2.An Adult with Aplastic Crisis induced by Human Parvovirus B19 as an Initial Presentation of Hereditary Spherocytosis.
Sook Eui OH ; Jung Han KIM ; Chi Hun CHOI ; Kwang Hyuk PARK ; Joo Young JUNG ; Young Iee PARK ; Min Jeong PARK
The Korean Journal of Internal Medicine 2005;20(1):96-99
The association between aplastic crisis and human parvovirus (HPV) B19 infection is well described in patients with sickle cell anemia. This association has also been described, although much less frequently, in patients with hereditary spherocytosis (HS). However, most cases of aplastic crises in patients with HS and induced by HPV B19 have been reported in children or adolescents. In this paper, we describe an aplastic crisis induced by HPV B19 in an adult with HS. A 34-year-old female presented with presyncope, febrile sensation, and myalgia. The complete blood counts showed severe anemia. The peripheral blood smear revealed spherocytosis with reticulocytopenia and pancytopenia. The direct Coombs' test was negative; the osmotic fragility test was positive. In the bone marrow aspirates, a few giant pronormoblasts with deep blue cytoplasm, pseudopods, and intracellular inclusion bodies were observed. The patient was given eight units of packed red blood cells. HPV B19 infection was proven by the presence of IgM antibodies to HPV B19 and the detection of viral DNA using the PCR technique. To the best of our knowledge, this is the first report in Korea that describes an adult with aplastic crisis presenting initially with HS.
Adult
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Anemia, Aplastic/*etiology
;
Female
;
Humans
;
Parvoviridae Infections/*complications/diagnosis
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Parvovirus B19, Human
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Spherocytosis, Hereditary/*diagnosis
3.Psoas Abscess Caused by Non-Typhoid Salmonella in a Patient with Severe Aplastic Anemia.
Chin Chi KUO ; Shih Chi KU ; Jann Tay WANG ; Ching Wei TSAI ; Vin Cent WU ; Wen Chien CHOU
Yonsei Medical Journal 2010;51(3):472-474
The clinical spectrum of infections caused by non-typhoid Salmonella spp. includes gastroenteritis, enteric fever, bacteremia, and extraintestinal localized complications, especially in immunocompromised hosts. Here we report a patient with severe aplastic anemia developing left iliopsoas abscess caused by non-typhoid Salmonella (NTS), which was successfully treated by prolonged antibiotic treatment and repeated debridement. Our data indicate that aplastic anemia is a risk factor for infection caused by NTS.
Anemia, Aplastic/*complications/microbiology
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Humans
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Male
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Middle Aged
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Psoas Abscess/*etiology/*microbiology
;
Salmonella Infections/*complications
4.Clinical features of hepatitis-associated aplastic anemia in children.
Yan-Long DUAN ; Run-Hui WU ; Shuang YANG ; Rui ZHANG ; Li ZHANG ; Xuan ZHOU ; Jin JIANG ; Mei JIN
Chinese Journal of Contemporary Pediatrics 2010;12(8):609-612
OBJECTIVETo study the clinical features of hepatitis-associated aplastic anemia (HAAA) in children.
METHODSThe clinical data of the children with newly diagnosed HAAA from January 2007 to December 2008 were respectively studied, including clinical manifestations, and blood routine, bone marrow examination, viral serology and immune function results as well as treatment and prognosis.
RESULTSA total of 8 children were confirmed as HAAA, accounting for 4.9% in children with aplastic anemia. There were 7 males and 1 female. The median age was 7.5 years (range 4.4 to 10.3 years) at diagnosis. They had negative serologic results and the causes of hepatitis could not be identified. The median interval from hepatitis occurrence to blood cell reduction was 6 weeks. Three cases were diagnosed as severe aplastic anemia and 5 cases as very severe aplastic anemia. Severe T cell immune disorders were found in all 8 cases. The percentage of Ts cells increased and the percentage of Th cells decreased significantly in the 8 children with HAAA. Four children survived after immune suppress treatment, three children died within one month after diagnosis and one child required own discharge without treatment.
CONCLUSIONSHAAA is more frequent in male school children. The children with HAAA have severe T cell immune disorders, with a higher early death rate. Immune suppress treatment is effective.
Anemia, Aplastic ; etiology ; immunology ; therapy ; Child ; Child, Preschool ; Female ; Hepatitis ; complications ; Humans ; Male ; Prognosis
5.Significance of paroxysmal nocturnal hemoglobinuria clone in immunosuppressive therapy for children with severe aplastic anemia.
Jun LI ; Su-Yu ZONG ; Zi-Xi YIN ; Yang-Yang GAO ; Li-Peng LIU ; Yang WAN ; Yang LAN ; Xiao-Wen GONG ; Xiao-Fan ZHU
Chinese Journal of Contemporary Pediatrics 2022;24(3):303-308
OBJECTIVES:
To study the association between paroxysmal nocturnal hemoglobinuria (PNH) clone and immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA).
METHODS:
A retrospective analysis was performed on the medical data of 151 children with SAA who were admitted and received IST from January 2012 to May 2020. According to the status of PNH clone, these children were divided into a negative PNH clone group (n=135) and a positive PNH clone group (n=16). Propensity score matching was used to balance the confounding factors, and the impact of PNH clone on the therapeutic effect of IST was analyzed.
RESULTS:
The children with positive PNH clone accounted for 10.6% (16/151), and the median granulocyte clone size was 1.8%. The children with positive PNH clone had an older age and a higher reticulocyte count at diagnosis (P<0.05). After propensity score matching, there were no significant differences in baseline features between the negative PNH clone and positive PNH clone groups (P>0.05). The positive PNH clone group had a significantly lower overall response rate than the negative PNH clone group at 6, 12, and 24 months after IST (P<0.05). The evolution of PNH clone was heterogeneous after IST, and the children with PNH clone showed an increase in the 3-year cumulative incidence rate of aplastic anemia-PNH syndrome (P<0.05).
CONCLUSIONS
SAA children with positive PNH clone at diagnosis tend to have poor response to IST and are more likely to develop aplastic anemia-PNH syndrome.
Anemia, Aplastic/drug therapy*
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Child
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Clone Cells
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Hemoglobinuria, Paroxysmal/etiology*
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Humans
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Immunosuppression Therapy
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Retrospective Studies
7.A commentary on a case of aplastic anemia applying for identification of occupational chronic severe benzene poisoning.
Hong-ping DENG ; Shi-xin ZHU ; Jian-yuan CAI
Chinese Journal of Industrial Hygiene and Occupational Diseases 2012;30(9):707-708
Anemia, Aplastic
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diagnosis
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etiology
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Benzene
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poisoning
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Expert Testimony
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Humans
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Male
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Occupational Diseases
;
diagnosis
8.Analysis on bone marrow feature of 56 clinic cases of benzene poisoning.
Jiang-feng LIU ; Wei HE ; Yong-yi WANG
Chinese Journal of Industrial Hygiene and Occupational Diseases 2011;29(1):64-67
OBJECTIVETo explore the bone marrow feature of hemopoietic system injured by benzene through analyzing 56 benzolism cases.
METHODSThe 56 benzolism cases were divided into mild poisoning group, midrange poisoning group, aplastic anemia group, pancytopenia group and leukemia group. All cases progressed bone marrow aspiration and smear, and counted hundred karyocytes by Wright-Giemsa tinct bone marrow smear to classification and observe the cells' feature.
RESULTSThe megakaryocytes and the extent of bone marrow hyperplasia were decreased by turns of mild poisoning group, midrange poisoning group and aplastic anemia group. The archaeocytes and juvenile cells proliferation in mild poisoning group and midrange poisoning group were inhibited and occurred cell paramorphia which related to intoxication. Comparing with the other groups and normal reference value, the pancytopenia group's percentage of bone marrow cells in karyocytes was significantly decreased (P < 0.01, P < 0.05) and the leukemia group's percentage of bone marrow cells in karyocytes was significantly increased (P < 0.01). The proportion of cell paramorphia and nucleus malformation of granulocytes and red blood cells in pancytopenia group and leukemia group were increased, especially in leukemia group.
CONCLUSIONWe saw the inhibition of archaeocytes and juvenile cells proliferation and some cell paramorphia appearances in mild poisoning and midrange poisoning cases of chronic benzolism. The abnormality changes which can be seen in bone marrow of severe benzolism cases were corresponding with the clinical classification.
Adult ; Anemia ; etiology ; pathology ; Anemia, Aplastic ; etiology ; pathology ; Benzene ; poisoning ; Bone Marrow ; pathology ; Bone Marrow Cells ; cytology ; Bone Marrow Examination ; Female ; Humans ; Leukemia ; etiology ; pathology ; Male ; Middle Aged