Splenomegaly Information and Courses from MediaLab, Inc.
These are the MediaLab courses that cover Splenomegaly and links to relevant pages within the course.
Learn more about laboratory continuing education for medical technologists to earn CE credit for AMT, ASCP, NCA, and state license renewal and recertification. Or get information about laboratory safety and compliance courses that deliver cost-effective OSHA safety training and continuing education to your laboratory's employees.
| Alpha Thalassemia Intermedia Alpha thalassemia intermedia (Hemoglobin H Disease) results from a deletion of three out of four alpha chain loci. Infants born with alpha thalassemia intermedia appear normal at birth but often develop anemia and splenomegaly by the end of their first year. Hepatomegaly is not a common finding and there may be some association with mental retardation. Due to the hemolytic nature of this anemia, there may be an increase in respiratory infections, leg ulcers and gallstones. Skeletal changes are not commonly seen in hemoglobin H disease. Every ethnic group can have occurrences of hemoglobin H disease; but it is most often seen in Southeast Asian, the Middle East and the Mediterranean islands. Development and life expectancy are usually normal, but some affected individuals may require splenectomy and transfusion therapy. | View Page |
| Summary The normal RBC count (4.84 x 1012/L) in this case, together with the decreased hemoglobin (8.4 g/dL) and MCV (59 fl) is an indicator of ineffective erythropoeisis that often points to thalassemia.The RBC morphology shows slight hypochromic microcytosis with codocytes, schizocytes, and basophilic stippling. Schizocytes form by several mechanisms, one being the removal of RBC inclusions.This patient's elevated bilirubin correlates with her presentation of sclera icterus; her splenomegaly is consistent with increased RBC destruction.The Hb electrophoresis demonstrated a normal pattern, initially, but the unstable Hemoglobin H was revealed upon repeat electrophoresis with reduced incubation time. Hemoglobin H is the result of beta globin chain tetramer formation due to the insufficient supply of alpha globin chains in alpha thalassemia intermedia.People with Hemoglobin H disease (alpha thalassemia intermedia) usually have a normal life expectancy without treatment. However, hemolysis may lead to moderate anemia that may be treated with splenectomy. | View Page |
| Beta Thalassemia Major Children with beta thalassemia major, also called Cooley's anemia, usually develop clinical signs during their first year of life. They appear to be malnourished and may exhibit abdominal girth expansion. They show skeletal deformations, which are a result of increased erythropoiesis. A common finding is facial bone changes. Other clinical signs include frequent infections, hepatomegaly, splenomegaly, cardiomegaly, gall stones, leg ulcers, and poor growth and sexual development. Death usually occurs by the time these patients are in their early twenties unless treated with blood transfusions along with iron-chelating agents. If no chelating agent is used during treatment life will only be prolonged by about a decade.Beta thalassemia is found most often in populations of people from the Mediterranean, southern China, and India. | View Page |
| Beta Thalassemia Intermedia Clinically, beta thalassemia intermedia is midway in severity between major and minor. Growth and development in children with this disorder can usually be considered normal and most patients have a normal life span. However, they can demonstrate some facial bone deformity and splenomegaly. Transfusions (again with iron-chelating agents) may be used as a supportive therapy. This form of beta thalassemia is most common in eastern Mediterranean countries. | View Page |
| The patient, an 8-month-old girl, was anemic, jaundiced, and had splenomegaly. Her family had immigrated from the Middle East. Based on the history and the peripheral blood picture, the most probable diagnosis is thalassemia. | View Page |