Poster on Hemoglobin H-Constant Spring at ASH
January 15, 2009 – The Thalassemia Clinical Research Network (TCRN) presented a poster on Hemoglobin H-Constant Spring , a form of alpha-thalassemia, at the annual meeting of the American Society of Hematology (ASH) in December.
The poster, entitled “Hemoglobin H-Constant Spring in North America: A Common Alpha Thalassemia with Frequent Complications,” reported on a study utilizing data from the TCRN’s registry. The study examined information pertaining to 46 individuals with Hemoglobin H-Constant Spring.
Individuals with Hemoglobin H disease make less than the usual amount of alpha globin, which can cause anemia. . In most cases, this anemia is mild to moderate; however, those individuals with the form of Hemoglobin H known as “Constant Spring” tend to have a more severe anemia and are more likely to need blood transfusions, sometimes on a regular basis.
The TCRN poster states that the Contsant Spring mutation is “the most prevalent non-deletional thalassemia mutation in southeast Asia and southern China.” The aims of the study were to:
1. Characterize the clinical and hematological complications in comparison to those with Hemoglobin H disease
2. Characterize the various treatments provided to these patients throughout the TCRN centers.
3. Establish preliminary data for future determination of standards for monitoring and treating patients with this disorder.
The study found that:
1. Patients with Hemoglobin H-Constant Spring have a more severe phenotype of alpha thalassemia than patients with Hemoglobin H. 25% are on regular transfusions; about 30% have splenomegaly or underwent splenectomy; about 20% have cholelithiasis or cholecystectomy (gallstones or gallbladder removal); and iron overload in non-transfused patients is common.
2. Transfusion therapy ameliorates many of these complications but has the known complications of iron overload.
3. Growth delay and reduced bone mass are common.
4. Guidelines are needed for regular monitoring of anemia, growth, bone mass, iron overload and for appropriate supportive care, starting at an early age.
5. Standardized considerations for start of transfusion are needed.