Pure red blood cell aplasia associated with parvovirus B19 infection in large granular lymphocyte leukemia.
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Papular-purpuric "gloves and socks" syndrome (PPGSS) is a rare acute dermatosis characterized by pruritic erythematous and slightly papular lesions on the hands and feet in a "gloves and socks" distribution associated with oral aphtoid lesions and fever. It was first described in 1990 by Harms et al. Until now 17 cases have been reported. In five of these cases an association with Parvovirus-B19 (PB19) was observed, suggesting that PPGSS could be another manifestation of PB19 infection. Since PB19 cannot be shown in all the cases, this virus should be considered as one possible etiologic factor and other viruses may be responsible for this entity as well. A 42 year old Danish woman with PPGSS is described. This case could not be associated to primary PB19 infection.
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FUNDAMENTALS: [corrected] Human Parvovirus B19 has been identified as the etiological agent implicated in several clinical forms of disease. The aim of this study was to define the clinical aspects observed in the children with human parvovirus infection in our hospital. METHODS: A retrospective study was done in 31 pediatric cases observed in our hospital from January-92 to July-94; all had positive IgM against Parvovirus (determined by enzymoinmunoanalysis). RESULTS: The most frequent signs and symptoms were fever in 66.6% of the cases and skin findings in 48%, manifested as typical infectious erythema in 26% and polimorphic nonspecific exanthema in the rest. There were variable blood manifestations, observed in 11 children (35%): Immune Thrombocytopenic purpura, Chronic anemia in immunocompromised hosts, bone marrow erithrophagocytosis and hemolytic crises in non-hemolytic anemia patients. The abnormal blood findings were mostly observed in children with compromised immunity. Articular disease was presented in four children (12%). Long lasting fever was observed in only one case. CONCLUSIONS: In our sample the diversity of clinical forms of presentation of Parvovirus B19 infection is well documented as it is reported by several authors. Although other studies as polymerase chain reaction (PCR), nucleic acid hybridization, direct visualization of the virus or its particles, or viral antigen detection are desirable to confirm the serologic studies.
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Knowledge of the pathophysiological mechanisms governing Parvovirus B19 infection has been modified with the recent discovery of its cellular receptor. The cell lines susceptible to infection can be identified on the basis of the nature and distribution of the virus in the human organism. The Parvovirus B19 can undergo two types of cycles within the host cell. The first is a complete cycle within the infected cell leading to cell death by lysis. In the second, the cycle aborts and cell death is caused by an association of immunological phenomena and the accumulation of viral proteins toxic to the cell. It is not known why complete cycles are restricted to a single erythrocyte line. These new data not only give a more precise explanation of the classical clinical expression of the disease, but also suggest possible mechanisms for the pathogenic power of the Parvovirus B19, some of which have already been proposed on the basis of clinical observations.
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Rheumatic complaints secondary to viral infections are usually brief, self-limited and nondestructive. They may accompany almost any type of viral illness, and the arthritic presentation is nonspecific. These manifestations are protean, ranging from arthralgia to vasculitis. Often the cause of the rheumatic complaint remains elusive because of the prompt resolution of the viral infection. Evaluation for autoimmune diseases should be postponed until the symptoms have been present for at least six weeks. However, some viral diseases, such as parvovirus, chronic hepatitis B virus and hepatitis C virus infections, can produce long-lasting rheumatic symptoms. Since the arthritis associated with hepatitis C infection has only recently been recognized, it is important to search for this association in patients who have atypical rheumatic complaints, risk factors for hepatitis and alterations in liver enzymes, so that an accurate diagnosis can be established and the pathophysiology can be better understood.
The pathogenesis of viral exanthems remains unclear. We have undertaken an immunohistochemical study of lesional skin biopsies in patients with the papular-purpuric gloves and socks syndrome (PPGSS) secondary to parvovirus B19 infection. Intracytoplasmic staining of the dermal endothelial cells, keratinocytes, and sweat glands was shown with an antibody to parvovirus B19. There were perivascular dermal infiltrates with T cells, sometimes with exocytosis. By polymerase chain reaction, virus DNA was detected in all skin biopsies and in one serum sample. The cutaneous manifestations of parvovirus infection seem secondary to infection of the endothelium and epidermis.
Anemia, mental status changes, and fatal respiratory failure complicated a febrile illness in a previously healthy 14-year-old black female. At autopsy, widespread fat emboli and bone marrow necrosis were found. Hemoglobin electrophoresis on an antemortem, pretransfusion specimen revealed hemoglobin S/beta+ thalassemia. Acute parvovirus B19 (PV B19) infection was suspected. Postmortem serum and a variety of paraffin-embedded tissues were assayed for PV B19 DNA using the polymerase chain reaction (PCR). The expected PCR product was identified in the serum specimen and in paraffin-embedded sections of bone marrow, kidney, spleen, parathyroid, thyroid, adrenal, and gastrointestinal tract: lung, liver, ovary, fallopian tube, uterus, brain, heart, and pancreas were negative. PV B19 infection is highly contagious and may be rapidly fatal in children with hemoglobinopathies by several mechanisms, including fat embolism. Therefore, there exists the risk of multiple deaths within a family. The acute infection may be easily and expeditiously diagnosed using serum or a variety of paraffin-embedded tissues.
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