Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bone marrow hemophagocytosis may occur as an incidental finding, or it may be a manifestation of a systemic and potentially lethal disorder. When systemic, the proliferation is termed hemophagocytic lymphohistiocytosis (HLH), a clinicopathologic entity characterized by a widespread proliferation of benign hemophagocytic histiocytes, fever, pancytopenia, deranged liver function, and frequently coagulopathy and hepatosplenomegaly. A variety of infectious agents, including Epstein-Barr virus (EBV), cytomegalovirus (CMV), human herpesvirus 6 (HHV6), and parvovirus B19 (PVB19), have been associated with HLH, but the relative frequency of each using one technique has not been evaluated. In addition, infectious causes of incidental bone marrow hemophagocytosis, not occurring in the setting of HLH, have not been evaluated. Review of bone marrow reports from bone marrow examinations done between December 1986 and June 1997 showed that 20 children aged 2 months to 15 years had bone marrow examinations that indicated hemophagocytosis. Archival materials from 19 patients were successfully retrieved, and DNA was extracted from archived unstained coverslips with subsequent polymerase chain reaction for EBV, CMV, HHV6, and PVB19 genomic DNA. DNA extracted from 16 bone marrow specimens of age-matched children was used as negative controls. Eleven of the 19 patients fulfilled the clinical and pathological criteria for HLH; the remaining eight patients had isolated hemophagocytosis without a systemic presentation. Viral DNA was detected in 8 of 11 patients with HLH but in none of eight patients with isolated hemophagocytosis. EBV was present in five of the bone marrows, followed in frequency by HHV6, CMV, and PVB19. Infection with more than one agent was present in three patients. Only one control patient was positive for HHV6 DNA; the remaining control patients were negative for all viruses. Viral infection, detected by PCR analysis of bone marrow, is a common finding in patients with HLH but not in patients with isolated bone marrow hemophagocytosis. This technique may provide another marker to aid in the diagnosis of HLH and suggests a different cause of hemophagocytosis occurring in patients with and without HLH.
 ...
PMID:Polymerase chain reaction amplification of archival material for Epstein-Barr virus, cytomegalovirus, human herpesvirus 6, and parvovirus B19 in children with bone marrow hemophagocytosis. 978 44

An 11-month-old patient with parvovirus infection mimicking juvenile myelomonocytic leukemia (JMML) is presented. The patient's history, presenting physical and laboratory features, was suggestive of JMML and consisted of fever, hepatosplenomegaly, lymphadenopathy, desquamation of the skin, anemia, leukocytosis with monocytosis and trilineage dysplastic findings of the peripheral blood and bone marrow. However, positive IgM titers for parvovirus B19 followed by seroconversion, negative cytogenetics and the benign follow-up of the patient suggested acute parvovirus infection as an etiologic factor for development of dysplastic features in the patient, and thus is recommended for consideration in the differential diagnosis of MDS. Although parvovirus B19 infection mimicking MDS has previously been shown in two patients with spherocytosis and one with subclinical immune deficiency; to our knowledge, the present report is the first describing the association of acute parvovirus B19 infection with dysplastic features mimicking myelodysplasia (MDS) in a child without a demonstrable underlying hematolymphoid disorder.
 ...
PMID:Acute parvovirus B19 infection mimicking juvenile myelomonocytic leukemia. 1107 69

Leukoerythroblastosis is a rarely observed disease characterized by the presence of leukocytosis, erythroid and myeloid blast cells in peripheral blood. To our knowledge, it had not been diagnosed in a premature newborn before the case we report have.A female baby weighing 1164 grams, who was born prematurely at the 29th week of gestation by Cesarean section was referred to our newborn intensive care unit due to prematurity and respiratory distress with no prenatal pathological findings. Physical examination revealed tachypnea and hepatosplenomegaly. Routine laboratory measurements showed significant leukocytosis (85,000/mm3) and anemia (Hb: 9.6 g/dL and Hct: 27.6%). The platelet count was normal. The peripheral blood smear suggested leukoerythroblastosis with the presence of nucleated erythrocytes, monocytosis, and 4% blasts. Bone marrow cytogenetic examination was normal. Parvovirus B19 Ig G and M serology were detected to be positive. The etiological factors observed in leukoerythroblastosis occurring during neonatal and early childhood period are congenital-postnatal viral infections, juvenile myelomonocytic leukemia and osteopetrosis. To our knowledge, no case of leukoerythroblastosis in such an early phase has been reported in the in literature. As a result, premature delivery and leukoerythroblastosis were thought to have developed secondary to intrauterine parvovirus B19 infection. Leukoerythroblastosis is a rarely observed disease characterized by the presence of leukocytosis, erythroid and myeloid blast cells in peripheral blood. It is reported that it can be observed following hematologic malignancies especially juvenile myelomonocytic leukemia, acute infections, hemolytic anemia, osteopetrosis, myelofibrosis, neuroblastoma and taking certain medicines. To our knowledge, it has not been diagnosed in a premature newborn before. Here we the case of a newborn who was referred to our intensive care unit due to being born prematurely at the 29th week of gestation and diagnosed with leukoerythroblastosis.
 ...
PMID:Premature labor and leukoerythroblastosis in a newborn with parvovirus B19 infection. 1626 29

We report a patient with acute heart failure due to human parvovirus B19 infection. The patient was a 36-year-old man with polyarthralgia, fatigue and swelling of his upper eyelids and all four limbs. These symptoms disappeared, but 5 days after the first consultation, the patient presented with severe exertional dyspnoea, chest pain and swelling of his whole body. Erythema was observed on the skin of hands, fingers and abdomen. Pleural and pericardial effusion, ascites and hepatosplenomegaly were detected. Laboratory examination showed positive results for anti-human parvovirus B19 IgM and B19 DNA in the serum. A diagnosis of acute heart failure by pericarditis caused by B19 was made. This case report suggests that B19 should be considered as a cause of acute heart failure through acute pericarditis.
 ...
PMID:Acute heart failure associated with human parvovirus B19 infection. 1835 55

A 5-year-old male, drowsy, jaundiced child presented with fulminant hepatitis and had HAV and HEV infection. He had hepatic encephalopathy grade 1, fever, pallor, hypotension, crepitations in his right lung base and hepatosplenomegaly with dyspnoea. He had highly raised liver enzymes and hypoalbuminemia (2.8 g/dl) but anemia (hemoglobin of 7.7 g/dl and 5.7 g/dl 2 days later), reticulocytopenia and severe thrombocytopenia (44 x 10(9)/l) were unexplained. Parvovirus B19-specific IgM antibodies and B19 DNA were found in the serum of the child. Chest X-ray showed pleural effusion and bronchopneumonia, while blood culture isolated coagulase-negative staphylococci (BACTEC 9120) and he had low oxygen saturation. Hence, he was treated with IV amoxicillin+ clavulinic acid and oxygen inhalation. He had seizures and cardiac arrest but was revived. On the third day his condition worsened and the child died despite intensive care. Hence it is concluded that his anemia and thrombocytopenia were B19 induced and this might have aggravated or caused fulminant hepatitis.
 ...
PMID:Parvovirus B19-induced thrombocytopenia and anemia in a child with fatal fulminant hepatic failure coinfected with hepatitis A and E viruses. 1920 89

The role of Parvovirus B19 in acute leukemia is under debate. This study aimed to detect parvovirus B19 DNA together with its antibodies in the sera of children with recent acute leukemia and those with acute leukemia receiving chemotherapy to clarify the contribution of this infection to changes observed in hematological and clinical presentations in these populations. Two groups were included: Group I comprised 45 children with acute leukemia receiving chemotherapy and Group II comprised 40 children with recently diagnosed acute leukemia. Serum parvovirus B19 IgG and IgM were investigated by enzyme-linked immunosorbant assay and the virus DNA was sought by polymerase chain reaction assay. Viral DNA was found in 22.2% of Group I patients and in 45% of Group II patients. Hemoglobin levels were significantly reduced in patients with recent infection, accompanied by statistically significant lymphocytosis in Group I patients. Group II patients with recent infection had marked neutropenia with lymphocytosis and thrombocytopenia. There was statistically significant lymphadenopathy and hepatosplenomegaly in patients with recent infection in both groups. Parvovirus B19 infection is an important cause of cytopenia in children with acute leukemia both when recently diagnosed and receiving chemotherapy. This can affect the schedule of chemotherapy. Moreover, the presence of Parvovirus B19 is associated with marked lymphadenopathy and hepatosplenomegaly.
 ...
PMID:Clinical and hematological study for Parvovirus b19 infection in children with acute leukemia. 1926 Oct 33

A 10-year-old girl was admitted with fever, cough, maculopapular rash, hemoptysis, dark-colored urine, edema, multiple lymphadenopathies, and hepatosplenomegaly. She developed acute glomerulonephritis during the course of these complex clinical features. Laboratory data showed hematuria, proteinuria, and hypocomplementemia. Serological tests showed positive human parvovirus B19 (HPVB19)-specific immunoglobin M (IgM) and HPVB19 DNA was detected in the patient's serum using polymerase chain reaction (PCR). Renal biopsy revealed acute endocapillary proliferative glomerulonephritis (AEPGN) with coarse granular C3 depositions in a "starry sky pattern," which is more peculiar to poststreptococcal glomerulonephritis. Electron microscopy showed subendothelial and small hump-shaped subepithelial electron-dense deposits in glomerular capillary walls. There was no evidence of either any mycobacterial, staphylococcal, or streptococcal infection, and the diagnosis of Goodpasture syndrome and connective tissue disorders was excluded during clinical and laboratory investigations. A diagnosis of HPVB19-induced pleuropneumonitis and glomerulonephritis was made. Through a literature search there was no documented pediatric case of AEPGN induced by HPVB19, and this case represents, to our knowledge, the first time that a direct relationship between parvovirus infection and AEPGN has been demonstrated in a child.
 ...
PMID:Parvovirus B19-induced multisystemic vasculitis and acute endocapillary proliferative glomerulonephritis in a child. 2044 92

Parvovirus B19 is the etiologic agent of erythema infectiosum (fifth disease), a fever-rash illness occurring in childhood. We present a 10 month old child with high grade fever for 10 days, generalized tonic-clonic seizure, bilateral cervical lymphadenopathy, generalized maculopapular rash, hematemesis and malena. Bone marrow aspiration and liver biopsy were done. EBV serology and parvovirus PCR were also performed. Bone marrow aspiration and biopsy showed giant pro-erythroblast consistent with parvovirus infection. PCR showed amplification of parvovirus genomic sequences. Present case highlights an atypical presentation of Parvovirus B19 infection as fever, rash and hepatosplenomegaly.
 ...
PMID:Seizure and hepatosplenomegaly-rare manifestation of parvovirus B-19: a case report and review of the literature. 2176 Aug 6

Anemic syndrome in childhood requires a diagnosis and urgent treatment guided by systematic protocols that can avoid unnecessary additional testing. The case of a 4 year-old girl with fatigue and intermittent fever of 7 days duration, accompanied by abdominal pain is presented. She had regular general health status, with mucocutaneous jaundice, a grade III/VI/iv murmur, and painful abdomen with hepatosplenomegaly. The blood analysis showed a hypo-regenerative anemia with increased LDH and indirect bilirubin. The Coombs Test was negative, with spherocytes being observed in the peripheral blood smear. The IgM and IgG were positive for parvovirus B19 IgM and Epstein Barr virus, leading to the diagnosis of aplastic crisis in a patient with hereditary spherocytosis. No specific treatment was required. Under the suspicion of anemic syndrome in emergencies, the ABCDE sequence must be followed. Through the history, physical examination and basic laboratory tests, an initial diagnostic approach can be made. Specific etiological tests should be based on this first study.
...
PMID:[Aplastic crisis due to parvovirus B19 and Epstein-Barr virus in a patient with hereditary spherocytosis]. 2462 5

An increased prevalence of syphilis has been observed in many developed countries over the last decade. During pregnancy, syphilis can affect the fetus through development of nonspecific symptoms such as microcephaly, ascites, hepatosplenomegaly, dilated and echogenic bowel, placentomegaly, and, uncommonly, fetal hydrops. Congenital syphilis also leads to hematologic abnormalities such as anemia, thrombocytopenia, leukopenia, and leukocytosis. We present a case of nonimmune fetal hydrops with anemia related to syphilis infection. Diagnosis was confirmed by a maternal serological test and microbiological testing on amniotic fluid, umbilical cord, and placental tissues. The patient was treated with penicillin and the fetus received an intrauterine red blood cell transfusion, but fetal death occurred shortly after. Such a presentation is mostly related to parvovirus B19, and syphilis etiology is poorly mentioned because physicians have rarely seen early congenital syphilis in the past. However, given the increasing prevalence of this disease in the adult population, clinicians should remain alert to the various presentations of congenital syphilis.
...
PMID:Syphilis Infection: An Uncommon Etiology of Infectious Nonimmune Fetal Hydrops with Anemia. 2513 25


1 2 Next >>