Several congenital disorders may cause neutropenia. The recent advances in the field of molecular biology have facilitated our knowledge concerning pathophysiological mechanisms leading to these disorders. The molecular basis of disorders with defective myelopoiesis (severe congenital neutropenia and cyclic neutropenia) and disorders due to defective RNA processing (Shwachman Diamond syndrome a...

We report a 51-year-old Vietnam War veteran with an unusual variant of macular amyloidosis presenting as poikilodermatous skin lesions. The extensive mottled brown pigmentation was checkered with small hypopigmented or normal skin-colored spots and intermingled with telangiectasia. Skin biopsy revealed subepidermal amyloid deposits. There was no evidence of extracutaneous involvements. This cas...

Neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, ranging from severe (<500 neutrophils/mm(3)) to mild (500-1500 neutrophils/mm(3)), which may also affect other organ systems such as the pancreas, central nervous system, heart, muscle and skin. Neutropenia can lead to life-threatening pyogenic infections whose severity is roughly inversely proportional ...

Chronic neutropenia (CN) is a rare disorder in children. It is defined by an absolute neutrophil count (ANC) below 1500 cells per cubic microliter of peripheral blood, lasting for at least 6 months. It can be acquired or congenital. More frequent – acquired chronic neutropenia is mainly caused by: malnutrition, exposure to drugs, chemical compounds and viral infections. CN is the cardinal sympt...

The object of this chapter was to provide an overview including relevant research progress some genetic disorders with periodontal manifestations. A number increase patient susceptibility disease, the latter exhibit rather rapid and aggressive presentations. Periodontal perhaps could be first detectable sign undiagnosed disorder. It is therefore important for dental practitioners familiar their...

Background Febrile neutropenia with childhood cancer alters the outcome significantly. To study the clinical and laboratory parameters, which predict the outcome among cancer patients with febrile neutropenia this study was undertaken. Materials and Methods The study included children less than 18 years with febrile neutropenia episodes. Clinical and hematological / laboratory parameters were r...

The underlying cause of neutropenia may be difficult to determine due to similar clinical presentation in many neutropenic conditions. The neutrophil protein hCAP-18 (pro-LL-37) is a major component of neutrophil secondary granules and in this prospective study we assessed the use of hCAP-18 levels in blood plasma for differential diagnosis of neutropenic patients (n = 133) of various aetiologi...

Chronic neutropenia with autoimmune diseases is associated mainly with rheumatoid arthritis (RA), as Felty's syndrome or large granular lymphocyte (LGL) leukemia, and with systemic lupus erythematosus (SLE). Recent advances have allowed better understanding regarding the mechanism of neutropenia and improved options for treatment. Target antigens for antineutrophil antibodies have been identifi...

Severe congenital neutropenia (SCN) is a heterogeneous bone marrow failure syndrome predisposing to myelodysplastic syndrome and acute myeloid leukaemia (MDS/AML). We studied 82 North American and Australian SCN patients enrolled in the Severe Chronic Neutropenia International Registry who were on long-term treatment with granulocyte colony-stimulating factor and for whom the neutrophil elastas...