we present a 24-year-old female referred with non-healing wound of a few days duration on anterior aspect of her right foreleg. biopsy of the wound was reported to be pyoderma gangrenosum on pathologic report. further work up of the patient for high grade fever and occasional leukopenias revealed the diagnosis of cyclic neutropenia. treatment with granulocyte colony-stimulating factor (g-csf) r...

cyclic neutropenia is a rare immunodeficiency syndrome, characterized by regular periodic oscillations in the circulating neutrophil count from normal to neutropenic levels through 3 weeks period, and lasting for 3-6 days. in order to determine the clinical features of cyclic neutropenia, this study was performed. seven patients with cyclic neutropenia (3 males and 4 females), who experienced n...

Human cyclic neutropenia occurs in children and adults. Adult-onset cyclic neutropenia is an acquired disease characterized by increased numbers of large granular lymphocytes (LGL), in contrast to childhood-onset cyclic neutropenia in which LGL counts are normal. We investigated the clonality of lymphocytes in these two groups of patients by assessing the rearrangement status of the T cell rece...

Human cyclic neutropenia shows many features in common with the animal model of cyclic neutropenia in grey collie dogs. Until now, however, evidence was lacking that cyclic neutropenia in man as in the dog is caused by a defect in a transplantable hematopoietic stem cell. A patient is presented who, while undergoing bone marrow transplantation as treatment for acute lymphoblastic leukemia in re...

Cyclic neutropenia is a rare hematologic disorder, characterized by repetitive episodes of fever, mouth ulcers, and infections attributable to recurrent severe neutropenia. Fluctuations in blood cells are due to oscillatory production of cells by the bone marrow. Recent genetic, molecular, and cellular studies have shown that autosomal-dominant cyclic neutropenia and sporadic cases of this dise...

Congenital neutropenia and cyclic neutropenia are disorders of neutrophil production predisposing patients to recurrent bacterial infections. Recently the locus for autosomal dominant cyclic neutropenia was mapped to chromosome 19p13.3, and this disease is now attributable to mutations of the gene encoding neutrophil elastase (the ELA2 gene). The authors hypothesized that congenital neutropenia...

A case of generalized amyloidosis associated with cyclic neutropenia is presented. A 24-yr-old female with cyclic neutropenia died from intestinal obstruction caused by necrosis and perforation of the small intestine. Post-mortem examination revealed generalized amyloidosis involving almost all organs. Amyloid deposits were prominent, especially in the alimentary tract, kidneys, spleen, and sma...

Human cyclic neutropenia occurs in adults as well as children. Clinical illness is similar in the childhood and adult diseases, but distinctly different modes of onset suggest heterogeneity in its pathophysiology. We studied seven patients with cyclic neutropenia, three with disease acquired in adulthood, and four with the childhood-onset disorder. All three patients with adult-onset cyclic neu...