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Splenomegaly is an enlargement of the spleen. [1] The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen.Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the ...
The risk is greatest for children and elderly (70+ years old), but it can happen at any age. Greater risk is associated with splenectomy for hematological conditions such as sickle cell anemia, thalassemia and tumours when compared to splenectomy due to trauma. [2] [4]
Acquired asplenia occurs for several reasons: . Following splenectomy due to splenic rupture from trauma or because of tumor; After splenectomy with the goal of interfering with splenic function, as a treatment for diseases (e.g. idiopathic thrombocytopenic purpura, thalassemia, spherocytosis), in which the spleen's usual activity exacerbates the disease
All people with ALPS have signs of lymphoproliferation, which makes it the most common clinical manifestation of the disease. The increased proliferation of lymphoid cells can cause the size of lymphoid organs such as the lymph nodes and spleen to increase (lymphadenopathy and splenomegaly, present in respectively over 90% and over 80% of patients).
The spleen is the largest collection of lymphoid tissue in the body. It is normally palpable in preterm infants, in 30% of normal, full-term neonates, and in 5% to 10% of infants and toddlers. A spleen easily palpable below the costal margin in any child over the age of three to four years should be considered abnormal until proven otherwise.
Enlargement of the spleen is a requirement for the diagnosis of SMZL and is seen in nearly all people affected by SMZL (often without lymphadenopathy). [1] Aside from the uniform involvement of the spleen, the bone marrow is frequently positive in patients with SMZL displaying a nodular pattern with morphology similar to what is observed in the splenic hilar lymph nodes. [7]
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Much of the spleen's protective roles can be maintained if a small amount of spleen can be left behind. [17] Where clinically appropriate, attempts are now often made to perform either surgical subtotal (partial) splenectomy, [ 18 ] or partial splenic embolization . [ 19 ]