T and B Cell Deficiency Associated with Yellow Nail Syndrome

Abstract

Yellow nail syndrome (YNS) is a rare disorder of unknown aetiology that is characterized by yellow nails associated with lymphoedema and chronic respiratory manifestations. There are no detailed immunological studies in YNS. In this study, we present first extensive immunological analysis of both adaptive and innate immunity in two patients with YNS. One patient has common variable immunodeficiency, whereas second patient has specific antibody deficiency syndrome. Severe lymphopaenia, a striking deficiency of naïve CD4+ and CD8+ T cells and total B cells, and increased transitional B cells were observed. T cell proliferative response to mitogens and antigens was significantly reduced in both patients. Both patients failed to make specific antibody response to pneumococci. Complement, natural killer cell activity and neutrophil oxidative burst were normal. Immunoglobulin administration resulted in decreased frequency and severity of infections, and an impressive effect was observed on lymphoedema and on the recurrence of pleural effusion. Our data show that YNS is associated with both T and B cell defects. Furthermore, Immunoglobulin may be beneficial in clinical manifestations of lymphoedema.

Yellow nail syndrome after allogeneic haematopoietic stem cell transplantation in two patients with multiple myeloma

“…1 In patients with immune deficiency, immunoglobulin administration may be useful. 3 In our patients, corticotherapy allowed an improvement of both GvHD and YNS manifestations.…”

Immunoglobulin replacement therapy for yellow nail syndrome

“…They described a single patient. 1 Yellow nail syndrome has an estimated prevalence of <1/1 000 000, 2 and <400 patients have been reported in the literature. 3 YNS most often occurs in adults over 50 years, with no sex predominance.…”

“…This triade is seen in one-third (27%-60%) of patients. 8,9 After reading the article by Gupta et al, 1 we decided to treat the patient with monthly intravenous injections of immunoglobulin (IVIG), observing an early improvement in YNS clinical manifestations. In particular, after the first 3 months of therapy, the lymphoedema and pleural effusion stopped to worsen and the patient no longer required pleural drainage or high dose of diuretics.…”

“…[2][3][4][5] The etiopathogenesis is unclear although it has been related to impaired lymphatic and vascular drainage and with immunological defects, including both T and B lymphocyte defects. 1,4 Respiratory tract involvement may include unilateral or bilateral pleural effusion(s), recurrent pneumonias, bronchiectasis, chronic cough and chronic sinusitis. 6 Lymphoedema may manifest many years after the nail changes.…”

A Case of Yellow Nail Syndrome Accompanying Idiopathic Interstitial Pneumonia; Successful Treatment with Clarithromycin, Methylprednisolone, and Alpha-Tocopherol

“…Gupta and colleagues have hypothesized that patients with YNS have frequent sinopulmonary infections due to immunodeficiency. The lymphedema and the pleural effusion of two immunocompromised patients were treated with intravenous immunoglobulin (IVIG) and responded well to this therapy (5). In this study, we report a case of YNS with bronchiectasis and idiopathic interstitial pneumonia which resolves after oral steroid and alpha-tocopherol treatment.…”