Hemolytic crisis due to parvovirus B19 in hereditary spherocytosis in two siblings

Author(s):  
Meltem Erol ◽  
Ozlem Bostan Gayret ◽  
Ozgul Yigit ◽  
Mahir Tiras ◽  
Sultan Bent
2016 ◽  
Vol 6 (3) ◽  
pp. 194-195
Author(s):  
Meltem Erol ◽  
Ozlem Bostan Gayret ◽  
Ozgul Yigit ◽  
Mahir Tiras ◽  
Sultan Bent

2000 ◽  
Vol 76 (4) ◽  
pp. 323-619 ◽  
Author(s):  
Elvis T. Valera ◽  
Rosana Cipolotti ◽  
José E. Bernardes ◽  
Rodolfo C. Pacagnella ◽  
Danielle M. Lima ◽  
...  

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3752-3752
Author(s):  
Leonardo Feldman ◽  
Guillermo Drelichman ◽  
Nora Basack ◽  
Ernesto Rodriguez ◽  
Daniel Freigeiro ◽  
...  

Abstract Hereditary Spherocytosis (HS) is the most common cause of non-immune hemolytic anemia in Argentine people of European ancestry, it is characterized by chronic hemolysis, which is reduced or abrogated by splenectomy.. Because of the risk of postsplenectomy sepsis, especially among children under 5 years old, partial splenectomy has been advocated as an alternative. This procedure seems to ensure prolonged reduction but not complete elimination of hemolysis and it is possible some regrowth of the splenic remnant and a secondary total splenectomy must be performed in some patients (pts). This problem is worse especially when pts. live in rural areas and a close observation is necessary. We analyzed retrospectively the evolution of 83 pediatric pts with HS with low resources, other health priorities and in some cases an underling nutritional deficit. Diagnostics was made in a public hospital between October 1971 and December 2002 and the last control was in December 2004. The pts. Median age at diagnostic was 4.7 years (14 days to 14 years). Severe forms were observed in 18%, mild in 20% and moderate in 62%. Twenty four pts were phenotypically sporadic. Biochemical analysis (SDS-PAGE) were performed in 30 pts and/or family members, combined spectrin- ankirin deficiency were observed in most of them. Sixty-nine hemolytic crisis was observed in 30 pts (37.5%). and 17.5% developed an aplastic crisis. Fifty-two pts. (65%) received 249 Red Blood Cell Transfusions (Trs), 36% between 1 and 2, 22.5% 3 to10, and 6% between 10 to 34 Trs. Gallstones were observed in 4 PTs (5%). Total splenectomy was performed in 46 of 83 pts (55%), median age: 7 years (4–15) 42 pts had conventional and 4 have undergone laparoscopyc procedure. Ninety six per cent of pts were splenectomized after age of 5. The median time from diagnostic to splenectomy was 1 year (0.2–8.9). All pts were immunized against Pneumococcus, Haemophilus, and Meningococus and received prophylactic antibiotics. With a median follow up of 18.5 years (3–30 y) after splenectomy all pts showed a significant clinical improvement and no further transfusion requirement. In all cases the hemoglobin increased (median pre and post splenectomy 8.6/13gr/dl) and reticulocyte counts decreased (8 / 1.4%). We do not observe procedure relate mortality, sepsis, thrombosis, and aplastic or hemolytic crisis post-splenectomy. On the basis of our experience we favor the total splenectomy performed by a training team in countries with low resources and in pts with deficient hygienic and housing conditions that inhabit in places with long distances from centers to ensure wider access to training health care providers.


1994 ◽  
Vol 44 (4) ◽  
pp. 409-411
Author(s):  
KOUSEI TAMURA ◽  
HITOSHI TAKE ◽  
HITOSHI KURABAYASHI ◽  
ETSUO KAWADA ◽  
KAZUO KUBOTA ◽  
...  

2014 ◽  
Vol 2014 (jul29 1) ◽  
pp. bcr2013202957-bcr2013202957 ◽  
Author(s):  
D. G. Forde ◽  
A. Cope ◽  
B. Stone

2005 ◽  
Vol 20 (1) ◽  
pp. 96 ◽  
Author(s):  
Sook Eui Oh ◽  
Jung Han Kim ◽  
Chi Hun Choi ◽  
Kwang Hyuk Park ◽  
Joo Young Jung ◽  
...  

2015 ◽  
Vol 34 (1-2) ◽  
pp. 39-44
Author(s):  
Andreea Jercan ◽  
Rusu Munteanu Gina ◽  
Camelia Dobrea ◽  
Daniel Coriu ◽  
Aurelia Tatic

Abstract Hereditary spherocytosis is an inherited hemolytic anemia due to red cell membrane defects, characterised by chronic hemolysis with different severity degrees, splenomegaly and microspherocytosis on the peripheral blood film. Among the possible complications in these patients are aplastic crisis and extramedullary hematopoiesis. In this article we present the case of a 42 years old man with hereditary spherocytosis diagnosed during childhood (average haemoglobin (Hb) value of 11-12 g/dl), which presented with worsening anemia, fever, chills, bone and muscle pain. The evolution was with accentuation of anemia (Hb 4.2 g/dl), decease of reticulocyte number (Ret 0,8%) and bilirubin (indirect bilirubin 2.7 g/dl). ParvovirusB19 DNA was 100.000.000 copies/ml. A computer tomography (CT)scan was performed and showed extramedullary hematopoiesis areas situated paravertebraly in the inferior thorax and hepatosplenomegaly. The infectious episode was self-limited and improved with substitution treatment.


IDCases ◽  
2020 ◽  
Vol 21 ◽  
pp. e00802
Author(s):  
Nicolas Cilla ◽  
Léa Domitien ◽  
Neila Arrada ◽  
Delphine Chiffre ◽  
Perrine Mahe ◽  
...  

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