID). These findings underline the need to better explore the complex link between these two diseases.

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Primary Splenic Neutropenia—Revisited

Cited by 2 publications

References 0 publications

Severe chronic neutropenia: Treatment and follow‐up of patients in the Severe Chronic Neutropenia International Registry

Severe chronic neutropenia: Treatment and follow‐up of patients in the Severe Chronic Neutropenia International Registry

Hyper-IgM, Neutropenia, Mild Infections and Low Response to Polyclonal Stimulation: Hyper-IgM Syndrome or Common Variable Immunodeficiency?

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