IHTC NEWS:
09.11.2010 - Sickle Cell-abration 2010
The IHTC is sponsoring a free concert to raise awareness of sickle cell disease! Come and enjoy a diverse blend of music at the Madame Walker Theater. The free concert is from 5 to 9 pm; doors open at 4:30 pm. You may also choose to share the gift of life with those who have sickle cell disease by...
08.28.2010 - Hemophilia of Indiana 2010 Annual Meeting
HII’s 2010 Annual Meeting will be held on August 28-29 at the Crowne Plaza Hotel, Indianapolis-Airport, 2501 South High School Road, Indianapolis, IN 46241. Click here for more information....
04.20.2010 - IHTC voted one of Indiana's best workplaces!
As reported in the IndyStar, over 600 companies in central Indiana were invited by Workplace Dynamics of Exton, PA, to participate in a workplace survey in January 2010. Nearly 20,000 employees from 101 large, middle, and small companies voted on their workplace management,...
Sickle Cell Disease
Splenic Sequestration
Normally flexible and smooth red blood cells pass swiftly through the spleen. The spleen receives about 3 to 5 % oe total blood volume per minute. The immunologically competent portion of the spleen (white pulp) receives a relatively small portion of blood flow as compared to the splenic filter portion (red pulp).In most older children and adults with Hg SS disease the repetitive intrasplenic sickling and local infarction eventually results in scarring, fibrosis, and a non-functional spleen (called functional asplenia or auto splenectomy). In infants and young children (up to age 5-6 years), some splenic congestion and splenomegaly is not unusual.
Acute splenomegaly, pallor, or lethergy can be the first clinical signs of a potentially life threatening splenic sequestration crisis.
Splenic sequestration refers to an acute condition of intrasplenic pooling of large amounts of blood. Children with sickle cell disease between ages 5 months and two years represent most cases of splenic sequestration. During severe sequestration crisis, the blood-filled spleen may enlarge to the point of filling the entire abdomen. The child's hemoglobin may drop acutely (to as low as 1-3g/dl) resulting in hypovolemic shock and death within hours of initial onset. Prompt treatment with volume expanders and blood transfusion to reverse the hypovolemic shock can help remobilize the blood sequestered in the spleen and lead to regression of the splenomegaly over a fairly short period of time.
Minor sequestration events are common in young children with sickle cell disease. Some cases are associated with viral illnesses. Mild episodes are characterized by moderate splenomagaly and often a 1-3g/dl decrease from the patient's baseline hemoglobin and/or thrombocytopenia.
Due to overall less sickle related infarction, the spleens of older children and young adults with Hg SC or sickle beta thalassemia may remain enlarged (persistent splenomegaly) or retain the ability to enlarge. Therefore, they are also at risk for sequestration.
Because sequestration tends to recur and because of the acute nature of potentially catastrophic symptoms and fatal outcome, Splenectomy should be considered if the child has had more than one episode.
