scholarly journals Clinical Case of Aplastic Crisis Associated with Extramedullary Hematopoiesis in an Adult With Hereditary Spherocytosis and Parvovirus B19 Infection

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.

1992 ◽  
Vol 34 (5) ◽  
pp. 479-482 ◽  
Author(s):  
R. C. N. Cubel ◽  
M. C. Valadão ◽  
W. V. Pereira ◽  
M. C. Magalhães ◽  
J. P. Nascimento

Specific anti-B19 IgM was demonstrated in sera from three children showing transient aplastic crisis. A two years-old boy living in Rio de Janeiro suffering from sickle-cell anaemia showed the crisis during August, 1990. Two siblings living in Santa Maria, RS, developed aplastic crisis during May, 1991, when they were also diagnosed for hereditary spherocytosis. For a third child from this same family, who first developed aplastic crisis no IgM anti-B19 was detected in her sera.


2016 ◽  
Vol 38 (1) ◽  
pp. 81-82 ◽  
Author(s):  
Anna Alonso-Saladrigues ◽  
Albert Català ◽  
Rubén Berrueco ◽  
Mireia Camós ◽  
Montserrat Torrebadell ◽  
...  

2020 ◽  
Vol 27 (2) ◽  
pp. 120-123
Author(s):  
Hyungsuk Jin ◽  
Ji Won Lee ◽  
Hee Young Ju ◽  
Hee Won Cho ◽  
Ju Kyung Hyun ◽  
...  

2020 ◽  
pp. 5456-5463
Author(s):  
Patrick G. Gallagher

The integrity of the red cell membrane depends on molecular interactions between proteins and the phospholipid membrane: vertical interactions stabilize the membrane lipid bilayer; horizontal interactions provide resistance against shear stress. Hereditary spherocytosis—affects 1 in 25 000 individuals of northern European descent. There is typically a dominant family history, but the condition is genetically heterogeneous: combined spectrin and ankyrin deficiency is the most common defect observed, followed by band 3 deficiency, isolated spectrin deficiency, and protein 4.2 deficiency. These affect vertical membrane interactions with loss of surface area relative to red cell volume. Clinical features—the key clinical manifestations are anaemia and signs of persistent haemolysis, with jaundice and a marked propensity to gallstones. Complications and treatment—parvovirus B19 infection of erythropoietic precursors may cause acute aplastic crises. Megaloblastic anaemia due to folate deficiency occurs in response to increased requirements during growth and pregnancy, but is preventable with supplementation. Splenectomy can alleviate the anaemia in many patients and reduces the risk of gallstones. Hereditary elliptocytosis—occurs with a frequency of 1 in 2000 to 1 in 4000 worldwide, and is more frequent in parts of Africa. The inheritance is usually dominant, with defects in red cell proteins such as α‎- and β‎-spectrin causing disturbances in horizontal interactions in the erythrocyte membrane. Clinical features, diagnosis, and treatment—most patients are asymptomatic and are typically diagnosed incidentally during testing for unrelated conditions, but about 10% experience haemolysis, anaemia, splenomegaly, and intermittent jaundice. Diagnosis is based on the presence of elliptocytes on a peripheral blood smear. Treatment is rarely required. Other conditions include hereditary pyropoikilocytosis, South-East Asian (or Melanesian) ovalocytosis, stomatocytosis, and acanthocytosis.


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 ◽  
...  

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