11/137. agranulocytosis in sjogren's syndrome: two case reports and analysis of 11 additional reported cases.OBJECTIVES: To report 2 patients who presented with agranulocytosis that was found to be immune-mediated and associated with occult primary sjogren's syndrome (primary SS) and to identify and study similar cases reported in the literature. methods: Two patients encountered in 2 large medical centers over a period of 5 years were studied in detail. All reported cases of agranulocytosis in primary SS identified through a medline search were reviewed. RESULTS: Two patients presented with marked systemic symptoms alone or associated with recurrent infections. agranulocytosis with either a pattern of maturation arrest or a hypercellular reactive bone marrow was found and was associated with "acute phase" markers, hypergammaglobulinemia, a small paraprotein peak, and high rheumatoid factor titers. A diagnosis of immune-mediated agranulocytosis associated with an occult primary SS was established and was successfully treated with intravenous immunoglobulins or prednisone. Both patients subsequently developed skin vasculitis. This rare association of agranulocytosis and sjogren's syndrome was identified in 11 other cases and was the presenting manifestation of primary SS in 10 of 13 (77%) patients. CONCLUSIONS: agranulocytosis should be recognized as a rare but well-established association of primary SS. Bone marrow neutrophil production may be affected, or neutrophils may be destroyed in the circulation, by both humoral and cellular immune-mediated mechanisms. agranulocytosis or neutropenia should be added to the varied hematologic manifestations of primary SS and may be its presenting feature and an important clue to diagnosis.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
12/137. Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis.Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.- - - - - - - - - - ranking = 44.089495326199keywords = bacterial infection, infection (Clic here for more details about this article) |
13/137. Early-onset group B streptococcal sepsis in a preterm infant with Kostmann syndrome.A preterm infant died of group B streptococcal sepsis 7 h after birth. The infant's complete blood count showed total agranulocytosis. Histopathology of the major organs showed significant bacterial invasion without infiltration of polymorphonuclear leucocytes. Examination of the bone marrow revealed normal cellularity of the granulocyte precursors with arrested maturation. These findings are consistent with Kostmann syndrome. CONCLUSION: It is suggested that in patients with deteriorating early-onset infection, underlying congenital abnormalities in host defence, such as Kostmann syndrome, should be considered.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
14/137. Influenza B pneumonia with Staphylococcus aureus superinfection associated with parvovirus B19 and concomitant agranulocytosis.An 11-year-old patient with anamnestic fever for 3 days and signs of upper respiratory tract infection underwent fulminant Staphylococcus aureus pneumonia with concomitant agranulocytosis. From autopsia influenza b virus and parvovirus B19 were detected by nucleic acid amplification technique (NAT). Specific IgG but no IgM points to preexisting parvovirus B19 infection. Whether in this case agranulocytosis can be interpreted as early manifestation of reactivated parvovirus B19 infection is under discussion. Therefore, parvovirus B19 could have provoked a foudroyant course of influenza B pneumonia which was superinfected with S. aureus.- - - - - - - - - - ranking = 7keywords = infection (Clic here for more details about this article) |
15/137. Is agranulocytosis following infectious mononucleosis caused by autoimmunity?A patient developed agranulocytosis after Epstein-Barr infection. bone marrow examination revealed normal myelopoiesis with reduced mature neutrophil polymorphonuclear cells. IgG-specific antineutrophil antibodies (anti-HNA-1a) were found in serum and on polymorphonuclear cells, suggesting that the agranulocytosis after infectious mononucleosis was caused by an autoimmune mechanism.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
16/137. Inflammatory pseudotumor of the liver in a patient with congenital granulocytopenia and HCV infection.Inflammatory pseudotumor (IPT) of the liver is a rare pathologic lesion. Although IPTs within the liver shows spontaneous regression, these lesions are frequently misdiagnosed as malignant on the basis of the clinical manifestation and the results of diagnostic imaging. With special regard to magnetic resonance imaging (MRI), differential diagnosis such as hepatocellular or cholangiocellular carcinoma (HCC/CCC) as well as regenerative liver lesions are discussed in a case of IPT with concomitant hepatitis c virus (HCV) infection and congenital granulocytopenia.- - - - - - - - - - ranking = 5keywords = infection (Clic here for more details about this article) |
17/137. agranulocytosis induced by vancomycin in an ESRD patient on CAPD.agranulocytosis is a rare adverse effect associated with prolonged vancomycin therapy, and is potentially serious, especially in end stage renal disease (ESRD) patients. We describe a continuous ambulatory peritoneal dialysis (CAPD) patient that developed vancomycin-induced agranulocytosis during treatment for methicillin-resistant staphylococcus aureus (MRSA)-associated external cuff infection and pneumonia. The agranulocytosis was rapidly resolved by granulocyte colony-stimulating factor (G-CSF) therapy and by the discontinuation of vancomycin.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
18/137. Recurrent granulocytic aplasia as clinical presentation of a persistent parvovirus B19 infection.We report a case of persistent infection with human parvovirus B19 (PVB19), manifesting clinically as recurrent agranulocytosis and in bone marrow biopsy as recurrent pure granulocytic aplasia. Persistence of parvovirus infection was documented by the presence of PVB19 dna and anti-PVB19-IgM antibodies in the serum for a period of 19 months. Granulocytic aplasia occurred only when anti-PVB19-IgG antibodies were not detectable in the serum and granulopoiesis showed immediate recovery with high dose intravenous immunoglobulin treatment. This case report suggests that the erythroid precursor cell may not be the only target cell of PVB19 infection. We suggest testing for active parvovirus infection in cases of aplasia of any lineage of the haematopoietic system.- - - - - - - - - - ranking = 8keywords = infection (Clic here for more details about this article) |
19/137. Invasive aspergillus infections in patients with a malignancy: description of an outbreak and overview of the literature.Improved results can be obtained by increasing the intensity of chemotherapy in patients with cancer. Prolonged granulocytopenia due to intensive chemotherapy schedules, however, is a frequent cause of infectious complications. Fungal infections, especially aspergillus infections, are being recognized with increasing frequency. We describe an outbreak of invasive aspergillus infections in six cancer patients treated with chemotherapy and we give an overview of the diagnostic and therapeutic dilemmas encountered in patients with invasive aspergillus infection.- - - - - - - - - - ranking = 8keywords = infection (Clic here for more details about this article) |
20/137. Necrotizing fasciitis in two children with acute lymphoblastic leukemia.Necrotizing fasciitis is a severe, soft tissue infection, and is an unusual condition in children. The cornerstone of therapy is prompt, aggressive surgical treatment. Despite vigorous treatment, mortality rates are high. We report the occurrence of necrotizing fasciitis in two children during the granulocytopenic phase of induction chemotherapy for acute lymphoblastic leukemia. The diagnosis and treatment of necrotizing fasciitis in these two children was made more difficult by their underlying disease and its chemotherapy. The successful treatment of their infections relied on a multimodality approach. Aggressive surgical debridement was the mainstay of therapy. Adjuvant therapy was vital to the successful outcomes and included meticulous wound care, intravenous hyperalimentation, appropriate antibiotics, and granulocyte transfusions.- - - - - - - - - - ranking = 2keywords = infection (Clic here for more details about this article) |
| <- Previous || Next -> |