scholarly journals Extramedullary Haematopoiesis Causing Spinal Cord Compression in a Thalassemia Intermedia Patient

2013 ◽  
Vol 2 (2) ◽  
pp. 81-85
Author(s):  
S Wahab ◽  
RA Khan ◽  
K Ahmad ◽  
A Wahab ◽  
I Ahmad

The thalassemias are a heterogenous group of inherited disorders of hemoglobin (Hb) synthesis characterized by a lack of or decreased /defective synthesis of globin chains. The name Mediterranean anemia, which Whipple introduced, is misleading because the condition can be found in any part of the world. In ?-thalassemia, ?-globin chain synthesis is reduced, whereas in ?-thalassemia, ?-globin chain synthesis is defective. Their clinical severity varies widely, ranging from asymptomatic forms to severe or even fatal entities. Like most anemias, erythropoietin production and erythropoiesis is increased in thalassemia causing erythroid marrow hyperplasia as well as extramedullary haematopoiesis which is a common compensatory mechanism for chronic anemia found in patients with hemoglobinopathies such as thalassemia, sickle cell anemia, and hereditary spherocytosis. These patients are usually asymptomatic. Extramedullary hematopoiesis (EMH) usually manifests in the liver, spleen, kidneys and as paravertebral masses. Extramedullary hematopoietic tissue occurring within the spinal canal and causing cord compression is an unusual but well-described entity. Total surgical excision is usually not feasible because of the diffuse spread of extramedullary hematopoietic tissue and the possibility of recurrence, but acute neurological impairment does require emergency surgery. Extramedullary hematopoiesis is radiosensitive so radiation therapy is widely accepted as a very effective mode of therapy. Repeated blood transfusion and cytostatic agents are also recommended for treatment.We report a case of a 35-year-old man with underlying beta thalassemia intermedia who developed pain in lower back and progressive paresthesia and weakness of both lower limbs due to multifocal extramedullary epidural hematopoietic tissue occurring within the spinal canal with compression over the cord. Nepalese Journal of Radiology; Vol. 2; Issue 2; July-Dec. 2012; 81-85 DOI: http://dx.doi.org/10.3126/njr.v2i2.7692

Blood ◽  
1972 ◽  
Vol 40 (1) ◽  
pp. 105-111 ◽  
Author(s):  
Mordechai Shchory ◽  
Bracha Ramot

Abstract α, β, and γ globin chain synthesis in bone marrow and peripheral blood reticulocytes were studied in two patients with thalassemia major, two with thalassemia intermedia, one with thalassemia minor, one with Hb H disease, and one with homozygous βδ-thalassemia. Nine nonthalassemic patients served as controls. In thalassemia major, a marked imbalance of α- to β-chain synthesis was found in the bone marrow as well as in reticulocytes. The imbalance, however, was slightly more evident in the latter. In the patients with thalassemia intermedia and minor the α- to β-globin chain ratios in the reticulocytes were of the same order of magnitude, despite the marked clinical differences between thalassemia intermedia and minor. A balanced synthesis was found in the bone marrow of the patient with thalassemia minor. The bone marrow globin synthesis in thalassemia intermedia was not studied. Contrary to that in Hb H disease and βδ-thalassemia, the imbalance was more apparent in the bone marrow. In the latter, no evidence for imbalance was detected in the reticulocytes. These results point out the need for further studies on globin chain synthesis in the bone marrow and reticulocytes of patients With the various thalassemia syndromes and the effect of the free globin chain pool on those results.


Hemoglobin ◽  
2019 ◽  
Vol 43 (6) ◽  
pp. 344-344
Author(s):  
Corinne Vasseur ◽  
Elisa Domingues-Hamdi ◽  
Katia Ledudal ◽  
Philippe Le Corvoisier ◽  
Caroline Barau ◽  
...  

1980 ◽  
Vol 46 (4) ◽  
pp. 557-564 ◽  
Author(s):  
D. Todd ◽  
Vivian Chan ◽  
Rose G. Schneider ◽  
Andree M. Dozy ◽  
Y. W. Kan ◽  
...  

2008 ◽  
Vol 184 (4) ◽  
pp. 224-226 ◽  
Author(s):  
Martin Goerner ◽  
Sabine Gerull ◽  
Erhard Schaefer ◽  
Marianne Just ◽  
Martin Sure ◽  
...  

Blood ◽  
1978 ◽  
Vol 51 (4) ◽  
pp. 653-658 ◽  
Author(s):  
RS Franco ◽  
JW Hogg ◽  
OJ Martelo

Abstract To define further the role of hemin-controlled repressor (HCR) in globin synthesis, we studied its effect on the synthesis of individual globin chains in a rabbit reticulocyte lysate cell-free system. In the presence of HCR there was a marked globin chain imbalance, resulting in a lowered alpha/beta ratio. These findings in vitro may have relevance to certain clinical heme deficiency states in which a similar globin chain imbalance has been observed.


Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 341-345 ◽  
Author(s):  
M Pirastu ◽  
R Galanello ◽  
MA Melis ◽  
C Brancati ◽  
A Tagarelli ◽  
...  

Abstract We have defined a new type of delta-thalassemia in which beta-globin chain synthesis is incompletely suppressed. Homozygotes have unusually low HbA2 levels, and double heterozygosity for this delta-thalassemia gene and beta-thalassemia normalizes the HbA2 level. The delta- thalassemia occurs on a chromosome that is identifiable using polymorphic restriction endonuclease sites. We call this condition delta +-thalassemia, to distinguish it from the previously described delta 0-thalassemia syndromes in which no delta-globin chain synthesis occurs.


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